Paranasal sinus mucocele.

Head & Neck, Sinonasal Tract
Thompson LD.
Ear Nose Throat J. 2012 Jul;91(7):276-8.
FIRST PARAGRAPHS: Radiographic findings are essential to the diagnosis of paranasal sinus mucocele. Usually opacification of the sinus with thinning, erosion, or destruction of the sinus wall are seen. Mucocele of the paranasal sinuses is a distinct clinicopathologic entity in which there is obstruction of the sinus cavity outflow tract, resulting in expansion of the sinus walls. The histologic features are quite nonspecific, requiring clinical, radiologic, and pathologic correlation. Most of these lesions result from increased pressure in the sinus due to sinus outlet obstruction, usually as a consequence of inflammatory or allergic processes. However, tumor, trauma, and previous surgery may play a role.
PubMed ID: 22829031
Article Size: <1 MB

Paraneoplastic syndromes in patients with laryngeal neuroendocrine carcinomas: Clinical manifestations and prognostic significance.

Head & Neck, Larynx
Ferlito A, Rinaldo A, Bishop JA, Hunt JL, Poorten VV, Williams MD, Triantafyllou A, Devaney KO, Gnepp DR, Kusafuka K, Halmos GB, Westra WH, Takes RP, Thompson LDR
Eur Arch Otorhinolaryngol. 2016 Mar;273(3):533-6.
Paraneoplastic syndromes are associated with a variety of malignant neoplasms and are systemic and nonmetastatic manifestations that develop in a minority of cancer patients. This review examines all published cases of paraneoplastic syndromes associated with neuroendocrine carcinomas of the larynx. There are a total of ten patients reported with paraneoplastic syndromes associated with laryngeal neuroendocrine carcinomas in the literature. Of these, nine died and the tenth is alive with liver metastases. There were five cases of small-cell neuroendocrine carcinoma, four cases of moderately differentiated neuroendocrine carcinoma, and one case of well-differentiated neuroendocrine carcinoma associated with paraneoplastic syndromes. As these syndromes have significant clinical relevance, physicians should be aware of the possible presence of paraneoplastic syndromes in the diagnostic process of patients with neuroendocrine carcinoma of the larynx.
PubMed ID: 25351497
Article Size: <1 MB
 

Parotid Gland Nodular Fasciitis: A Clinicopathologic Series of 12 Cases with a Review of 18 Cases from the Literature.

Head & Neck, Salivary Gland
Gibson TC, Bishop JA, Thompson LD.
Head Neck Pathol. 2015 Sep;9(3):334-44.
Nodular fasciitis (NF), very uncommon in the parotid gland, is a benign myofibroblastic proliferation that may be mistaken for other neoplastic proliferations. The mass-like clinical presentation and histologic features result in frequent misclassification, resulting in inappropriate clinical management. There are only a few reported cases in the English literature. Cases within the files of the authors’ institutions (retrospective) confined to the parotid gland were compared to cases reported in the English literature (Medline 1966–2014). The patients included five females and seven males, aged 11–70 years (mean 45.2 years). All patients presented with a mass lesion, present on average 1.9 months, without a documented history of trauma. The lesions were 0.7–5.2 cm (mean 2.2 cm). Seven patients had fine needle aspiration. The majority of the lesions were circumscribed (n = 9), composed of spindle-shaped to stellate myofibroblasts (MF) arranged in a storiform growth pattern, juxtaposed to hypocellular myxoid tissue-culture-like areas with extravasation of erythrocytes. Dense, keloid-like collagen (n = 7) and occasional giant cells were seen (n = 6). Mitotic figures (without atypical forms) were readily identifiable (mean 4/10 HPFs). By immunohistochemical staining, the MF were reactive with vimentin, actins, and calponin, while the histiocytes were reactive with CD68. All patients had surgical excision. One patient developed local recurrence (12 months later). All were alive and disease free at last follow-up, with a mean 133 months of follow-up. The principle differential diagnoses include fibrosarcoma, fibromatosis, pleomorphic adenoma, myoepithelioma, neurofibroma, schwannoma, solitary fibrous tumor, leiomyoma, fibrous histiocytoma and myxoma. NF of the parotid gland occurs in middle-aged patients who present with a mass (mean 2.2 cm) in the parotid gland of short duration (1.9 months). FNA misinterpretation frequently leads to excision. Separation from myoepithelial and mesenchymal lesions affecting the parotid gland results in appropriate management.
PubMed ID: 25472697
Article Size: 3.7 MB
 
 
 
 

Parotid Gland Solitary Fibrous Tumor: A Case Report and Clinicopathologic Review of 22 Cases from the Literature.

Head & Neck, Salivary Gland
Bauer JL, Miklos AZ, Thompson LD.
Head Neck Pathol. 2012 Mar;6(1):21-31.
Solitary fibrous tumors (SFTs) are rare tumors in the head and neck, and even more so in the parotid gland. The mass-like clinical presentation and histologic features result in frequent misclassification, resulting in inappropriate clinical management. There are only a few reported cases in the English literature. Twenty-one patients with parotid gland solitary fibrous tumor were compiled from the English literature (Medline 1960-2011) and integrated with this case report. The patients included 11 males and 11 females, aged 11-79 years (mean, 51.2 years), who presented with a parotid gland painless mass gradually increasing in size or with compression symptoms, with a mean duration of symptoms of 24.7 months. The mean tumor size was 4.5 cm. Grossly, all tumors were described as well-circumscribed to encapsulated, firm, homogenous white to tan masses. Seven patients had a preoperative fine needle aspiration performed, with the majority interpreted to represent pleomorphic adenoma or cementifying fibroma. Histologically, the tumors were well circumscribed, although many tumors showed focally entrapped normal salivary gland acini and ducts at the edge. The tumors were cellular, arranged in haphazard short interlacing fascicles of spindled to epithelioid cells. The spindled cells showed tapering cytoplasm with monotonous, round to oval nuclei with coarse nuclear chromatin distribution. Keloid-like to wiry collagen was present between the neoplastic cells. Mitoses were identified in most cases, while necrosis was absent. Isolated, patulous vessels were present, but a well developed ‘hemangiopericytoma-like’ vascular pattern was not seen. Three tumors were classified as malignant, showing marked nuclear pleomorphism and increased mitoses. When immunohistochemistry was performed, all tumors showed strong and diffuse vimentin, with a majority showing CD34, bcl-2 and CD99 immunoreactivity; all cases tested were negative for S100 protein, cytokeratin, EMA, CAM5.2, smooth muscle actin, muscle specific actin, desmin, MYOD1, myogenin, CD117, GFAP, CD31, FVIII-Rag, collagen IV, p63, p53, calponin, caldesmon, CD56, NFP, and ALK-1. The principle differential diagnoses include pleomorphic adenoma, myoepithelioma, nodular fasciitis, schwannoma, fibromatosis coli, spindle cell ‘sarcomatoid’ carcinoma, and spindle cell melanoma. All patients were managed with surgery, while two patients also received radiation therapy. Metastatic disease was identified in one patient immediately after excision. All patients with follow-up were alive without evidence of disease (n = 18), but the average follow-up is only 1.9 years. One patient is alive with disease at 12 months. Parotid gland SFT is a rare tumor, usually presenting in middle aged adults as a slowly growing mass. Characteristic histologic appearance with CD34 and bcl-2 immunoreactivity support the diagnosis. Surgery is the treatment of choice to yield a good outcome.
PubMed ID: 22002440
Article Size: 1 MB
 

Pharyngeal dermoids (‘hairy polyps’) as accessory auricles.

Head & Neck, Oropharynx
Heffner DK, Thompson LD, Schall DG, Anderson V.
Ann Otol Rhinol Laryngol. 1996 Oct;105(10):819-24.
The purpose of this study is to clarify the origin and nature of so-called hairy polyps or dermoids of the pharynx, which are often thought to be a variant of pharyngeal teratoma. For this purpose, a case is reported of a dermoid polyp involving the middle ear of an infant, the features of multiple examples of pharyngeal dermoid polyps and teratomas received for consultation by the Armed Forces Institute of Pathology are examined, and selected pertinent reports from the literature are reviewed. All three means are used to support the conclusion that these lesions are choristomatous developmental anomalies arising from the first branchial cleft area and that they essentially represent heterotopic accessory ‘ears’ (auricles) without the growth potential of a teratoma.
PubMed ID: 8865778
Article Size: <1 MB
 

Polymorphous hemangioendothelioma of the neck.

Head & Neck, Neck
Tadros M, Rizk SS, Opher E, Thompson LD.
Ann Diagn Pathol. 2003 Jun;7(3):165-8.
Polymorphous hemangioendotheliomas are rare, low-grade borderline malignant vascular tumors of endothelial cell origin. To the best of our knowledge (MEDLINE 1966-2002), there have been nine cases of polymorphous hemangioendothelioma reported in the English literature. Most of the initial patients reported were men, but we present the third case in a woman. Her previous radiation history to the neck makes this report unique. Polymorphous hemangioendothelioma is characterized by the variety of patterns of growth within and between tumors, making histologic recognition of the tumor difficult. Because management remains conservative via wide local excision, the misdiagnosis of this lesion as a malignancy has possible treatment implications. Alternatively, the high propensity for local recurrence underscores the necessity for accurate classification of the neoplasm and close clinical follow-up.
PubMed ID: 12808568
Article Size: <1 MB
 

Polymorphous low grade adenocarcinoma.

