Keratocystic odontogenic tumor.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Thompson LD.
Ear Nose Throat J. 2014 Sep;93(9):386-8.
FIRST PARAGRAPH: Keratocystic odontogenic tumor (KCOT; formerly known as odontogenic keratocyst) is a distinct developmental odontogenic cyst that may be locally aggressive and may be part of the nevoid basal cell carcinoma syndrome (NBCCS, or Gorlin syndrome). Inherited as an autosomal dominant trait, there is high penetrance, although with variable expression, associated with loss of function of the PTCH gene (chromosome 9q22.3-q31), a tumor suppressor gene.
PubMed ID: 25255344
Article Size: <1 MB

Parathyroid adenoma.

Endocrine, Parathyroid
Thompson LD.
Ear Nose Throat J. 2014 Jul;93(7):246-68.
FIRST PARAGRAPH: A parathyroid adenoma is a benign neoplasm of parathyroid parenchymal cells. There is an association with the HRPT2 gene (1q25-q31), which is associated with hyperparathyroidism-jaw tumor syndrome (an autosomal dominant disorder). Parathyroid adenoma is the single most common cause of hyperparathyroidism. It is usually seen in patients in the fourth and fifth decades of life, and women are affected more often than men by a margin of 3 or 4 to 1.
PubMed ID: 25025408
Article Size: <1 MB

Chronic lymphocytic thyroiditis (Hashimoto thyroiditis).

Endocrine, Thyroid
Thompson LD.
Ear Nose Throat J. 2014 Apr-May;93(4-5):152-3.
FIRST PARAGRAPH: Chronic lymphocytic thyroiditis, eponymically referred to as Hashimoto thyroiditis (named after Hakarum Hashimoto, who first described the disease in 1912), is an autoimmune thyroiditis that results from autoantibodies to thyroid-specific antigens. There is a poorly understood interaction between susceptibility genes and environmental triggers, resulting in antimicrosomal and antithyroglobulin antibodies. Many patients may have another simultaneous autoimmune disease (such as Sjögren syndrome, diabetes mellitus, or myasthenia gravis), while there is some overlap with Graves disease (diffuse hyperplasia). Hypothyroidism may also result from destruction of the thyroid epithelium by inflammatory cells.
PubMed ID: 24817227
Article Size: <1 MB

Merkel cell carcinoma.

Other Anatomic Sites, Skin
Shiffer JD, Thompson LD.
Ear Nose Throat J. 2014 Mar;93(3):100-2.
FIRST PARAGRAPH: Merkel cell carcinoma is a rare and highly aggressive cutaneous neuroendocrine carcinoma that, in most cases, is caused by Merkel cell polyomavirus (approximately 80% of cases). The tumor usually arises on sun-exposed skin of elderly patients, particularly on the head and neck and extremities. There is a slight female preponderance and an increased risk in individuals who are immunosuppressed. Clinically, the tumors are often indistinguishable from other skin cancers and typically present as a firm, painless, rapidly growing nodule. They may be flesh-colored, red, or blue tumors that typically vary in size from 0.5 cm to more than 5 cm (average, 2 cm). When they have a reddish nodular appearance, they may be mistaken for angiosarcoma or granulation tissue.
PubMed ID: 24652557
Article Size: <1 MB

Canalicular Adenoma: A Clinicopathologic and Immunohistochemical Analysis of 67 Cases with a Review of the Literature

Head & Neck, Salivary Gland
Thompson LD, Bauer JL, Chiosea S, McHugh JB, Seethala RR, Miettinen M, Müller S.
Head Neck Pathol. 2015 Jun;9(2):181-95.
There is a lack of a comprehensive immunohistochemical (IHC) analysis of canalicular adenoma (CanAd), especially when combined with a description of the unique histologic features. Given the usual small biopsies, IHC may be useful in distinguishing CanAd from other tumors in the differential diagnosis. Retrospective. The patients included 54 females and 13 males (4.2:1), aged 43-90 years, with a mean age at presentation of 69.9 years. Clinical presentation was generally a mass (n = 61) slowly increasing in size (mean 38.5 months), affecting the upper lip (n = 46), buccal mucosa (n = 17) or palate (n = 4), involving the right (n = 29), left (n = 24) or midline (n = 9), without any major salivary gland tumors. The tumors ranged in size from 0.2 to 3 cm (mean 1.2 cm). Most tumors were multilobular or bosselated (76 %), often surrounded by a capsule. Histologically, the tumors were characterized by cystic spaces, tumor cords with beading, tubule formation, and by the presence of luminal squamous balls (n = 41). The cells were cuboidal to columnar with stippled chromatin. Mitoses were inconspicuous. A myxoid stroma (n = 64), sclerosis (n = 42), luminal hemorrhage (n = 51), and luminal microliths (calcifications) (n = 33) were characteristic. Nine (13.4 %) were multifocal. CanAd showed the following characteristic immunohistochemistry findings: CK-pan and S100 protein (strong, diffuse reaction); peripheral or luminal GFAP reaction; CK5/6 and p16 luminal squamous ball reaction; SOX10 nuclear reaction; cytoplasmic p63 reaction. CanAd are unique minor salivary gland tumors showing a distinct architecture and phenotype. They predilect to older women, with the majority multilobulated and affecting the upper lip, multifocal in 13 %; no major salivary gland tumors were identified. S100 protein, CK-pan, GFAP and SOX10 are positive, with luminal squamous balls highlighted by CK5/6 or p16.
PubMed ID: 25141970
Article Size: 4.5 MB
 
 
 
 

Oncocytic Lipoadenoma of the Salivary Gland: A Clinicopathologic Analysis of 7 Cases and Review of the Literature

Head & Neck, Salivary Gland
Lau SK, Thompson LD.
Head Neck Pathol. 2015 Mar;9(1):39-46.
Oncocytic lipoadenoma is an exceedingly uncommon neoplasm of the salivary gland composed of oncocytic epithelium and adipose tissue. Retrospective. Seven cases of oncocytic lipoadenoma were analyzed in order to further characterize the clinical and pathologic features of this rare tumor. The patients included six males and one female who ranged from 40 to 83 years of age (mean 62 years) at presentation. All tumors arose in the parotid gland. Grossly, the tumors were solitary, well circumscribed and had light brown to yellow cut surfaces. Histologically, the tumors were composed of an admixed population of oncocytes and adipocytes in varying proportions, with the lipomatous component ranging from 5 to 70 %. Other common features included the presence of serous acini, ductal elements, sebaceous glands, and a patchy chronic inflammation. Clinical follow up information, available in all cases, with a duration of 3-148 months (mean 57 months), showed no evidence of tumor recurrence. Due to its rarity, oncocytic lipoadenoma can pose problems in diagnosis, although the distinctive morphologic features of this neoplasm allow for separation from more commonly recognized oncocytic neoplasms of the salivary glands.
PubMed ID: 24737102
Article Size: 2.2 MB
 

Molecular diagnostic alterations in squamous cell carcinoma of the head and neck and potential diagnostic applications.