Head & Neck, Salivary Gland
Thompson LDR.
Pathol Case Rev 2004;9:259-263.
Polymorphous low-grade adenocarcinomas are minor salivary gland neoplasms with a predilection for intraoral sites. Women are affected twice as frequently as men, and generally present in the fifth to sixth decade of life with a painless ihaoral mass. The palatal mass is, on average, about 2 cm in greatest dimension. The tumors are submucosal, identified below an intact mucosa as a well-circumscribed although unencapsulated mass. The tumor is characterized by a polymorphous growth pattern, with individual tumors demonstrating multiple patterns, including solid, ductal-tubular, cribriform, trabecular, and single-file growth. Neurotropism is common, frequently forming a central nidus around which a “targetoid” pattern is formed. The neoplastic cells are isomorphic, containing round to oval vesicular nuclei with small nucleoli. Mitotic activity and necrosis are inconspicuous. There is frequently a slate gray-blue stroma separating the tumor cells. Immunohistochemical analysis demonstrates reactivity with cytokeratin, vimentin, S-100 protein, CD117, glial fibrillary acidic protein, and actin. Bcl-2 is overexpressed and there is generally a low proliferation index as determined by Ki-67 reactions. The tumor must be separated from pleomorphic adenoma (benign mixed tumor) and adenoid cystic carcinoma. Complete surgical excision will yield a more than 95% 10-year survival, although persistence or recurrence can emerge often in about 10% of patients more than 10 years later.
PubMed ID: n/a
Article Size: 4.9 MB

Polymorphous low grade adenocarcinoma: a clinicopathologic study of 164 cases.

Head & Neck, Salivary Gland
Castle JT, Thompson LD, Frommelt RA, Wenig BM, Kessler HP.
Cancer. 1999 Jul 15;86(2):207-19.
BACKGROUND: Polymorphous low grade adenocarcinomas (PLGA) are minor salivary gland neoplasms with a predilection for intraoral sites.
METHODS: One hundred sixty-four cases of PLGA diagnosed between 1970-1994 were retrieved from the files of the Armed Forces Institute of Pathology, Washington, DC. Histologic features were reviewed, immunohistochemical studies and prognostic markers were performed, and patient follow-up was obtained. The data were analyzed statistically.
RESULTS: The patients included 109 women and 55 men, ages 23-94 years (average, 57.6 years). The patients usually presented clinically with a palatal mass that ranged in size from 0.4-6 cm (average, 2.2 cm). The tumors were infiltrative and characterized by a polymorphous growth pattern, with individual tumors demonstrating multiple patterns, including solid, ductotubular, cribriform, trabecular, and single file growth. Neurotropism was identified frequently. The neoplastic cells were isomorphic with vesicular nuclei. Mitotic activity was inconspicuous. At an average of 115.4 months after presentation, approximately 97.6% of all patients were either alive or had died without evidence of recurrent disease after treatment with surgical excision only. Four patients had evidence of disease at last follow-up; three had died with evidence of tumor, and one patient was alive with tumor.
CONCLUSIONS: PLGA is a neoplasm of minor salivary gland origin that must be separated from adenoid cystic carcinoma and benign mixed tumor for therapeutic and prognostic considerations. Conservative but complete surgical excision is the treatment of choice for these slow-growing tumors with a low proliferation index; adjuvant therapy does not appear to alter the prognosis.
PubMed ID: 10421256
Article Size: 2 MB
 

Primary ameloblastoma of the sinonasal tract: a clinicopathologic study of 24 cases.

Head & Neck, Sinonasal Tract
Schafer DR, Thompson LD, Smith BC, Wenig BM.
Cancer. 1998 Feb 15;82(4):667-74.
BACKGROUND: Ameloblastomas are locally aggressive jaw tumors with a high propensity for recurrence and are believed to arise from the remnants of odontogenic epithelium. Extragnathic ameloblastomas are unusual and primary sinonasal tract origin is extraordinarily uncommon.
METHODS: Twenty-four cases of ameloblastoma confined to the sinonasal tract were retrieved from the Otorhinolaryngic-Head & Neck Pathology and Oral-Maxillofacial Pathology Tumor Registries of the Armed Forces Institute of Pathology between 1956 and 1996.
RESULTS: The patients included 5 females and 19 males with an age range of 43-81 years, with a mean age at presentation of 59.7 years. The patients presented with an enlarging mass in the maxillary sinus or nasal cavity (n = 24), sinusitis (n = 9), or epistaxis (n = 8). Unilateral opacification of the maxillary sinus (n = 12) was the most common radiographic finding. Histologically, the tumors exhibited the characteristic features of ameloblastoma, including peripherally palisaded columnar cells with reverse polarity. The majority of the tumors showed a plexiform growth pattern. Fifteen tumors demonstrated surface epithelial derivation. Surgical excision is the treatment of choice, ranging from conservative surgery (polypectomy) to more aggressive surgery (radical maxillectomy). Five patients experienced at least 1 recurrence, usually within 1 year of initial surgery. With follow-up intervals of up to 44 years (mean, 9.5 years), all 24 patients were alive without evidence of disease or had died of unrelated causes, without evidence of disease.
CONCLUSIONS: Primary ameloblastoma of the sinonasal tract is rare. In contrast to their gnathic counterparts, sinonasal tract tumors have a predilection for older age men. Therapy should be directed toward complete surgical resection to prevent local tumor recurrence.
PubMed ID: 9477098
Article Size: 1 MB
 

Primary ear and temporal bone meningiomas: a clinicopathologic study of 36 cases with a review of the literature.

Ear & Temporal Bone, Head & Neck
Thompson LD, Bouffard JP, Sandberg GD, Mena H.
Mod Pathol. 2003 Mar;16(3):236-45.
‘Primary’ ear and temporal bone meningiomas are tumors that are frequently misdiagnosed and unrecognized, resulting in inappropriate clinical management. To date, a large clinicopathologic study of meningiomas in this anatomic site has not been reported. Thirty-six cases of ear and temporal bone meningiomas diagnosed between 1970 and 1996 were retrieved from our files. Histologic features were reviewed, immunohistochemical analysis was performed (n = 19), and patient follow-up was obtained (n = 35). The patients included 24 females and 12 males, aged 10-80 years (mean, 49.6 years), with female patients presenting at an older age (mean, 52.0 years) than male patients (mean, 44.8 years). Patients presented clinically with hearing changes (n = 20), otitis (n = 7), pain (n = 5), and/or dizziness/vertigo (n = 3). Symptoms were present for an average of 24.6 months. The tumors affected the middle ear (n = 25), external auditory canal (n = 4), or a combination of temporal bone and middle ear (n = 7). The tumors ranged in size from 0.5 to 4.5 cm in greatest dimension (mean, 1.2 cm). Radiographic studies demonstrated a central nervous system connection in 2 patients. Histologically, the tumors demonstrated features similar to those of intracranial meningiomas, including meningothelial (n = 33), psammomatous (n = 2), and atypical (n = 1). An associated cholesteatoma was identified in 9 cases. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (79%) and vimentin (100%). The differential diagnosis includes paraganglioma, schwannoma, carcinoma, melanoma, and middle ear adenoma. Surgical excision was used in all patients. Ten patients developed a recurrence from 5 months to 2 years later. Five patients died with recurrent disease (mean, 3.5 years), and the remaining 30 patients were alive (n = 25, mean: 19.0 years) or had died (n = 5, mean: 9.5 years) of unrelated causes without evidence of disease. We conclude that extracranial ear and temporal bone meningiomas are rare tumors histologically similar to their intracranial counterparts. They behave as slow-growing neoplasms with a good overall prognosis (raw 5-y survival, 83%). Extent of surgical excision is probably the most important factor in determining outlook because recurrences develop in 28% of cases.
PubMed ID: 12640104
Article Size: 2 MB
 

Prognostic biological features in neck dissection specimens.

Head & Neck, Oropharynx
Woolgar JA, Triantafyllou A, Lewis JS Jr, Hunt J, Williams MD, Takes RP, Thompson LD, Slootweg PJ, Devaney KO, Ferlito A.
Eur Arch Otorhinolaryngol. 2013 May;270(5):1581-92.
The superior prognostic value offered by routine histopathological staging of neck dissections, as compared to clinical staging using palpation and modern imaging techniques, is well established in the literature concerning the management of squamous cell carcinoma of the head and neck. In this review, we discuss the definitions and criteria used in standardised routine histopathological reporting and explore additional potential nodal prognostic features. In addition, we critically appraise the value of immunohistochemistry, histochemistry, molecular and other non-morphological techniques and suggest tumour and host features that merit further investigations.
PubMed ID: 22983222
Article Size: 1 MB
 

Protocol for the Examination of Specimens from Patients with Carcinomas of the Larynx

Head & Neck, Larynx
College of American Pathologists (CAP)
Protocol applies to all invasive carcinomas of the larynx, including supraglottis, glottis, and subglottis. Mucosal malignant melanoma is included. Lymphomas and sarcomas are not included.