Head & Neck, Larynx
Hunt JL, Barnes L, Lewis JS Jr, Mahfouz ME, Slootweg PJ, Thompson LD, Cardesa A, Devaney KO, Gnepp DR, Westra WH, Rodrigo JP, Woolgar JA, Rinaldo A, Triantafyllou A, Takes RP, Ferlito A.
Eur Arch Otorhinolaryngol. 2014 Feb;271(2):211-23
Head and neck squamous cell carcinoma (HNSCC) is a common malignancy that continues to be difficult to treat and cure. In many organ systems and tumor types, there have been significant advances in the understanding of the molecular basis for tumorigenesis, disease progression and genetic implications for therapeutics. Although tumorigenesis pathways and the molecular etiologies of HNSCC have been extensively studied, there are still very few diagnostic clinical applications used in practice today. This review discusses current clinically applicable molecular markers, including viral detection of Epstein-Barr virus and human papillomavirus, and molecular targets that are used in diagnosis and management of HNSCC. The common oncogenes EGFR, RAS, CCND1, BRAF, and PIK3CA and tumor suppressor genes p53, CDKN2A and NOTCH are discussed for their associations with HNSCC. Discussion of markers with potential future applications is also included, with a focus on molecular alterations associated with targeted therapy resistance.
PubMed ID: 23467835
Article Size: <1 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
 

Epstein-Barr virus and human herpes virus-8 are not associated with juvenile nasopharyngeal angiofibroma.

Head & Neck, Sinonasal Tract
Carlos R, Thompson LD, Netto AC, Pimenta LG, Correia-Silva Jde F, Gomes CC, Gomez RS.
Head Neck Pathol. 2008 Sep;2(3):145-9.
BACKGROUND: Nasopharyngeal angiofibroma (also known as juvenile nasopharyngeal angiofibroma) is a rare fibroblastic tumor with a vascular component that occurs in the nasopharynx and posterolateral nasal wall of adolescent boys. The etiology of nasopharyngeal angiofibroma remains elusive. This investigation was undertaken to determine if human herpes simplex virus-8 and Epstein-Barr virus are possible etiologic viruses and to determine if they have any association with the age of the patient and/or the proliferative state of the lesion.
MATERIALS AND METHODS: Formalin fixed, routinely processed, and paraffin embedded surgical specimens of 15 angiofibromas were submitted to PCR for EBV and HHV-8, while in situ hybridization was also employed for EBV. Immunohistochemical analysis for ki-67 was performed using MIB immunostaining.
RESULTS: None of the tumors were positive for HHV-8. The PCR technique produced a false positive reaction in five cases, with all cases non-reactive with EBV-ISH. The age of the patients did not show correlation with the Ki-67 labeling index.
CONCLUSION: Angiofibroma does not appear to be associated with either HHV-8 or EBV, thereby excluding these viruses as potential etiologic agents. The lack of a correlation between the proliferative index and the age of the patient suggests the proposed puberty induced, testosterone-dependent tumor growth may not play a significant role in tumor development.
PubMed ID: 20614308
Article Size: <1 MB
 

Intradural, extramedullary spinal cord granular cell tumor: a case report and clinicopathologic review of the literature.

Neuro (brain), Other Anatomic Sites
Weinstein BJ, Arora T, Thompson LD.
Neuropathology. 2010 Dec;30(6):621-6.
Granular cell tumor (GCT) of the spine is uncommon, with intradural extramedullary location being exceptionally rare. The non-specific clinical presentation and variable histologic patterns can make recognition of this tumor challenging. Two previous reports of GCT of the spine were reviewed (Medline 1960-2009) and analyzed with respect to this case report. The patients included two women and one man (mean age, 28.7 years). Patients presented with 3 to 4 months of lower back pain and/or lower extremity radiculopathy. The lesions appeared radiographically to be intradural and extramedullary or intramedullary. The tumors were found at T10 or L1-L2 space. Radiographically, all tumors enhanced homogenously on T1 post-gadolinium imaging with a mean tumor size of approximately 1.6 cm. Histologically, the tumors were composed of large, polygonal granular cells. The abundant cytoplasm was fine or coarsely granular, surrounding small, pale-staining nuclei, which were eccentrically located in the cell. The tumor cells were periodic acid Schiff positive, diastase resistant, and were positive with S-100 protein, CD68, inhibin, and neuron-specific enolase immunohistochemistry. The clinical and histologic differential diagnosis includes schwannoma, neurofibroma, meningioma, astrocytoma, melanocytoma, and metastatic tumors. Patients were managed with excision. One patient had symptomatic and radiographic local recurrence that was subsequently treated with radiation, resulting in stabilization of disease and symptoms. Intradural GCTs of the spine are rare and radiographically indistinguishable from tumors that more commonly arise in this location. Histologic recognition of this rare tumor is important because the subsequent clinical course of the disease differs from other similar lesions.
PubMed ID: 20113407
Article Size: 1 MB
 

Middle ear adenomas stain for two cell populations and lack myoepithelial cell differentiation.