Based on AJCC/UICC TNM, 7th edition
Protocol web posting date: October 2009
Protocol effective date: January 2010

Article Size: 1 MB

Protocol for the Examination of Specimens from Patients with Carcinomas of the of the Nasal Cavity and Paranasal Sinuses

Head & Neck, Sinonasal Tract
College of American Pathologists (CAP)

Protocol applies to all invasive carcinomas of the nasal cavity and paranasal sinuses. Mucosal malignant melanoma is included. Lymphomas, neuroectodermal neoplasms, and sarcomas are not included.

Based on AJCC/UICC TNM, 7th edition
Protocol web posting date: October 2009
Protocol effective date: January 2010

Article Size: 1 MB

Relapsing polychondritis.

Ear & Temporal Bone, Head & Neck
Thompson LD.
Ear Nose Throat J. 2002 Oct;81(10):705.
FIRST PARAGRAPH: Regardless of age, patients with relapsing polychondritis (systemic chondromalacia or polychondropathy) have at least one of eight progressively degenerative changes: (1) recurrent bilateral auricular chondritis, (2) inflammatory polyarthritis, (3) nasal chondritis, (4) ocular inflammation, (5) tracheal chondritis, (6) laryngeal chondritis, (7) cochlear damage, and (8) vestibular damage. Relapsing polychondritis is associated with other immunologically mediated diseases, such as systemic lupus erythematosus, rheumatoid arthritis, and Sjögren’s syndrome.
PubMed ID: 12405090
Article Size: <1 MB

Replication of HIV-1 in dendritic cell-derived syncytia at the mucosal surface of the adenoid.

Head & Neck, Oropharynx
Frankel SS, Wenig BM, Burke AP, Mannan P, Thompson LD, Abbondanzo SL, Nelson AM, Pope M, Steinman RM.
Science. 1996 Apr 5;272(5258):115-7. — Comment in: Science. 1996 Nov 15;274(5290):1067-8.
Human immunodeficiency virus-type 1 (HIV-1) replicates actively in infected individuals, yet cells with intracellular depots of viral protein are observed only infrequently. Many cells expressing the HIV-1 Gag protein were detected at the surface of the nasopharyngeal tonsil or adenoid. This infected mucosal surface contained T cells and dendritic cells, two cell types that together support HIV-1 replication in culture. The infected cells were multinucleated syncytia and expressed the S100 and p55 dendritic cell markers. Eleven of the 13 specimens analyzed were from donors who did not have symptoms of acquired immunodeficiency syndrome (AIDS). The interaction of dendritic cells and T cells in mucosa may support HIV-1 replication, even in subclinical stages of infection.
PubMed ID: 8600520
Article Size: 2.5 MB
 

Rhabdomyoblastic Differentiation in Head and Neck Malignancies Other Than Rhabdomyosarcoma.

Head & Neck, Sinonasal Tract
Bishop JA, Thompson LD, Cardesa A, Barnes L, Lewis JS Jr, Triantafyllou A, Hellquist H, Stenman G, Hunt JL, Williams MD, Slootweg PJ, Devaney KO, Gnepp DR, Wenig BM, Rinaldo A, Ferlito A.
Head Neck Pathol. 2015 Dec;9(4):507-18.
Rhabdomyosarcoma is a relatively common soft tissue sarcoma that frequently affects children and adolescents and may involve the head and neck. Rhabdomyosarcoma is defined by skeletal muscle differentiation which can be suggested by routine histology and confirmed by immunohistochemistry for the skeletal muscle-specific markers myogenin or myoD1. At the same time, it must be remembered that when it comes to head and neck malignancies, skeletal muscle differentiation is not limited to rhabdomyosarcoma. A lack of awareness of this phenomenon could lead to misdiagnosis and, subsequently, inappropriate therapeutic interventions. This review focuses on malignant neoplasms of the head and neck other than rhabdomyosarcoma that may exhibit rhabdomyoblastic differentiation, with an emphasis on strategies to resolve the diagnostic dilemmas these tumors may present. Axiomatically, no primary central nervous system tumors will be discussed.
PubMed ID: 25757816
Article Size: 3.8 MB
 
 
 
 

Rhinoscleroma.

Head & Neck, Sinonasal Tract
Thompson LD.
Ear Nose Throat J. 2002 Aug;81(8):506.
FIRST PARAGRAPH: Rhinoscleroma (‘hard nose’) is caused by Klebsiella rhinoscleromatis, a gram-negative encapsulated bacterium of low infectivity. The disease is uncommon in the United States; most cases are found in the Middle East (especially Egypt), in parts of Latin America, and in Eastern Europe. The disease process usually involves the nasal cavity and the nasopharynx, but it can also involve the larynx, trachea, bronchi, middle ear, and orbit.
PubMed ID: 12199166
Article Size: <1 MB
 

Routine histologic examination is unnecessary for tonsillectomy or adenoidectomy.

Head & Neck, Oropharynx
Randall DA, Martin PJ, Thompson LD.
Laryngoscope. 2007 Sep;117(9):1600-4
OBJECTIVE: To determine whether the current practice and incurred cost of histologic examination of tonsillectomy and adenoidectomy specimens is warranted.
STUDY DESIGN: Review article based on medical literature.
SUBJECTS AND METHODS: A retrospective PubMed review of all pertinent literature regarding tonsillectomy, adenoidectomy, and related surgical pathology was conducted. References of the articles obtained were reviewed for additional sources.
RESULTS: Twenty studies report 54,901 patients and found 54 malignancies (0.087% prevalence). Of these, 48 (88% of the patients) had suspicious features such as tonsillar asymmetry, cervical lymphadenopathy, or abnormal tonsil appearance, preoperatively. The remaining six patients without any suspicious features (better representing true occult malignancy) were 0.011% of the total cases.
CONCLUSION: Submission of tonsillectomy, adenoidectomy, or both specimens is warranted only when patients demonstrate findings associated with malignancy: tonsillar asymmetry, history of cancer, neck mass, tonsil firmness or lesion, weight loss, and constitutional symptoms.
PubMed ID: 17762791
Article Size: <1 MB
 

Salivary duct carcinoma and the concept of early carcinoma ex pleomorphic adenoma

Head & Neck, Salivary Gland
Griffith CC, Thompson LD, Assaad A, Purgina BM, Lai C, Bauman JE, Weinreb I, Seethala RR, Chiosea SI.
Histopathology. 2014 Dec;65(6):854-60.
AIMS: The data on the histological type of carcinomatous component and the extent of extracapsular invasion for salivary carcinomas ex pleomorphic adenoma (PA) are conflicting. We aimed to determine the prognostic value of extracapsular invasion in salivary duct carcinomas (SDC) ex PA.
METHODS AND RESULTS: A total of 117 patients with SDC were identified retrospectively; 44 cases involving major salivary glands had pre-existing PA (44 of 117, 37%). The morphological spectrum of SDC ex PA was characterized. The primary endpoint was overall survival (OS). Most SDC ex PA were widely invasive at presentation (27 of 44; 61%). Five patients with intracapsular SDC ex PA experienced no disease progression. The assessment of extracapsular invasion was precluded in eight cases (e.g. positive margins of resection). The rate of lymph node involvement was similar in cases with extracapsular invasion of ?2 mm (two of three) and >7 mm (22 of 26). Only pT correlated with OS [116 months, 95% confidence interval (CI) 22-210 months for pT1 versus 20 months (95% CI 6-34) for pT4; P = 0.013].
CONCLUSIONS: Intracapsular SDC ex PA are potentially indolent. SDC ex PA with extracapsular invasion of ?2 mm are rare, and appear to be clinically aggressive. Several histological parameters preclude assessment of extracapsular invasion.
PubMed ID: 24804831
Article Size: <1 MB
 
 
 
 

Salivary duct carcinoma.

Head & Neck, Salivary Gland
Thompson LD.
Ear Nose Throat J. 2012 Sep;91(9):356-9.
FIRST PARAGRAPH: Salivary duct carcinoma is a high-grade adenocarcinoma that resembles breast ductal carcinoma. It is believed to be derived from intra- and interlobular excretory ducts. Salivary duct carcinoma may arise de novo or as a relatively common malignant component of a carcinoma ex pleomorphic adenoma. It accounts for about 9% of all malignant salivary gland tumors. Although there is a wide age range at presentation, most patients present in the seventh decade of life; men are affected much more frequently than women (4:1).
PubMed ID: 22996706
Article Size: <1 MB

Salivary Duct Carcinoma: The Predominance of Apocrine Morphology, Prevalence of Histologic Variants, and Androgen Receptor Expression

Head & Neck, Salivary Gland
Williams L, Thompson LD, Seethala RR, Weinreb I, Assaad AM, Tuluc M, Ud Din N, Purgina B, Lai C, Griffith CC, Chiosea SI.
Am J Surg Pathol. 2015 May;39(5):705-13.
Salivary duct carcinoma (SDC) is a prototypic aggressive salivary gland carcinoma. Our aim is to determine the prevalence of histologic variants (micropapillary, basal-like) and androgen receptor (AR) expression in a large multi-institutional series of SDC. AR status was determined by immunohistochemistry (IHC). Most SDCs were characterized by an apocrine phenotype and AR expression. Cases with a nonapocrine phenotype and AR-negative status were studied by additional IHC and fluorescence in situ hybridization for ETV6 or MYB/NFIB. The diagnosis of SDC was confirmed in 187 of 199 (94%) cases. Variant morphologies were identified in 12 cases: micropapillary (n=6), sarcomatoid (n=3), mucinous (n=2), and basal-like (n=1). AR IHC was performed in 183 cases, of which 179 (97.8%) showed AR expression. On the basis of morphologic appearance and results of additional studies, 12 cases were reclassified as squamous cell carcinoma (SCC) (n=4), epithelial-myoepithelial carcinoma with high-grade transformation (HGT) (n=2), myoepithelial carcinoma (n=2), mammary analogue secretory carcinoma, high grade (ETV6 translocated; n=1), adenoid cystic carcinoma with HGT (n=1), acinic cell carcinoma with HGT (n=1), and adenosquamous carcinoma (n=1). AR-negative SDC is extremely rare, and the majority of such cases are more accurately classified as other entities. HGTs of other salivary carcinomas and squamous cell carcinoma are the most common mimics of SDC. SDCs with variant morphologies still show at least a minor component of conventional apocrine appearance. Thus, apocrine morphology defines SDC.
PubMed ID: 25871467
Article Size: 1 MB
 
 
 
 

Salivary gland acinic cell carcinoma.

Head & Neck, Salivary Gland
Thompson LD.
Ear Nose Throat J. 2010 Nov;89(11):530-2.
FIRST PARAGRAPH: Acinic cell carcinoma (AcCC) is a malignant epithelial salivary gland neoplasm that demonstrates serous acinar cell differentiation with cytoplasmic zymogen secretory granules. While serous-type cells tend to predominate, ductal cells are also part of this neoplasm. There are a few cases that are thought to be related to radiation exposure. AcCC accounts for about 6% of all salivary gland tumors and 10 to 12% of all malignant salivary gland tumors. Patients present at a wide range of ages (mean: 40s). Children are also affected, as AcCC is the second most common neoplasm in the pediatric age group after mucoepidermoid carcinoma. Overall, females are more affected than males by a ratio of 3:2.
PubMed ID: 21086276
Article Size: <1 MB

Sinonasal carcinomas.

Head & Neck, Sinonasal Tract
Thompson LDR.
Curr Diag Pathol 2006 12, 40–53.
Malignant neoplasms of the sinonasal tract encompass a wide variety of epithelial, lymphoid and mesenchymal tumours. The separation and classification of epithelial or neuroepithelial tumours is sometimes challenging, especially when treatment and prognosis are different. Squamous cell carcinoma, keratinizing or non-keratinizing and, usually, the poorly differentiated type need to be separated from sinonasal undifferentiated carcinoma, lymphoepithelial carcinoma, neuroendocrine carcinoma and olfactory neuroblastoma. Whereas melanoma and lymphoma are also included in the broad differential, along with primitive neuroectodermal tumours and rhabdomyosarcomas, the focus of this commentary will be to present the major clinical, radiographical, histological, immunohistochemical, ultrastructural and molecular features which allow for separation of the principle mucosal epithelial neoplasms of the sinonasal tract.
PubMed ID: n/a
Article Size: 2 MB

Sinonasal mucosal malignant melanoma: report of an unusual case mimicking schwannoma.

Head & Neck, Sinonasal Tract
Kardon DE, Thompson LD.
Ann Diagn Pathol. 2000 Oct;4(5):303-7.
Primary mucosal melanoma of the sinonasal tract is a rare malignancy that has a more aggressive clinical course than its cutaneous counterpart. The histology of these lesions varies, with differing degrees of melanin production and an epithelioid or spindle-cell growth pattern. Cutaneous melanocytic lesions may differentiate in accordance with their neural crest derivation and express morphology similar to nerve sheath tumors. We believe the following case study reports the first instance of a mucosal melanoma with a Schwannian pattern of growth, arising from the nasal cavity of a 26-year-old man. Extracranial meningiomas of the sinonasal tract are rare tumors. These tumors are frequently misclassified, resulting in inappropriate clinical management. To date, there has been no comprehensive study to evaluate the clinicopathologic aspects of meningioma in these anatomic sites. Thirty cases of sinonasal tract meningiomas diagnosed between 1970 and 1992 were retrieved from the files of the Otorhinolaryngic Registry of the AFIP. Histologic features were reviewed, immunohistochemical studies were performed, patient follow up was obtained, and the results were statistically analyzed. The patients included 15 females and 15 males, aged 13 to 88 years (mean, 47.6 yrs). Patients presented clinically with a mass, epistaxis, sinusitis, pain, visual changes, or nasal obstruction, dependent on the anatomic site of involvement. Symptoms were present for an average of 31.1 months. The tumors affected the nasal cavity (n = 14), nasopharynx (n = 3), frontal sinus (n = 2), sphenoid sinus (n = 2). or a combination of the nasal cavity and ethmoid, frontal, sphenoid, and/or maxillary sinuses (n = 9). The tumors ranged in size from 1.0 to 8.0 cm in greatest dimension (mean, 3.5 cm). Radiographic studies demonstrated a central nervous system connection in six cases. The tumors often eroded the bones of the sinuses (n = 18) and involved the surrounding soft tissues, the orbit, and occasionally the base of the skull. Histologically, the tumors demonstrated features similar to intracranial meningiomas. The majority were of the meningothelial type (n = 23), although there were three atypical meningiomas. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (EMA) and vimentin (all tested). The differential diagnosis includes paraganglioma, carcinoma, melanoma, psammomatoid ossifying fibroma, and angiofibroma. Surgical excision was used in all patients. Three patients died with recurrent disease (mean, 1.2 yrs), one was alive with recurrent disease (25.6 years), and the remaining 24 patients were alive or had died of unrelated causes (mean, 13.9 yrs) at the time of last follow up (two patients were lost to follow up). Extracranial sinonasal tract meningiomas are rare tumors which need to be considered in the differential diagnosis of sinonasal tumors. A whorled growth pattern and psammoma bodies, combined with positive EMA and vimentin immunohistochemical reactions, can confirm the diagnosis of meningioma. The overall prognosis is good, without a difference in outcome between benign and atypical meningiomas.
PubMed ID: 11073336
Article Size: <1 MB
 

Sinonasal polyps.

Head & Neck, Sinonasal Tract
Thompson LD.
Ear Nose Throat J. 2007 Jun;86(6):322, 325
FIRST PARAGRAPH: Sinonasal polyps are caused by a multitude of factors. The most common causes are repeated bouts of sinusitis, allergy, vasomotor rhinitis, infectious rhinosinusitis, and asthma. Less often, they occur in association with diabetes mellitus, cystic fibrosis, and aspirin intolerance. They form as a result of an influx of fluids into the schneiderian mucosal lamina propria. Occasionally, antral (maxillary) polyps expand and prolapse through sinus ostia to present intranasally or in the nasopharynx (antrochoanal polyps). Sinonasal polyps have no predisposition to age or sex. Polyps are uncommon in children, but when they do occur, as many as 30% are associated with cystic fibrosis.
PubMed ID: 17703805
Article Size: <1 MB

Sinonasal respiratory epithelial adenomatoid hamartomas: Series of 51 cases and literature review.

Head & Neck, Sinonasal Tract
Lee JT, Garg R, Brunworth J, Keschner DB, Thompson LD.
Am J Rhinol Allergy. 2013 Jul;27(4):322-8.
BACKGROUND: Respiratory epithelial adenomatoid hamartomas (REAHs) are rare, benign glandular proliferations of the nasal cavity, paranasal sinuses, and nasopharynx. This study aimed to expand our understanding of this entity by presenting a series of REAHs combined with a review of the pertinent literature.
METHODS: A retrospective review was performed on all patients with a diagnosis of REAH from 2002 to 2011. Data were collected with respect to age, gender, clinical presentation, imaging, histopathology, treatment, and outcome. Because olfactory cleft expansion by imaging evaluation has been reported to suggest REAH, maximum olfactory cleft (MOCs) widths were also measured.
RESULTS: Fifty-one cases of REAH included 37 male (72.5%) and 14 female subjects (27.5%) with a mean age of 58.4 years. Headache, nasal obstruction, rhinorrhea, and hyposmia were the most common presenting symptoms. Although 35(68.6%) were associated with concurrent inflammatory pathology, 16 (31.4%) presented as isolated lesions of the nasal cavity. Enlargement of MOCs was evident on computed tomography, with mean MOCs of 8.64 and 9.4 mm, in the coronal/axial planes, respectively. There were no statistically significant differences between MOCs of isolated (7.96 mm) versus MOCs of associated (9.63 mm) lesions (p = 0.25). Forty-nine were treated with endoscopic resection without evidence of recurrence after a mean follow-up of 27.2 months.
CONCLUSION: REAHs are rare sinonasal lesions that may appear as localized, isolated masses or more diffuse when in conjunction with other inflammatory processes. Irrespective of clinical presentation, endoscopic removal appears to be curative. Differentiation from more aggressive lesions is paramount to avoid unnecessarily radical surgery for an otherwise benign process.
PubMed ID: 23883815
Article Size: MB
 

Sinonasal Tract Adenoid Cystic Carcinoma Ex-Pleomorphic Adenoma: A Clinicopathologic and Immunophenotypic Study of 9 Cases Combined with a Comprehensive Review of the Literature.