Ear & Temporal Bone, Head & Neck
Lott Limbach AA, Hoschar AP, Thompson LD, Stelow EB, Chute DJ.
Head Neck Pathol. 2012 Sep;6(3):345-53.
Middle ear adenomas (MEAs) are benign neoplasms along a spectrum with neuroendocrine neoplasms (carcinoid tumors). Immunohistochemical (IHC) staining for myoepithelial markers has not been reported in these tumors. The archives of the Cleveland Clinic, University of Virginia and Armed Forces Institute of Pathology were retrospectively searched for tumors arising within the middle ear with material available for IHC staining. Twelve cases of MEAs, four cases of jugulotympanic paragangliomas (JPGs), 10 cases of ceruminous adenomas (CAs) and four cases of ceruminous adenocarcinomas (CACs) were obtained. IHC staining was performed for smooth muscle actin (SMA), p63, S-100 protein, cytokeratin 5/6 (CK5/6), and cytokeratin 7 (CK7). The MEAs were positive for: CK7 (92 %, luminal), CK5/6 (92 %, abluminal), p63 (83 %, abluminal), and negative for SMA and S-100 protein. The JPGs were negative for CK7, CK5/6, p63 and SMA; S-100 protein highlighted sustentacular cells. The CAs were positive for: CK7 (100 %, luminal), CK5/6 (100 %, abluminal), S-100 protein (80 %, abluminal), p63 (100 %, abluminal), and SMA (90 %, abluminal). CACs demonstrated two patterns, (1) adenoid cystic carcinoma-type: positive for CK7 (100 %, luminal), CK5/6, S-100 protein, p63, and SMA (all 100 %, abluminal); and (2) conventional-type: CK7 (50 % luminal), and no CK5/6, SMA, S-100 protein, or p63 expression. The IHC profile of MEAs suggests that these tumors harbor at least two cell populations, including luminal and basal cells. However, unlike ceruminous adenomas, MEAs lack true myoepithelial differentiation given the absence of S-100 protein and SMA staining in all cases.
PubMed ID: 22623086
Article Size: 1 MB
 

Oral Alveolar Soft Part Sarcoma in Childhood and Adolescence: Report of Two Cases and Review of Literature.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Argyris PP, Reed RC, Manivel JC, Lopez-Terrada D, Jakacky J, Cayci Z, Tosios KI, Pambuccian SE, Thompson LD, Koutlas IG.
Head Neck Pathol. 2013 Mar;7(1):40-9.
Alveolar soft part sarcoma (ASPS) constitutes a rare soft tissue malignant neoplasm comprising less than 1 % of all soft tissue sarcomas. ASPS demonstrates a strong predilection for adolescents and young adults, with a female predominance reported. The head and neck region is the most commonly affected region in pediatric patients with the tongue and orbit affected most commonly. Herein we present the clinical, radiographic, histopathologic, immunohistochemical and molecular features of two examples of ASPS affecting the oral cavity of 4 and 13 year-old boys, along with a focused review of the literature on intraoral ASPS in pediatric patients.
PubMed ID: 22961078
Article Size: <1 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
 

Oral lichen planus.

Head & Neck, Sinonasal Tract
Thompson LD.
Ear Nose Throat J. 2012 Mar;91(3):102-4.
FIRST PARAGRAPHS: The cause of lichen planus is not known. Its treatment depends on the specific type–reticular, erosive, or bullous–and usually includes topical or systemic corticosteroids and topical antifungal agents. Patients require lifelong monitoring and/or therapy. Lichen planus (LP) is a chronic, self-limited, inflammatory disorder of unknown etiology that involves mucous membranes, skin, nails, and hair. It is postulated that there is an abnormal T-cell-mediated immune response that results in disruption of the basement membrane. Several drugs are known to be associated with the onset of LP, but the exact mechanism is unknown.
PubMed ID: 22430334
Article Size: MB

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

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

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
 

Orbital infantile myofibroma: a case report and clinicopathologic review of 24 cases from the literature.

Other Anatomic Sites, Soft Tissue
Mynatt CJ, Feldman KA, Thompson LD.
Head Neck Pathol. 2011 Sep;5(3):205-15.
Isolated orbital infantile myofibroma are rare tumors in the head and neck. The mass-like clinical presentation and variable histologic features result in frequent misdiagnosis and potentially inappropriate clinical management. There are only a few reported cases in the English literature. Twenty-four patients with orbital infantile myofibroma or myofibromatosis were compiled from the English literature (Medline 1960–2011) and integrated with this case report. The patients included 14 males and 10 females, aged newborn to 10 years (mean, 34.8 months), who presented with a painless mass in the infra- or supraorbital regions, usually increasing in size andassociated with exophthalmos (n = 5). Females were on average older than their male counterparts (38.9 vs. 31.9 months, respectively; P = 0.71). The tumors were twice as frequent on the left (n = 16) than right (n = 8). Patients experienced symptoms for an average of 2.7 months before clinical presentation. The tumors involved the bone (n = 17) or the soft tissues (n = 7) of the orbit, with extension into the nasal or oral cavity (n = 3). The mean size was 3.0 cm, with a statistically significant difference between males and females (mean: 3.9 vs. 1.82; P = 0.0047), but without any differences based on age at presentation (P = 0.25), duration of symptoms (P = 0.66), or bone or soft tissue involvement (P = 0.51). Grossly, all tumors were wellcircumscribed, firm to rubbery, homogenous, and white– grey. Histologically, the tumors were biphasic, showing whorled and nodular areas of fusiform cells with extracellular collagen, mixed with a population of small, primitiveappearing, darkly staining cells. Necrosis was not present, but mitoses could be seen. Tumors with immunohistochemistry performed showed strong and diffuse smooth muscle actin and vimentin immunoreactivity, but were negative with muscle specific actin, desmin, MYOD1, myogenin, S100 protein, GFAP, keratin, CD31, 34, Factor VIIIR-Ag, and CD45RB. The principle histologic differential diagnosis includes juvenile hyaline fibromatosis, fibrous hamartoma of infancy, fibromatosis coli, leiomyoma, infantile hemangiopericytoma, infantile fibrosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, and lymphoma. All patients were managed with surgery. Recurrences developed in two patients at 4 and 6 months, respectively. Follow-up data was available on all but two patients (n = 22). These patients were either alive without evidence of disease (n = 18), alive but with disease (n = 3), or had died unrelated to this disease (i.e., neuroblastoma, n = 1). Orbital infantile myofibroma is a rare tumor, presenting in infancy as an enlarging mass of the orbit, with characteristic histomorphologic and immunophenotypic features. Orbital disease is usually isolated rather than part of systemic disease, and shows an excellent long-term prognosis, making appropriate separation from other conditions important.
PubMed ID: 21512784
Article Size: 2.5 MB
 

Ectopic sphenoid sinus pituitary adenoma (ESSPA) with normal anterior pituitary gland: A clinicopathologic and immunophenotypic study of 32 cases with a comprehensive review of the English literature.