Head & Neck, Sinonasal Tract
Toluie S, Thompson LD.
Head Neck Pathol. 2012 Dec;6(4):409-21.
Primary sinonasal tract carcinoma ex-pleomorphic adenoma (CEPA) is very uncommon, with adenoid cystic carcinoma (ACC) CEPA exceptional. These tumors are often misclassified. This is a retrospective study. Nine cases of ACC CEPA included 7 females and 2 males, aged 39-64 years (mean, 51.1 years). Patients presented most frequently with obstructive symptoms (n = 5), epistaxis (n = 3), nerve changes or pain (n = 3), present for a mean of 25 months (men: 9.5 versus women: 29.4 months; p = 0.264). The tumors involved the nasal cavity alone (n = 5), nasopharynx (n = 2), or a combination of locations (n = 2) with a mean size of 2.9 cm (females: 3.3; males: 1.7; p = 0.064). Most patients presented at a low clinical stage (n = 7, stage I), with one patient each in stage II and IV, respectively. Histologically, the tumors showed foci of PA associated with areas of ACC. Tumors showed invasion (lymph-vascular: n = 4; perineural: n = 6; bone: n = 6). The neoplastic cells were arranged in tubules, cribriform and solid patterns, with peg-shaped cells arranged around reduplicated basement membrane and glycosaminoglycan material. Mitoses ranged from 0 to 33, with a mean of 8.7 mitoses/10 HPFs. Necrosis (n = 2) and atypical mitotic figures (n = 1) were seen infrequently. Immunohistochemical studies showed positive reactions for cytokeratin, CK5/6, p63, CK7, EMA, SMA, calponin, S100 protein and CD117, several highlighting luminal versus basal cells components. GFAP, CK20 and MSA were non-reactive. p53 and Ki-67 were reactive to a variable degree. Surgery (n = 8), accompanied by radiation therapy (n = 5) was generally employed. Five patients developed a recurrence, all of whom died with disease (mean, 8.4 years), while 4 patients are either alive (n = 2) or had died (n = 2) without evidence of disease (mean, 15.9 years). In summary, ACC CEPA probably arises from the minor mucoserous glands of the upper aerodigestive tract, usually presenting in patients in middle age with obstructive symptoms in a nasal cavity based tumor. Most patients present with low stage disease (stage I and II), although invasive growth is common. Recurrences develop in about a 55 % of patients, who experience a shorter survival (mean, 8.4 years) than patients without recurrences (mean, 15.9 years). The following parameters, when present, suggest an increased incidence of recurrence or dying with disease: bone invasion, lymph-vascular invasion, and perineural invasion.
PubMed ID: 22941242
Article Size: 2.5 MB
 

Sinonasal Tract and Nasopharyngeal Adenoid Cystic Carcinoma: A Clinicopathologic and Immunophenotypic Study of 86 Cases.

Head & Neck, Sinonasal Tract
Thompson LD, Penner C, Ho NJ, Foss RD, Miettinen M, Wieneke JA, Moskaluk CA, Stelow EB.
Head Neck Pathol. 2014 Mar;8(1):88-109.
Primary sinonasal tract and nasopharyngeal adenoid cystic carcinomas (STACC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. Eighty-six cases of STACC included 45 females and 41 males, aged 12-91 years (mean 54.4 years). Patients presented most frequently with obstructive symptoms (n = 54), followed by epistaxis (n = 23), auditory symptoms (n = 12), nerve symptoms (n = 11), nasal discharge (n = 11), and/or visual symptoms (n = 10), present for a mean of 18.2 months. The tumors involved the nasal cavity alone (n = 25), nasopharynx alone (n = 13), maxillary sinus alone (n = 4), or a combination of the nasal cavity and paranasal sinuses (n = 44), with a mean size of 3.7 cm. Patients presented equally between low and high stage disease: stage I and II (n = 42) or stage III and IV (n = 44) disease. Histologically, the tumors were invasive (bone: n = 66; neural: n = 47; lymphovascular: n = 33), composed of a variety of growth patterns, including cribriform (n = 33), tubular (n = 16), and solid (n = 9), although frequently a combination of these patterns was seen within a single tumor. Pleomorphism was mild with an intermediate N:C ratio in cells containing hyperchromatic nuclei. Reduplicated basement membrane and glycosaminoglycan material was commonly seen. Necrosis (n = 16) and atypical mitotic figures (n = 11) were infrequently present. Pleomorphic adenoma was present in 9 cases; de-differentiation was seen in two patients. Immunohistochemical studies showed positive reactions for pan-cytokeratin, CK7, CK5/6, CAM5.2, and EMA, with myoepithelial reactivity with SMA, p63, calponin, S100 protein and SMMHC. CD117, CEA, GFAP and p16 were variably present. CK20 and HR HPV were negative. STACC needs to be considered in the differential diagnosis of most sinonasal malignancies, particularly poorly differentiated carcinoma, olfactory neuroblastoma and pleomorphic adenoma. Surgery (n = 82), often accompanied by radiation therapy (n = 36), was generally employed. A majority of patients developed a recurrence (n = 52) 2-144 months after initial presentation. Overall mean follow-up was 19.4 years (range 0.4-37.5 years): 46 patients died with disease (mean 6.4 years); 5 were alive with disease (mean 5.4 years), and 35 patients were either alive or had died of unrelated causes (mean 16.3 years). ACC of the SNT is uncommon. Recurrences are common. The following parameters, when present, suggest an increased incidence of either recurrence or dying with disease: mixed site of involvement, high stage disease (stage IV), skull base involvement, tumor recurrence, a solid histology, perineural invasion, bone invasion, and lymphovascular invasion.
PubMed ID: 24037641
Article Size: 1.5 MB
 

Sinonasal tract and nasopharyngeal melanomas: a clinicopathologic study of 115 cases with a proposed staging system.

Head & Neck, Sinonasal Tract
Thompson LD, Wieneke JA, Miettinen M.
Am J Surg Pathol. 2003 May;27(5):594-611.
Primary sinonasal tract mucosal malignant melanomas are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. A total of 115 cases of sinonasal tract mucosal malignant melanoma included 59 females and 56 males, 13-93 years of age (mean 64.3 years). Patients presented most frequently with epistaxis (n = 52), mass (n = 42), and/or nasal obstruction (n = 34) present for a mean of 8.2 months. The majority of tumors involved the nasal cavity (n = 34), septum alone, or a combination of the nasal cavity and sinuses (n = 39) with a mean size of 2.4 cm. Histologically, the tumors were composed of a variety of cell types (epithelioid, spindled, undifferentiated), frequently arranged in a peritheliomatous distribution (n = 39). Immunohistochemical studies confirmed the diagnosis of sinonasal tract mucosal malignant melanomas with positive reactions for S-100 protein, tyrosinase, HMB-45, melan A, and microphthalmia transcription factor. Sinonasal tract mucosal malignant melanomas need to be considered in the differential diagnosis of most sinonasal malignancies, particularly carcinoma, lymphoma, sarcoma, and olfactory neuroblastoma. Surgery accompanied by radiation and/or chemotherapy was generally used. The majority of patients developed a recurrence (n = 79), with 75 patients dying with disseminated disease (mean 2.3 years), whereas 40 patients are either alive or had died of unrelated causes (mean 13.9 years). A TNM-type classification separated by anatomic site of involvement and metastatic disease is proposed to predict biologic behavior.
PubMed ID: 12717245
Article Size: 2 MB
 

Sinonasal Tract Angiosarcoma: A Clinicopathologic and Immunophenotypic Study of 10 Cases with a Review of the Literature.

Head & Neck, Sinonasal Tract
Nelson BL, Thompson LDR.
Head Neck Pathol. 2007 Sep;1(1):1-12.
BACKGROUND: Primary sinonasal tract angiosarcoma are rare tumors that are frequently misclassified, resulting in inappropriate clinical management. There are only a few reported cases in the English literature.
MATERIALS AND METHODS: Ten patients with sinonasal tract angiosarcoma were retrospectively retrieved from the Otorhinolaryngic Registry of the Armed Forces Institute of Pathology.
RESULTS: Six males and four females, aged 13 to 81 years (mean, 46.7 years), presented with epistaxis and bloody discharge. Females were on average younger than their male counterparts (37.8 vs. 52.7 years, respectively). The tumors involved the nasal cavity alone (n = 8) or the maxillary sinus (n = 2), with a mean size of 4.3 cm; the average size was different between the genders: males: 2.8 cm; females: 6.4 cm. Histologically, all tumors had anastomosing vascular channels lined by remarkably atypical endothelial cells protruding into the lumen, neolumen formation, frequent atypical mitotic figures, necrosis, and hemorrhage. All cases tested (n = 6) demonstrated immunoreactivity with antibodies to Factor VIII-RA, CD34, CD31, and smooth muscle actin, while non-reactive with keratin and S-100 protein. The principle differential diagnosis includes granulation tissue, lobular capillary hemangioma (pyogenic granuloma), and Kaposi’s sarcoma. All patients had surgery followed by post-operative radiation (n = 4 patients). Follow-up was available in all patients: Six patients died with disease (mean, 28.8 months); two patients had died without evidence of disease (mean, 267 months); and two are alive with no evidence of disease at last follow-up (mean, 254 months).
CONCLUSIONS: Sinonasal tract angiosarcoma is a rare tumor, frequently presenting in middle-aged patients as a large mass usually involving the nasal cavity with characteristic histomorphologic and immunophenotypic features. Sinonasal tract angiosarcoma will often have a poor prognosis making appropriate separation from other conditions important.
PubMed ID: 20614274
Article Size: <1 MB
 

Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases.

Head & Neck, Sinonasal Tract
Thompson LD, Heffner DK.
Am J Clin Pathol. 2001 Feb;115(2):243-8.
Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrosis without a well-developed differential diagnosis. Three cases of sinonasal tract EAF were identified in 2 women and 1 man, aged 49, 64, and 28 years, respectively. The patients experienced a nasal cavity mass, maxillary pain, or nasal obstructive symptoms of long duration. The process involved the nasal septum (n = 2), nasal cavity (n = 1), and/or the maxillary sinus (n = 1). There was no evidence for Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, granuloma faciale, or erythema elevatum diutinum. Histologically, the lesions demonstrated a characteristic perivascular ‘onion-skin’ fibrosis and a full spectrum of inflammatory cells, although eosinophils predominated. Necrosis and foreign body-type giant cells were not identified. Surgical excision was used for all patients, who are all alive but with disease at last follow-up. Sinonasal tract EAF is a unique fibroproliferative disorder that does not seem to have systemic associations with known diseases. The characteristic histomorphologic features permit accurate diagnosis.
PubMed ID: 11211613
Article Size: <1 MB
 

Sinonasal tract glomangiopericytoma (hemangiopericytoma).

Head & Neck, Sinonasal Tract
Thompson LD.
Ear Nose Throat J. 2004 Dec;83(12):807.
FIRST PARAGRAPH: A glomangiopericytoma (sinonasal-type hemangio-pericytoma) is a tumor believed to derive from perivascular modified smooth-muscle cells. Its origin is similar to that of a glomus tumor (not to be confused with glomus jugulare, which is a different neoplasm) but distinctly different from soft-tissue hemangiopericytoma. There is a very slight female preponderance, and the tumor’s peak incidence occurs during the seventh decade of life. Most affected patients experience nasal obstruction and epistaxis along with a wide array of other nonspecific findings that are generally present for less than 1 year. Glomangiopericytomas have a predilection for the nasal cavity and paranasal sinuses, where they grow as polypoid masses. Their average size is approximately 3 cm, and they are often mistaken clinically for inflammatory polyps.
PubMed ID: 15724732
Article Size: <1 MB

Sinonasal Tract Mucoepidermoid Carcinoma: A Clinicopathologic and Immunophenotypic Study of 19 Cases Combined with a Comprehensive Review of the Literature.

Head & Neck, Sinonasal Tract
Wolfish EB, Nelson BL, Thompson LD.
Head Neck Pathol. 2012 Jun;6(2):191-207.
Primary sinonasal tract mucoepidermoid carcinomas (MEC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. The design of this study is retrospective. Nineteen cases of MEC included 10 females and 9 males, aged 15-75 years (mean, 52.7 years); males, on average were younger by a decade than females (47.2 vs. 57.7 years). Patients presented most frequently with a mass, obstructive symptoms, pain, and/or epistaxis present for a mean of 12.6 months. The majority of tumors involved the nasal cavity alone (n = 10), maxillary sinus alone (n = 6), or a combination of the nasal cavity and paranasal sinuses (n = 3) with a mean size of 2.4 cm. Most patients presented at a low clinical stage (n = 15, Stage I & II), with only 4 patients presenting with Stage III disease. Histologically, the tumors were often invasive (bone or perineural invasion), with invasion into minor mucoserous glands. Surface involvement was common. The neoplastic cells were composed of a combination of squamoid cells, intermediate cells, and mucocytes. Cystic spaces were occasionally large, but the majoritywere focal to small. Pleomorphism was generally low grade. Necrosis (n = 5) and atypical mitotic figures (n = 6) were seen infrequently. Over half of the tumors were classified as low grade (n = 11), with intermediate (n = 4) and high grade (n = 4) comprising the remainder. Mucicarmine was positive in all cases tested. Immunohistochemical studies showed positive reactions for keratin, CK5/6, p63, CK7, EMA, and CEA in all cases tested, while bcl-2 and CD117 were rarely positive. GFAP, MSA, TTF-1, and S100 protein were non-reactive. p53 and Ki-67 were reactive to a variable degree. MEC need to be considered in the differential diagnosis of a number of sinonasal lesions, particularly adenocarcinoma and necrotizing sialometaplasia. The patients were separated into stage I (n = 9), stage II (n = 6), and stage III (n = 4), without any patients in stage IV at presentation. Surgery occasionally accompanied by radiation therapy (n = 2) was generally employed. Six patients developed a recurrence, with 5 patients dying with disease (mean, 2.4 years), while 14 patients are either alive (n = 9) or had died (n = 5) of unrelated causes (mean, 14.6 years). MEC probably arises from the minor mucoserous glands of the upper aerodigestive tract, usually presenting in patients in middle age with a mass. Most patients present with low stage disease (stage I and II), although invasive growth is common. Recurrences develop in about a third of patients, who experience a shorter survival (mean, 6.5 years). The following parameters, when present, suggest an increased incidence of recurrence or dying with disease: size ?4.0 cm (P = 0.034), high mitotic count (P = 0.041), atypical mitoses (P = 0.007), mixed anatomic site (P = 0.032), development of recurrence (P = 0.041), high tumor grade (P = 0.007), and higher stage disease (P = 0.027).
PubMed ID: 22183767
Article Size: 1.2 MB
 

Sinonasal tract mucosal melanoma.

Head & Neck, Sinonasal Tract
Thompson LD.
Ear Nose Throat J. 2014 Oct-Nov;93(10-11):E49-50.
FIRST PARAGRAPH: Mucosal melanoma is a neural-crest–derived neoplasm that originates in melanocytes and demonstrates melanocytic differentiation. Exposure to formalin and ultraviolet light are known etiologic factors; another possible factor is exposure to radiation. Approximately 15 to 20% of all skin melanomas develop on the head and neck, but less than 1% of all melanomas develop in ocular or mucosal sites, including the sinonasal tract.
PubMed ID: 25397394
Article Size: <1 MB

Sinonasal Tract Neurofibroma: A Clinicopathologic Series of 12 Cases with a Review of the Literature

Head & Neck, Sinonasal Tract
Azani AB, Bishop JA, Thompson LD.
Head Neck Pathol. 2015 Sep;9(3):323-33.
Neurofibroma (NF), a benign peripheral nerve sheath tumor, is very uncommon in the sinonasal tract, with only a few reported cases in the English literature. Cases within the files of the authors’ institutions confined to the sinonasal tract were compared to cases reported in the English literature (Medline 1966-2014). The 12 patients included 6 females and 6 males, aged 26-75 years (mean 46.2 years). The patients usually presented clinically with a mass lesion (n = 11), obstruction (n = 4) or pain (n = 3), with an average symptom duration of 42.9 months. Two patients had neurofibromatosis (NF1). Tumors involved the nasal cavity alone (n = 8), maxillary sinus alone (n = 2), or mixed sites (n = 2), with a range of 0.4-4.1 cm (mean 2.2 cm). The tumors were circumscribed, composed of spindled to wavy cells with curvilinear nuclei set in a background of collagenized stroma and mast cells. Nuclear palisading and perivascular hyalinization were not seen. Mitoses were scant. Pleomorphism, necrosis and increased cellularity were absent. By immunohistochemistry, the lesional cells were S100 protein, SOX10 and NFP positive, while CD34 highlighted the perineurium. INI1 was intact, with strong nuclear expression in all cases. All patients had surgical excision without recurrence (mean follow-up 8.6 years). The principle differential diagnoses include schwannoma, perineurioma, fibromatosis, and solitary fibrous tumor. NF of the sinonasal tract occurs in middleaged patients without a gender predilection, usually with non-specific symptoms present for a long duration. Tumors are relatively large (mean 2.2), and usually affect one site only. Surgery is curative, with only 16.7 % NF1 associated. S100 protein, SOX10 and NFP highlight the Schwann cells, with CD34 highlighting the perineural fibroblasts.
PubMed ID: 25503638
Article Size: 3.7 MB
 

Sinonasal tumors: a clinicopathologic update of selected tumors.

Head & Neck, Sinonasal Tract
Slootweg PJ, Ferlito A, Cardesa A, Thompson LD, Hunt JL, Strojan P, Takes RP, Triantafyllou A, Woolgar JA, Rinaldo A, Devaney KO, Barnes L.
Eur Arch Otorhinolaryngol. 2013 Jan;270(1):5-20.
The sinonasal cavities show a wide variety of neoplasms of epithelial, mesenchymal, neural/neuroectodermal or hematopoietic origin. The differential diagnosis for these tumors may be difficult due to overlapping morphologies, variable patterns in ancillary studies, and potentially confusing terminology. In this report, an updated review of the spectrum of neoplasia is provided, using the World Health Organization 2005 classification as a guide. Classic tumors that are generally limited to the sinonasal tract are described and new information regarding molecular pathogenesis is reviewed. Also new entities that have the sinonasal tract as a site of predilection, such as sinonasal renal cell-like adenocarcinoma and NUT midline carcinoma are highlighted.
PubMed ID: 22610012
Article Size: 2.2 MB
 

Sinonasal-type hemangiopericytoma: a clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation.