Endocrine, Pituitary
Thompson LD, Seethala RR, Müller S.
Head Neck Pathol. 2012 Mar;6(1):75-100.
Ectopic sphenoid sinus pituitary adenoma (ESSPA) may arise from a remnant of Rathke’s pouch. These tumors are frequently misdiagnosed as other neuroendocrine or epithelial neoplasms which may develop in this site (olfactory neuroblastoma, neuroendocrine carcinoma, sinonasal undifferentiated carcinoma, paraganglioma, melanoma). Thirty-two patients with ESSPA identified in patients with normal pituitary glands (intact sella turcica) were retrospectively retrieved from the consultation files of the authors’ institutions. Clinical records were reviewed with follow-up obtained. An immunohistochemical panel was performed on available material. Sixteen males and 16 females, aged 2-84 years (mean, 57.1 years), presented with chronic sinusitis, headache, obstructive symptoms, and visual field defects, although several were asymptomatic (n = 6). By definition, the tumors were centered within the sphenoid sinus and demonstrated, by imaging studies or intraoperative examination, a normal sella turcica without a concurrent pituitary adenoma. A subset of tumors showed extension into the nasal cavity (n = 5) or nasopharynx (n = 9). Mean tumor size was 3.4 cm. The majority of tumors were beneath an intact respiratory epithelium (n = 22), arranged in many different patterns (solid, packets, organoid, pseudorosette-rosette, pseudopapillary, single file, glandular, trabecular, insular). Bone involvement was frequently seen (n = 21). Secretions were present (n = 16). Necrosis was noted in 8 tumors. The tumors showed a variable cellularity, with polygonal, plasmacytoid, granular, and oncocytic tumor cells. Severe pleomorphism was uncommon (n = 5). A delicate, salt-and-pepper chromatin distribution was seen. In addition, there were intranuclear cytoplasmic inclusions (n = 25) and multinucleated tumor cells (n = 18). Mitotic figures were infrequent, with a mean of 1 per 10 HPFs and a <1% proliferation index (Ki-67). There was a vascularized to sclerotic or calcified stroma. Immunohistochemistry highlighted the endocrine nature of the tumors, with synaptophysin (97%), CD56 (91%), NSE (76%) and chromogranin (71%); while pan-cytokeratin was positive in 79%, frequently with a dot-like Golgi accentuation (50%). Reactivity with pituitary hormones included 48% reactive for 2 or more hormones (plurihormonal), and 33% reactive for a single hormone, with prolactin seen most frequently (59%); 19% of cases were non-reactive. The principle differential diagnosis includes olfactory neuroblastoma, neuroendocrine carcinoma, melanoma, and meningioma. All patients were treated with surgery. No patients died from disease, although one patient died with persistent disease (0.8 months). Surgery is curative in the majority of cases, although recurrence/persistence was seen in 4 patients (13.8%). In conclusion, ESSPAs are rare, affecting middle aged patients with non-specific symptoms, showing characteristic light microscopy and immunohistochemical features of their intrasellar counterparts. When encountering a tumor within the sphenoid sinus, ectopic pituitary adenoma must be considered, and pertinent imaging, clinical, and immunohistochemical evaluation undertaken to exclude tumors within the differential diagnosis. This will result in accurate classification, helping to prevent the potentially untoward side effects or complications of incorrect therapy.
PubMed ID: 22430769
Article Size: 4 MB
 

Pilomatricoma.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2012 Jan;91(1):18-20.
FIRST PARAGRAPH: Pilomatricoma, also referred to as pilomatrixoma and calcifying epithelioma of Malherbe, is a benign dermal-subcutaneous tumor derived from the matrix of the hair follicle. Its development is associated with a known mutation in the CTNNB1 gene, the gene that encodes for beta-catenin. Pilomatricomas are relatively common tumors. They usually arise during the first 2 decades of life, and they have no predilection for either sex.
PubMed ID: 22278863
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
 

Biomarkers predicting malignant progression of laryngeal epithelial precursor lesions: a systematic review.

Head & Neck, Larynx
Rodrigo JP, García-Pedrero JM, Suárez C, Takes RP, Thompson LD, Slootweg PJ, Woolgar JA, Westra WH, Brakenhoff RH, Rinaldo A, Devaney KO, Williams MD, Gnepp DR, Ferlito A.
Eur Arch Otorhinolaryngol. 2012 Apr;269(4):1073-83
Some laryngeal epithelial precursor lesions progress to invasive carcinoma and others do not. Routine light microscopic classification has limited value in predicting the evolution of these lesions. This article reviews the experience to date with the use of molecular markers for the prognostic evaluation of laryngeal epithelial precursor lesions. We conducted a thorough review of the published literature to identify those studies using biomarkers to predict malignant progression of laryngeal epithelial precursor lesions. Of the 336 studies identified in this systematic search, 15 met the inclusion criteria and form the basis of this review. Limited studies suggest that certain biomarkers are potentially reliable predictors of malignant progression including various regulators of cell adhesion and invasion (e.g. FAK, cortactin, osteopontin, and CD44v6) and proliferation-associated markers such as TGF-?RII and Kv3.4. The predictive value of these markers, however, has yet to be confirmed in large-scale prospective studies. Although the cell cycle-related proteins are the most frequently studied markers, none have been consistently reliable across multiple studies. The absence of standardization in methodologies, test interpretation, and other parameters may contribute to study inconsistencies. Various biomarkers have proved to have potential prognostic value and could be clinically relevant. The utility and prognostic power of these biomarkers should be confirmed in large, well-designed, standardized prospective studies.
PubMed ID: 22081098
Article Size: <1 MB
 

Lymphadenoma of the salivary gland: clinicopathological and immunohistochemical analysis of 33 tumors.