Head & Neck, Sinonasal Tract
Thompson LD, Miettinen M, Wenig BM.
Am J Surg Pathol. 2003 Jun;27(6):737-49.
Sinonasal-type hemangiopericytoma is an uncommon upper aerodigestive tract tumor of uncertain cellular differentiation. We report 104 cases of sinonasal-type hemangiopericytoma diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was airway obstruction (n = 57) and/or epistaxis (n = 54), with symptoms averaging 10 months in duration. The tumors involved the nasal cavity alone (n = 47) or also a paranasal sinus (n = 26), were polypoid, and measured an average of 3.1 cm. Histologically, the tumors were submucosal and unencapsulated and showed a diffuse growth with fascicular (n = 37) to solid (n = 50) to focally whorled (n = 7) patterns. The tumor cells were uniform in appearance with minimal pleomorphism and had spindle-shaped (n = 82) to round/oval (n = 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated (tumor) giant cells were identified in a minority of cases (n = 5). Mitotic figures were inconspicuous and necrosis was absent. The tumors were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n = 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n = 87). The immunohistochemical profile included reactivity with vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%). Surgery was the treatment of choice for all of the patients; adjunctive radiotherapy was given to four patients. Recurrences developed in 18 patients within 1-12 years from diagnosis. Ninety-seven patients were either alive (n = 51, mean 16.5 years) or dead (n = 46, mean 9.6 years) but free of disease. Four patients had disease at the last follow-up: three died with disease (mean 3.6 years) and one patient is alive with disease (28.3 years). Recurrent tumor (17.8%) can be managed by additional surgery. The majority of sinonasal-type hemangiopericytomas behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. Sinonasal-type hemangiopericytomas have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of glomus tumors.
PubMed ID: 12766577
Article Size: 2 MB
 
 
 
 

Solitary fibrous tumor of the larynx.

Head & Neck, Larynx
Thompson LDR, Karamurzin Y, Wu ML, Kim JH.
Head Neck Pathol. 2008 Jun;2(2):67-74.
BACKGROUND: True mesenchymal, non-cartilaginous neoplasms of the larynx are rare. Extrapleural solitary fibrous tumor (SFT) is a localized neoplasm characterized by proliferation of thin-walled vessels and collagen-producing cells and is considered within the ‘hemangiopericytoma-solitary fibrous tumor’ spectrum. SFT primary in the larynx is exceptional.
DESIGN: Case report set in a comparison with other cases reported in the English literature (MEDLINE 1966 to 2007).
RESULTS: A 49-year old white male presented with difficulty breathing, progressive over the past 2 years. He denied dysphagia and weight loss. Past medical history was significant for asthma. He denied cigarette smoking or alcohol abuse. There were no cervical deformities on physical exam. Fiberoptic laryngoscopy was performed upon stabilization of respiratory function. A smooth, round, submucosal mass measuring 2.3 cm in greatest diameter arising from the inferior surface of left true vocal cord was causing near total obstruction of the endolaryngeal space. The mass was excised. The surface mucosa was intact and unremarkable. A cellular, spindle cell neoplasm was arranged in loose fascicles, associated with heavy collagen fiber deposition. The collagen was wiry and heavy. Cells were bland with cytoplasmic extensions. The nuclei were vesicular to hyperchromatic and elongated with inconspicuous nucleoli. Vessels were prominent and delicate, with patulous spaces. Mitotic figures were easily identified, but atypical forms were not present. The cells were strongly and diffusely immunoreactive with CD34 and bcl-2, while non-reactive with cytokeratin, EMA, actin, ALK-1, S100, desmin, and CD117. These findings confirmed a diagnosis of extraplural solitary fibrous tumor. Without further disease, the patient is alive without evidence of disease, 12 months after surgery. Conclusions The characteristic histologic pattern of solitary fibrous tumor can be noted in extrapulmonary locations. Development in the larynx is uncommon, but the tumor presents as a polypoid mass with characteristic histologic and immunophenotypic features. Conservative local excision is the treatment of choice to yield an excellent prognosis.
CONCLUSIONS: The characteristic histologic pattern of solitary fibrous tumor can be noted in extrapulmonary locations. Development in the larynx is uncommon, but the tumor presents as a polypoid mass with characteristic histologic and immunophenotypic features. Conservative local excision is the treatment of choice to yield an excellent prognosis.
PubMed ID: 20614325
Article Size: <1 MB
 

Spindle cell (sarcomatoid) carcinomas of the larynx: a clinicopathologic study of 187 cases.

Head & Neck, Larynx
Thompson LD, Wieneke JA, Miettinen M, Heffner DK.
Am J Surg Pathol. 2002 Feb;26(2):153-70.
Laryngeal spindle cell (sarcomatoid) carcinomas are uncommon tumors, frequently misdiagnosed as reactive lesions or mesenchymal malignancies. The records of 187 patients with tumors diagnosed as laryngeal spindle cell (sarcomatoid) carcinoma were retrieved from the files of the Otorhinolaryngic Tumor Registry of the Armed Forces Institute of Pathology. There were 174 men and 13 women, 35-92 years of age (average, 65.6 years). Nearly all patients experienced hoarseness (n = 165 [88%] patients) for a mean duration of 11.0 months. Patients admitted to smoking (n = 162 [87%] patients) and/or alcohol use (n = 90 [48%] patients). Most tumors were glottic (n = 132 [71%]), T1 (n = 111 [59%]), 1 and polypoid (n = 185 [99%]), with a mean tumor size of 1.8 cm. Histologically, squamous cell carcinoma (n = 157 [84%]) was noted, ulcerated, and blended with the spindle cell component, which was most frequently arranged in a storiform pattern (n = 92 [49%] tumors). Foci of benign or malignant cartilage and/or bone (n = 13 [7%]) were noted in the spindle cell component. All patients were treated with surgery (n = 90 [48%] patients) or surgery with radiation (n = 97 [52%] patients). Recurrences developed in 85 (45%) patients. Overall, T1 glottic tumors managed by complete surgical eradication had the best outcome (mean follow-up, 7.8 years).
PubMed ID: 11812937
Article Size: <1 MB
 

Spindle Cell Lipoma of the Tongue: A Clinicopathologic Study of 8 Cases and Review of the Literature.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Lau SK, Bishop JA, Thompson LD.
Head Neck Pathol. 2015 Jun;9(2):253-9.
Spindle cell lipoma is a histologically distinct variant of lipoma characteristically arising in the subcutis of the posterior neck, upper back, or shoulder. Spindle cell lipomas infrequently occur within the oral cavity and, in particular, rarely involve the tongue. The clinical and pathologic features of eight cases of spindle cell lipoma affecting the tongue were analyzed. The study group included five men and three women ranging in age from 35 to 80 years (mean 57.4 years). Most lesions presented as either a painless or slowly growing lingual mass. The tumors were well circumscribed and characterized microscopically by a mixture of mature adipocytes, cytologically bland spindle cells, and interspersed bundles of thick collagen fibers in variable proportions. Myxoid stroma was a prominent feature in three lesions. The spindle cells were positive with CD34, while negative with S-100 protein, desmin, and smooth muscle actin. Treatment consisted of local excision in all cases. There have been no recurrences to date, with clinical follow up information available for all patients (range 11-118 months; mean 50.8 months). Lingual examples of spindle cell lipoma should be distinguished from other fat containing spindle cell neoplasms that can arise at this anatomic site.
PubMed ID: 25319950
Article Size: 2 MB
 

Squamous cell carcinoma variants of the head and neck.

Head & Neck, Sinonasal Tract
Thompson LDR.
Curr Diag Pathol 2003 9, 384–396.
Variants of squamous cellcarcinoma (SCC) frequently arise within the mucosa of the upper aerodigestive tract, accounting for up to15% of SCCs in these areas. The most common variants include verrucous, exophytic or papillary, spindle-cell (sarcomatoid), basaloid and adenosquamous carcinoma. Each of these variants has a unique histomorphologic appearance, which raises a number of different differential diagnostic considerations, with the attendant clinically relevant management decision.
Verrucous squamous cell carcinoma has a broad border of pushing infiltration of a non-dysplastic squamous epithelium, essentially devoid of mitotic figures, displaying hyperkeratosis on elongated rete pegs. Papillary and exophytic SCC have a papillary or exophytic architecture, but have malignant cytologic features within the epithelium. Spindle-cell (sarcomatoid) carcinoma is an SCC blended with a spindle-cell morphology, frequently mimicking other mesenchymal tumours. Epithelial markers are often negative. Basaloid SCC is a high-grade SCC variant with small cells arranged in a palisaded architecture, with hyperchromatic nuclei and only focal areas of squamous differentiation. A denosquamous carcinoma is a rare variant, which is a composite of adenocarcinoma and squamous cell carcinoma, often with areas of transition.The cytomorphologic features are described in detail in an attempt to allow the general surgical pathologist to separate these variants of SCC in order to achieve appropriate clinical management.
PubMed ID: n/a
Article Size: 3 MB

Synovial sarcoma.