Head & Neck, Salivary Gland
Seethala RR, Thompson LD, Gnepp DR, Barnes EL, Skalova A, Montone K, Kane S, Lewis JS Jr, Solomon LW, Simpson RH, Khan A, Prasad ML.
Mod Pathol. 2012 Jan;25(1):26-35.
Lymphadenomas (LADs) are rare salivary gland tumors. Their clinicopathologic characteristics and etiopathogenesis are poorly understood. We examined 33 LADs in 31 patients (17 women and 14 men) aged 11-79 years (median 65 years). There were 22 sebaceous LADs in 21 patients (9 women and 12 men) and 11 non-sebaceous LADs in 10 patients (8 women and 2 men). Two patients had synchronous double tumors. Twenty-six tumors (79%) arose in parotid, three in the neck, and two each in submandibular gland and oral cavity. Extraparotid tumors were seen in 2 of 21 (10%) patients with sebaceous and 4 of 10 (40%) patients with non-sebaceous LADs. Seven of twenty-three (30%) patients had immunosuppressive therapy for unrelated diseases. The tumors were well circumscribed, encapsulated (n=28, 84%) painless masses, varying in size from 0.6 to 6?cm (median 2.2). The cut surfaces were gray-tan to yellow, homogeneous and multicystic (n=24, 72%). The epithelial cells were basaloid, squamous and glandular, forming solid nests, cords, tubules, and cysts. Sebaceous differentiation was restricted to sebaceous lymphadenoma. The epithelial cells expressed basal cell markers (p63, 34BE12, and/or CK5/6, 18/18, 100%) and the luminal glandular cells expressed CK7 (12/12, 100%). Myoepithelial cells were absent (n=10/16, 63%) or focal. The lymphoid stroma was reactive, with germinal centers in 28 (84%). There was no evidence of HPV (0/11), EBV (0/7), and HHV-8 (0/8). Malignant transformation to sebaceous and basal cell adenocarcinoma was seen in one patient each. None of the 11 patients with follow-up (1-8 years) recurred. In summary, sebaceous and non-sebaceous LADs are benign, encapsulated, solid and cystic tumors affecting older adults. Non-sebaceous LADs affect women and extraparotid sites more frequently than sebaceous LADs. Altered immune status may have a role in their etiopathogenesis. Multiple synchronous tumors, origin in buccal mucosa, and malignant transformation may rarely occur.
PubMed ID: 21892186
Article Size: 1 MB
 

Hyalinizing trabecular adenoma of the thyroid gland.

Endocrine, Thyroid
Thompson LD.
Ear Nose Throat J. 2011 Sep;90(9):416-7.
FIRST PARAGRAPH: Hyalinizing trabecular ade-noma (HTA) is a very rare tumor (<1% of all primary thyroid gland tumors) of thyroid follicular cell origin with a trabecular pattern of growth and marked intratrabecular hyalinization. For all intents and purposes, this is a benign tumor, although there is a case report of pulmonary metastasis with invasion, suggesting the term tumor be used instead of adenoma. A few cases have occurred following radiation exposure, and there may be a relationship to thyroid papillary carcinoma, as there have been reports of RET/PTC rearrangements.
PubMed ID: 21938699
Article Size: <1 MB

Laryngeal spindle cell squamous cell carcinoma.

Head & Neck, Larynx
Thompson LD.
Ear Nose Throat J. 2011 May;90(5):214-6.
FIRST PARAGRAPH: Spindle cell squamous cell carcinoma (SCSCC) is a squamous cell carcinoma (SCC) with a biphasic appearance, yielding a spindle cell transformation. Many names have been used in the past, but the terminology used here highlights the spindled and squamous cell appearance. As with all cases of upper aerodigestive tract SCC, there is a strong association with smoking and alcohol abuse. Furthermore, radiation exposure is occasionally reported in patients with SCSCC. This SCC variant accounts for approximately 2 to 3% of all laryngeal tumors. Men are affected much more frequently than women (12:1 ratio), and the incidence peaks in the seventh decade of life.
PubMed ID: 21563088
Article Size: <1 MB

Ear ceruminous adenoma.

Ear & Temporal Bone, Head & Neck
Thompson LD.
Ear Nose Throat J. 2011 Jul;90(7):304-5.
FIRST PARAGRAPH: Ceruminous adenoma, also called ceruminoma, ceruminal adenoma, apocrine adenoma, or even cylindroma in the past (the latter three terms are discouraged) is a benign glandular neoplasm of ceruminous glands (modified apocrine sweat glands) that arises solely from the external auditory canal. By definition, this tumor type cannot involve the auricular cartilages, ear lobe, or other such external ear apparatus.
PubMed ID: 21792797
Article Size: <1 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
 

Allergic fungal sinusitis.

Head & Neck, Sinonasal Tract
Thompson LD.
Ear Nose Throat J. 2011 March;90(3):106-107.
FIRST PARAGRAPH: Allergic fungal sinusitis, also known as allergic mucin and eosinophilic fungal rhinosinusitis, is an allergic response in the sinonasal tract mucosa to aerosolized fungal allergens, amplified and perpetuated by eosinophils. The class II genes in the major histocompatibility complex are involved in antigen presentation and immune response (modulation), and an allergic reaction develops to inhaled fungal elements in immunocompetent people. Aspergillus species are the most common agents (widespread in soil, wood, and decomposing plant material), but Alternaria, Bipolaris, Curvularia, Exserohilum, and Phialophora species have also been reported
PubMed ID: 21412738
Article Size: <1 MB

Chordoma.

Head & Neck, Neck
Thompson LD.
Ear Nose Throat J. 2011 January;90(1):16-18.
FIRST PARAGRAPH: Chordomas are low- to intermediate-grade malignant tumors that recapitulate the notochord. They are divided into three broad categories: sacrococcygeal (60% of cases), spheno-occipital (25%), and vertebral (15%). About 10% of all tumors are cervical. Vertebral or neck chordomas typically develop in the fifth and sixth decades of life; they have no predilection for either sex. Nerve impingement, progressive pain, and headaches are common. When a chordoma arises within the parapharyngeal space, the mass may be detected clinically. Radiographically, chordomas are usually solitary, lytic lesions; they are associated with matrix calcification in as many as 70% of cases.
PubMed ID: 21229504
Article Size: <1 MB

Skin basal cell carcinoma.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2010 Sep;89(9):418-20.
FIRST PARAGRAPH: Basal cell carcinoma (BCC) is a low-grade malignancy of basal keratinocytes, the cells responsible for epidermis formation. Melanocytes are seen between the keratinocytes, but they are not responsible for this tumor type. The etiology is multifactorial, related to ultraviolet sun exposure, radiation, and immunosuppression, among other factors. This is one of the most common cancers in humans. The tumors will typically present in older adults, although young adults can also develop this tumor. There is a slight male predilection, but this may be due to differences in sun exposure rather than gender variance. Caucasians and light-skinned people have a higher incidence than dark-skinned people.
PubMed ID: 20859866
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

Medullary thyroid carcinoma.

Endocrine, Thyroid
Thompson LD.
Ear Nose Throat J. 2010 Jul;89(7):301-2.
FIRST PARAGRAPH: Medullary thyroid carcinoma (MTC) is a malignant epithelial tumor of the thyroid gland that exhibits C-cell differentiation. C cells arise from the ultimobranchial body, which is derived from the fourth pharyngeal pouch, and they are found in the upper and middle areas of the thyroid lobes. These cells produce calcitonin, a hormone involved in calcium homeostasis. While a number (20%) of MTCs are associated with the autosomal-dominant inherited multiple endocrine neoplasia (MEN) syndromes (specifically MEN2A and MEN2B), most (80%) cases are sporadic. Germline or somatic mutations of the RET gene are characteristic of this tumor. They usually involve an activating point mutation of 10q11.2. Specifically, codon 634 in exon 11 is most common in MEN2A, while codon 918 in exon 16 is most common in MEN2B.
PubMed ID: 20628986
Article Size: <1 MB

Olfactory neuroblastoma.

Head & Neck, Sinonasal Tract
Thompson LD.
Head Neck Pathol. 2009 Sep;3(3):252-9.
Few neoplasms are unique to the sinonasal tract, but sinonasal undifferentiated carcinoma and olfactory neuroblastoma are malignant tumors which require unique management. Due to the rarity of these tumors, practicing pathologists are not always aware of their distinctive clinical, radiographic, histologic, immunohistochemical, and molecular features. These cases are frequently submitted for consultation, further suggesting the diagnostic difficulties inherent to these tumors. Specifically, olfactory neuroblastoma is a neoplasm that can histologically mimic many tumors within the sinonasal tract, making recognition of this tumor important, as the management frequently requires a bicranial-facial surgical approach, a trephination procedure which can be quite technically difficult and challenging to achieve a good result. The management is therefore quite unique in comparison to other sinonasal tract malignancies, setting it apart diagnostically and managerially from other lesions.
PubMed ID: 20596981
Article Size: <1 MB
 
 
 
 

Intestinal-type sinonasal adenocarcinoma.

Head & Neck, Sinonasal Tract
Thompson LD.
Ear Nose Throat J. 2010 Jan;89(1):16-8.
FIRST PARAGRAPH: Adenocarcinomas of the sinonasal tract can originate in the respiratory epithelium or the underlying mucoserous glands. Most (60%) arise from the mucoserous glands. These tumors are divided into two categories: salivary-gland-type and nonsalivary-gland-type adenocarcinomas (table). The latter are subdivided into two major categories: intestinal-type adenocarcinomas (ITACs) and nonintestinal-type adenocarcinomas.
PubMed ID: 20155693
Article Size: <1 MB

Spindle-cell lipoma.

Other Anatomic Sites, Soft Tissue
Thompson LD.
Ear Nose Throat J. 2009 Jul;88(7):992-3.
FIRST PARAGRAPH: Histologically, spindle-cell lipoma is a distinctive type of lipoma on a continuum with pleomorphic lipoma. It accounts for approximately 1.5% of all adipose tissue neoplasms. Men are affected significantly more commonly than women (9:1) at a mean age in the sixth decade. The vast majority of tumors are located in the subcutaneous tissue of the posterior neck, upper back, and shoulders. Patients present with a painless, mobile, subcutaneous mass. In rare cases, these tumors develop in other head and neck mucosal sites, such as the buccal fat pad. Spindle-cell lipomas grow as large as 13 cm (mean: 3.5). Grossly, they resemble ordinary lipomas, although they may be somewhat firmer, especially if the spindle-cell component predominates.
PubMed ID: 19623524
Article Size: <1 MB

Cholesteatoma.

Ear & Temporal Bone, Head & Neck
Caponetti G, Thompson LD, Pantanowitz L.
Ear Nose Throat J. 2009 Nov;88(11):1196-8.
FIRST PARAGRAPH: Cholesteatoma is a lesion formed from keratinizing stratified squamous epithelium. It may present intradurally (an epidermoid) or extradurally. Extradural lesions most commonly involve the middle ear cleft; involvement of the mastoid or external auditory canal is less common. The term cholesteatoma is actually a misnomer as these masses rarely contain cholesterol. Although they are not true neoplasms either, clinically they can mimic malignant neoplasms because of their propensity to destroy surrounding tissue and recur after excision.
PubMed ID: 19924660
Article Size: <1 MB

Primary Paraganglioma of the Parathyroid: A Case Report and Clinicopathologic Review.

Endocrine, Parathyroid
Levy MT, Braun JT, Pennant M, Thompson LDR.
Head Neck Pathol. 2010 Mar;4(1):37-43.
Paragangliomas are relatively uncommon neoplasms that arise in adrenal and extra-adrenal paraganglia of the autonomic nervous system. Parasympathetic paraganglioma develop predominantly in the head and neck. It is exceedingly uncommon to develop a primary intraparathyroid paraganglioma. There is only a single case report in the English literature. The information from the single previous case report (Medline 1960–2009) was combined with this case report. Our patient was a 69 year old woman who presented with a thyroid gland mass, with extension into the substernal space. The patient had a history of renal cell carcinoma removed 18 months before. At surgery, a thyroid lobectomy and a parathyroidectomy were performed. The parathyroid tissue showed a very well defined zellballen arrangement of paraganglion cells within the parenchyma of the parathyroid gland. The cells had ample basophilic, granular cytoplasm. The nuclei were generally round to oval with ‘salt-and-pepper’ nuclear chromatin distribution. There was a richly vascularized stroma. Mitotic figures, necrosis, invasive growth, and profound nuclear pleomorphism were absent. The neoplastic cells were strongly and diffusely immunoreactive with chromogranin, synaptophysin, CD56, and focally with cyclin-D1. The paraganglioma showed a delicate S-100 protein positive supporting sustentacular framework. Keratin, CD10, PTH, calcitonin and RCC markers were negative. The patient showed no stigmata of Multiple Endocrine Neoplasia (MEN) and has no paraganglioma in any other anatomic site. She is alive without any additional findings 12 months after surgery. Isolated paraganglioma within the parathyroid is rare, and should be separated from parathyroid adenoma, hyperplasia or metastatic disease to assure appropriate management.
PubMed ID: 20237987
Article Size: <1 MB
 

Laryngeal granular cell tumor.