Head & Neck, Neck
Folk GS, Thompson LD.
Ear Nose Throat J. 2006 Jul;85(7):418-9.
FIRST PARAGRAPH: Synovial sarcoma (SS) is a unique tumor that usually involves the large joints. Approximately 10% of these tumors develop in the neck, oropharynx, hypopharynx, and larynx. Despite the name, these tumors neither originate from synovium nor express synovial differentiation. SS typically presents in young adults (median age: 25 yr). The male-to-female ratio is 3:1. Symptoms are site-specific but tend to be nonspecific. SS typically appears as a solitary, painless mass, occasionally accompanied by dyspnea or hoarseness. The diameter of the lesion ranges from 1 to 12 cm. The cut surface is yellow, firm, whorled, gritty, and friable; cyst formation with hemorrhage or mucoid material is often seen.
PubMed ID: 16909806
Article Size: <1 MB

Temporal bone schwannoma.

Ear & Temporal Bone, Head & Neck
Thompson L.
Ear Nose Throat J. 2006 Nov;85(11):704.
FIRST PARAGRAPH: A schwannoma (acoustic neuroma or neurilemmoma) is a globular, firm, tan-yellow, solid to cystic neoplasm of nerve sheath (Schwann) cells. Schwannomas are the most common neoplasms of the ear and temporal bone. The vast majority arise at the cerebropontine angle, and 95% are unilateral and sporadic. Bilateral schwannomas and those that arise in young patients are highly associated with neurofibromatosis type 2 (NF2). Schwannomas affect men and women equally, usually in the fifth or sixth decade of life except in patients with NF2, who generally present at a younger age.
PubMed ID: 17168142
Article Size: <1 MB

Teratoma.

Head & Neck, Neck
Thompson L.
Ear Nose Throat J. 2005 Feb;84(2):75.
FIRST PARAGRAPH: Teratomas are neoplasms made up of tissues foreign to the site of occurrence. They contain tissue from all three embryonic germ layers (endoderm, mesoderm, and ectoderm). Other terms–choristoma, hamartoma, heterotopia, epignathus, and dermoid–refer to separate, unique entities that are not covered here.
PubMed ID: 15794539
Article Size: <1 MB

The clinical importance of cystic squamous cell carcinomas in the neck: a study of 136 cases.

Head & Neck, Oropharynx
Thompson LD, Heffner DK.
Cancer. 1998 Mar 1;82(5):944-56.
BACKGROUND: Predominantly cystic squamous cell carcinomas in the neck often present without a clinically apparent primary and therefore are frequently considered to be of branchial cleft origin. It is the authors’ hypothesis that the anatomic site of the primary carcinoma that produced the neck metastasis can often be predicted on the basis of the histologic features.
METHODS: Cases of cystic squamous cell carcinoma in the neck diagnosed between 1971 and 1991 were retrieved from the Otorhinolaryngic Pathology Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed and patient follow-up was obtained and analyzed.
RESULTS: In cases wherein the primary site was discovered subsequently, 64% of the primaries were in the lingual or faucial tonsil. An additional 8% of cases were in nasopharyngeal tonsillar tissue. The cases that did not originate in Waldeyer’s tonsillar ring generally differed in histologic appearance from the tonsillar cases. The tonsillar primaries were discovered within an average of 12.4 months, but many were not discovered for years (up to 11 years). Most were small, indicating a slower growth of the primary than is usually expected for squamous cell carcinoma. Patients with such carcinomas had a much better prognosis than patients with metastatic squamous cell carcinomas of other upper airway mucosal sites.
CONCLUSIONS: In most cases of prominently cystic squamous cell carcinomas in the upper neck, the origin of the primary site will be in faucial or lingual tonsillar crypt epithelium. Knowledge of the probable site of origin allows for more tailored therapy in which the patients can be treated relatively conservatively with surgical excision and subsequent field-limited radiation therapy only, with 77% survival at 5 years. None of the cases reviewed in this study was a branchiogenic carcinoma.
PubMed ID: 9486586
Article Size: 2 MB
 

The importance of histological types for treatment and prognosis in laryngeal cancer.

Head & Neck, Larynx
Ferlito A, Thompson LDR, Cardesa A, Gnepp DR, Devaney KO, Rodrigo JP, Hunt JL, Rinaldo A, Takes RP.
Eur Arch Otorhinolaryngol. 2013 Feb;270(2):401-3
FIRST PARAGRAPH: The classification of neoplasms is an important matter, with correlation of a tumor’s type with its biological behavior. To provide internationally acceptable criteria for the histological diagnosis of tumors, the World Health Organization (WHO) published an updated Pathology and Genetics of Head and Neck Tumours Classification of Tumours in 2005, which included tumors of the hypopharynx, larynx and trachea.
PubMed ID: 23315201
Article Size: <1 MB
 

The sinonasal tract: Another potential ‘hot spot’ for carcinomas with transcriptionally-active human papillomavirus.

Head & Neck, Sinonasal Tract
Lewis JS Jr, Westra WH, Thompson LD, Barnes L, Cardesa A, Hunt JL, Williams MD, Slootweg PJ, Triantafyllou A, Woolgar JA, Devaney KO, Rinaldo A, Ferlito A.
Head Neck Pathol. 2014 Sep;8(3):241-249.
While high risk human papillomavirus (HPV) is well established as causative and clinically important for squamous cell carcinoma (SCC) of the oropharynx, its role in non-oropharyngeal head and neck SCC is much less clearly elucidated. In the sinonasal region, in particular, although it is a relatively uncommon site for SCC, as many as 20 % of SCC harbor transcriptionally-active high risk HPV. These tumors almost always have a nonkeratinizing morphology and may have a better prognosis. In addition, specific variants of SCC as well as other rare carcinoma types, when arising in the sinonasal tract, can harbor transcriptionally-active HPV. This article reviews the current literature on HPV in sinonasal carcinomas, attempts to more clearly demonstrate what tumors have it and how this relates to possible precursor lesions like inverted papilloma, and discusses the possible clinical ramifications of the presence of the virus.
PubMed ID: 24338611
Article Size: <1 MB
 

Tonsil with Tangier disease.

Head & Neck, Oropharynx
Nelson BL, Thompson LD.
Ear Nose Throat J. 2003 Mar;82(3):178.
FIRST PARAGRAPH: Tangier disease is a rare autosomal-recessive inherited disorder that is caused by a defect in chromosome 9q31. It is characterized by a severe deficiency or absence of high-density lipoproteins in plasma. A defect in cellular cholesterol removal results in the massive, abnormal accumulation of cholesterol esters in macrophages in many tissues. Although this accumulation is most conspicuous in the tonsils, progressive accretion of these esters also occurs in nerves (neuropathy) and vessels (atherosclerosis). The pathognomonic finding is a low plasma cholesterol concentration accompanied by normal or elevated triglyceride levels and large, lobulated, hyperplastic, bright orange-yellow tonsils and adenoid tissue. Affected families have been identified on Tangier Island, Va., in Chesapeake Bay as well as in Missouri, Kentucky, and Europe.
PubMed ID: 12696235
Article Size: <1 MB

Tonsillar lymphangiomatous polyps: a clinicopathologic series of 26 cases.

Head & Neck, Oropharynx
Kardon DE, Wenig BM, Heffner DK, Thompson LD.
Mod Pathol. 2000 Oct;13(10):1128-33.
BACKGROUND: Lymphangiomatous polyps are uncommon benign tumors of the tonsils.
METHODS: Twenty-six cases of lymphangiomatous polyps diagnosed between 1980 and 1999 were retrieved from the files of the Otorhinolaryngic-Head and Neck Tumor Registry of the Armed Forces Institute of Pathology. Hematoxylin and eosin-stained slides were reviewed to characterize the histologic features of these tumors. Immunohistochemical stains were performed on 15 cases. Clinical follow-up data were obtained.
RESULTS: The patients included 13 males and 13 females, ages 3 to 63 years (mean, 25.2 years). Patients experienced dysphagia, sore throat, and the sensation of a mass in the throat. Symptoms were present from a few weeks to years. The tonsillar masses were unilateral in all cases. Clinically, the lesions were frequently mistaken for a neoplasm (n = 18 patients). Grossly, all of the lesions were polypoid and measured 0.5 to 3.8 cm (mean, 1.6 cm). Histologically, the polyps were covered by squamous epithelium showing variable epithelial hyperplasia, dyskeratosis, and lymphocytic epitheliotropism. The masses showed a characteristic submucosal proliferation of small to medium-sized, endothelial-lined, lymph-vascular channels lacking features of malignancy. Collagen, smooth muscle, and adipose tissue were present in the stroma. Intravascular proteinaceous fluid and lymphocytes were noted. Immunohistochemical findings confirmed the endothelial origin of the vascular proliferation and a mixed lymphoid population. The differential diagnosis included fibroepithelial polyp, lymphangioma, juvenile angiofibroma, and squamous papilloma. In all patients with follow-up, complete surgical excision was curative (mean follow-up, 5.4 years; range, 1 mo to 14 years).
CONCLUSIONS: We detail the clinical and pathologic features of tonsillar lymphangiomatous polyps. These tumors are uncommon and may clinically be mistaken for a malignant neoplasm. The characteristic histologic features should allow for its correct diagnosis and differentiation from similar appearing tonsillar lesions.
PubMed ID: 11048808
Article Size: 2.5 MB