Head & Neck, Larynx
Thompson LD.
Ear Nose Throat J. 2009 Mar;88(3):824-5.
FIRST PARAGRAPH: Granular cell tumors, also called Abrikossoff tumors, are benign, slowly growing neoplasms, presumably of Schwann cell origin. They may occur anywhere in the body, although 50% occur in the head and neck. The most common site is the tongue; the larynx is involved in approximately 10% of all cases. Granular cell tumors typically develop in the fourth and fifth decades of life; they are quite rare in children. Blacks are affected more commonly than other races. A slight female preponderance has been reported. As many as 10% of patients experience multifocal synchronous or metachronous tumors.
PubMed ID: 19291628
Article Size: <1 MB

Primary extracranial meningiomas: An analysis of 146 cases.

Other Anatomic Sites, Soft Tissue
Rushing, EJ, Bouffard JP, McCall S, Olsen C, Mena H, Sandberg GD, Thompson LDR.
Head Neck Pathol. 2010 Mar;4(1):37-43.
Primary extracranial meningiomas are rare neoplasms, frequently misdiagnosed, resulting in inappropriate clinical management. To date, a large clinicopathologic study has not been reported. One hundred and forty-six cases diagnosed between 1970 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemistry analysis was performed (n = 85), and patient follow-up was obtained (n = 110). The patients included 74 (50.7%) females and 72 (49.3%) males. Tumors of the skin were much more common in males than females (1.7:1). There was an overall mean age at presentation of 42.4 years, with a range of 0.3–88 years. The overall mean age at presentation was significantly younger for skin primaries (36.2 years) than for ear (50.1 years) and nasal cavity (47.1 years) primaries. Symptoms were in general non-specific and reflected the anatomic site of involvement, affecting the following areas in order of frequency: scalp skin (40.4%), ear and temporal bone (26%), and sinonasal tract (24%). The tumors ranged in size from 0.5 up to 8 cm, with a mean size of 2.3 cm. Histologically, the majority of tumors were meningothelial (77.4%), followed by atypical (7.5%), psammomatous (4.1%) and anaplastic (2.7%). Psammoma bodies were present in 45 tumors (30.8%), and bone invasion in 31 (21.2%) of tumors. The vast majority were WHO Grade I tumors (87.7%), followed by Grade II (9.6%) and Grade III (2.7%) tumors. Immunohistochemically, the tumor cells labeled for EMA (76%; 61/80), S-100 protein (19%; 15/78), CK 7 (22%; 12/55), and while there was ki-67 labeling in 27% (21/78), 3% of cells were positive. The differential diagnosis included a number of mesenchymal and epithelial tumors (paraganglioma, schwannoma, carcinoma, melanoma, neuroendocrine adenoma of the middle ear), depending on the anatomic site of involvement. Treatment and follow-up was available in 110 patients: Biopsy, local excision, or wide excision was employed. Follow-up time ranged from 1 month to 32 years, with an average of 14.5 years. Recurrences were noted in 26 (23.6%) patients, who were further managed by additional surgery. At last follow-up, recurrent disease was persistent in 15 patients (mean, 7.7 years): 13 patients were dead (died with disease) and two were alive; the remaining patients were disease free (alive 60, mean 19.0 years, dead 35, mean 9.6 years). There is no statistically significant difference in 5-year survival rates by site: ear and temporal bone: 83.3%; nasal cavity: 81.8%; scalp skin: 78.5%; other sites: 65.5% (P = 0.155). Meningiomas can present in a wide variety of sites, especially within the head and neck region. They behave as slow-growing neoplasms with a good prognosis, with longest survival associated with younger age, and complete resection. Awareness of this diagnosis in an unexpected location will help to avoid potential difficulties associated with the diagnosis and management of these tumors.
PubMed ID: 19644540
Article Size: <1 MB
 

Malacoplakia of the tongue: a case report and clinicopathologic review of 6 cases.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Diapera MJ, Lozon CL, Thompson LD.
Am J Otolaryngol. 2009 Mar-Apr;30(2):101-5.
BACKGROUND: Extra-urogenital tract malacoplakia is uncommon, with tongue malacoplakia being exceptionally rare. The nonspecific clinical presentation and variable histologic patterns can make recognition of this lesion and separation from other lesions challenging. There are only a few reported cases in the English literature.
MATERIALS AND METHODS: Five case reports of tongue malacoplakia were compiled from the literature (MedLine 1960-2008) and integrated with this case report.
RESULTS: The patients included 4 males and 2 females, ranging in age from 9 to 98 years (mean, 64 years). Patients presented with difficulty swallowing, foreign body sensation, a mass lesion, or referred pain (neck or ear). Symptoms were present from a few days up to 18 months. The base of the tongue was the most frequent site, although midline tongue and half of the tongue were also affected. Radiographic studies demonstrated a mass, with a single lesion showing positron emission tomography positivity. Two patients had previous cancers (prostate and colorectal; larynx). This case report was a farm hand for horses, with gram-negative rods, suggestive of Rhodococcus equi identified. The lesions were 1 to 2 cm in greatest dimension. Histologically, there is pseudoepitheliomatous hyperplasia or ulceration with a heavy acute and chronic inflammatory infiltrate. The subepithelial spaces are completely filled with eosinophilic histiocytes, most of which contain granular material in their cytoplasm. Well-formed, blue, calcific bodies are noted, a few showing a ‘targetoid appearance’ and concentric lamination. These Michaelis-Gutmann bodies are positive with von Kossa, iron, and periodic acid-Schiff stains. These findings support a diagnosis of malacoplakia. The differential diagnosis includes granular cell tumor, poorly differentiated carcinoma, and Langerhans histiocytosis. Patients are managed with antibiotic therapy and excision.
CONCLUSIONS: Tongue malacoplakia is rare, often presenting as a mass lesion. Histologic recognition of this abnormal phagocytic disorder will prevent potentially disfiguring surgery.
PubMed ID: 19239951
Article Size: 3 MB
 

Laryngeal squamous papilloma.

Head & Neck, Larynx
Thompson L.
Ear Nose Throat J. 2007 Jul;86(7):379
FIRST PARAGRAPH: Squamous papilloma (SP) is the most common benign laryngeal tumor. It is caused by the human papillomavirus. Clinically, SP rarely occurs as a solitary lesion; most arise as multiple, recurrent tumors, usually in children. SPs generally originate in the true and false vocal folds; they may spread to other sites in the oral cavity and aerodigestive tract. They form at the juxtaposition of the squamous and respiratory epithelia. If an area of juxtaposition is artificially induced (such as by squamous metaplasia), spread of the disease may result. There is a characteristic bimodal age distribution, with a juvenile peak at 5 years and an adult peak between 20 and 40 years. The disease course tends to be more aggressive in children, who frequently develop recurrent and progressive disease. The relatively small diameter of the airways in children may account for some of the severe respiratory embarrassment they experience. There is a slight male predominance in adults. Patients usually present with dysphonia and hoarseness.
PubMed ID: 17702311
Article Size: <1 MB

Parathyroid carcinoma.

Endocrine, Parathyroid
Thompson LD.
Ear Nose Throat J. 2008 Sep; 87(9):502-504.
FIRST PARAGRAPH: Parathyroid carcinoma is rare, comprising less than 1% of all cases of primary hyperparathyroidism. Parathyroid carcinoma occurs in patients of all ages, and there is no predilection for either sex. Its clinical features are primarily attributable to the effects of hypercalcemia and excessive secretion of parathyroid hormone (PTH). Most of its symptoms–weakness, fatigue, anorexia, weight loss, and nausea–are nonspecific, but an excessively high serum calcium level (>16 mg/dl) can be associated with nephrolithiasis, renal insufficiency, and bone ‘brown tumors.’ A palpable neck mass suggests a parathyroid carcinoma. Parathyroid carcinoma is a suggested component of hyperparathyroidism-jaw tumor syndrome.
PubMed ID: 18800320
Article Size: <1 MB
 

Ceruminous Gland Carcinomas: A Clinicopathologic and Immunophenotypic Study of 17 Cases.

Ear & Temporal Bone, Head & Neck
Crain N, Nelson BL, Barnes EL and Thompson LDR.
Head Neck Pathol. 2009 Mar;3(1):1-17.
BACKGROUND: Ceruminal gland carcinomas are rare neoplasms confined to the skin lining the cartilaginous part of the external auditory canal.
STUDY DESIGN:Retrospective.
RESULTS: The patients included 11 men and 6 women, aged 33-82 years (mean, 59.5 years). Patients presented clinically with a mass of the outer half of the external auditory canal (n = 14), hearing changes (n = 5), drainage (n = 4), or paralysis of the facial nerve (n = 3). The polypoid masses ranged in size from 0.5 to 3 cm in greatest dimension (mean, 1.8 cm). Histologically, the tumors demonstrated a solid to cystic pattern, composed of an infiltrating glandular to cribriform arrangement of epithelial cells. Histologic features included a dual cell population (although not the dominant histology), increased cellularity, moderate to severe nuclear pleomorphism, irregular nucleoli, increased mitotic figures (mean, 3/10 HPF), including atypical forms, and tumor necrosis (n = 2). Tumors were divided into three types of adenocarcinoma based on pattern of growth and cell type (ceruminous, NOS [n = 12], adenoid cystic [n = 4], mucoepidermoid [n = 1]). CK7 and CD117 highlighted the luminal cells, while S1-00 protein showed a predilection for the basal cells of ceruminous and adenoid cystic carcinomas. Metastatic adenocarcinoma or direct extension from salivary gland neoplasms are the principle differential considerations. Surgical resection was used in all patients with radiation used in four patients. Eleven patients were alive or had died of unrelated causes without evidence of disease (mean, 11.2 years); six patients had died with disease (mean, 4.9 years), all of whom had developed local recurrence.
CONCLUSION: Ceruminous-type carcinomas, with the exception of ceruminous mucoepidermoid carcinoma, all demonstrated a dual cell population of basal myoepithelial-type cells and luminal apocrine cells. The specific histologic sub-type does not influence the long-term patient outcome.
PubMed ID: 20596983
Article Size: 1 MB
 

Gout.

Ear & Temporal Bone, Head & Neck
Hollowell M, Thompson LD, Pantanowitz L.
Ear Nose Throat J. 2008 Mar;87(3):132, 134
FIRST PARAGRAPH: Gout is caused by disordered purine metabolism resulting in hyperuricemia. Symptoms are related to the precipitation of monosodium urate (uric acid) crystals, typically in joint spaces or soft tissue. Primary gout is caused by an increase in uric acid production, while secondary gout is caused by either a decrease in urinary uric acid excretion or an overproduction of purine secondary to increased cell turnover (e.g., tumor lysis). Predisposing clinical factors include older age (fifth and older decade), male sex, obesity, heavy alcohol ingestion, a purine-rich diet, certain medications (e.g., thiazide diuretics), and genetic factors.
PubMed ID: 18404905
Article Size: <1 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
 

Update on Nasopharyngeal Carcinoma.

Head & Neck, Sinonasal Tract
Thompson LDR.
Head Neck Pathol. 2007 Sep;1(1):81-6.
FIRST PARAGRAPH: The most common type of nasopharyngeal tumor is nasopharyngeal carcinoma. The etiology is multifactorial with race, genetics, environment and Epstein-Barr virus (EBV) all playing a role. While rare in Caucasian populations, it is one of the most frequent nasopharyngeal cancers in Chinese, and has endemic clusters in Alaskan Eskimos, Indians, and Aleuts. Interestingly, as native-born Chinese migrate, the incidence diminishes in successive generations, although still higher than the native population.
PubMed ID: 20614287
Article Size: <1 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
 

Traumatic fracture callus

Bone, Other Anatomic Sites
Gannon FH, Thompson L.
Ear Nose Throat J. 2007 Apr;86(4):200
FIRST PARAGRAPH: Bones of the craniofacial region are frequently broken, traumatically or iatrogenically. Whereas traumatic fractures can be readily identified clinically and radiologically, they can represent a diagnostic challenge histologically. A short discussion about the histologic evolution of traumatic fractures will help a pathologist know what to expect histologically, based on the time frames of development. The repair process follows a predictable histologic evolution of five distinct phases: culatory, cellular, vascular, metabolic, and mechanical.
PubMed ID: 17500387
Article Size: <1 MB