Mucoepidermoid carcinoma.

Head & Neck, Salivary Gland
Thompson LD.
Ear Nose Throat J. 2005 Dec;84(12):762-3.
FIRST PARAGRAPH: Mucoepidermoid carcinoma (MEC) is the most common primary salivary gland malignancy, accounting for approximately 25% of all malignancies. More than half of these cases involve the major salivary glands, primarily the parotid glands. MEC can also involve a variety of other sites that have minor mucoserous glands. Women are more commonly affected than men (3:2), and the mean age at onset is in the 5th decade of life. MEC is also the most common salivary gland malignancy in children.
PubMed ID: 16408550
Article Size: <1 MB

Seborrheic keratosis.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2006 Feb;85(2):79.
FIRST PARAGRAPH: Seborrheic keratosis is a benign proliferation of the epidermis. Many names are applied to this lesion (senile wart, melanoacanthoma), but seborrheic keratosis is the most widely accepted. Considered one of the most common skin lesions, it usually occurs in older patients; there is no predilection for either sex. The lesions appear “stuck on” the skin, usually on sun-exposed skin. They can be tan, brown, or black. Frequent irritation or friction may cause a papule/nodule to become red and scaly and result in a clinical misdiagnosis. Many variants of seborrheic keratosis are recognized clinically and histologically, but they have no management implications.
PubMed ID: 16579189
Article Size: <1 MB

Pituitary adenoma.

Endocrine, Pituitary
Thompson LD.
Ear Nose Throat J. 2006 Mar;85(3):152-3.
FIRST PARAGRAPH: Pituitary tumors account for approximately 15% of all intracranial neoplasms. They can be either benign or malignant, primary or secondary, and epithelial or nonepithelial. Pituitary carcinoma can only be diagnosed when there is confirmed metastatic disease. The vast majority are primary epithelial pituitary adenomas, which generally arise from hormone-producing cells, regardless of whether a particular tumor is active or inactive (i.e., non-hormone-producing). Pituitary adenomas are further classified on the basis of size, function, cell type, hormone production, location, and biologic behavior. Most pituitary adenomas are benign neoplasms, either null-cell or prolactin-producing microadenomas (<1 cm in their greatest dimension -- usually seen radiographically) that are seen within the pituitary gland. Most pituitary adenomas occur in women; they can arise at any age, but they are more common in the third through sixth decades of life. Patients with a pituitary adenoma present with either an endocrinopathy or a mass effect. Nonfunctioning adenomas exhibit no biochemical or clinical evidence of excessive hormone production. Patients with functional tumors present with specific syndromes, such as Cushing’s syndrome (adrenocorticotropic hormone-producing).
PubMed ID: 16615592
Article Size: <1 MB

Epithelial-myoepithelial carcinoma.

Head & Neck, Salivary Gland
Folk GS, Thompson LD.
Ear Nose Throat J. 2006 Apr;85(4):214, 216.
FIRST PARAGRAPH: Epithelial-myoepithelial carcinoma (EMC) is an uncommon salivary gland malignancy, representing 1% of all salivary gland tumors. Women are more frequently affected (2:1). The incidence of EMC peaks between the ages of 50 and 60 years, although the age range is broad. The parotid gland is most frequently affected (60%), but both major and minor salivary glands can be involved. Patients typically present with a painless, slowly growing mass; occasionally there is rapid growth, pain, or facial weakness.
PubMed ID: 16696350
Article Size: <1 MB

Melanotic neuroectodermal tumor of infancy.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Nelson BL, Thompson LD.
Ear Nose Throat J. 2006 Jun;85(6):365.
FIRST PARAGRAPH: Melanotic neuroectodermal tumor of infancy is a rare, neural-crest-derived neoplasm that is believed to be congenital. The tumor has a marked predilection for the head and neck — particularly the maxilla, where approximately 70% of these tumors are located. The anterior maxilla is most commonly affected. There is no predilection for either sex. Nearly all patients present with an enlarging mass, usually within a few years of birth. Intraoral lesions may appear ‘blue,’ suggesting the presence of pigment. Radiographic images will often show a destructive lesion with tooth displacement, but they are nonspecific. Laboratory studies show high urinary levels of vanillylmandelic acid.
PubMed ID: 16866106
Article Size: <1 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

Nasal glial heterotopia: a clinicopathologic and immunophenotypic analysis of 10 cases with a review of the literature.

Head & Neck, Sinonasal Tract
Penner CR, Thompson L.
Ann Diagn Pathol. 2003 Dec;7(6):354-9.
Nasal glial heterotopia (also known as ‘nasal glioma’), is a rare developmental abnormality seen in a wide age group but typically presenting at birth or in early childhood. Failure to recognize the entity is the principle difficulty in diagnosis. Ten cases of nasal glial heterotopic diagnosed between 1970 and 2000 were identified. Histologic and immunohistochemical features were evaluated and patient follow-up was obtained. The patients included five females and five males with a mean age at presentation of 8.6 years (range, birth to 44 years). Most patients presented clinically with a polypoid mass in the nasal cavity, although two patients had a mass on the nasal bridge. Symptoms were present for an average of 2 to 3 months. A connection to the central nervous system was identified in one case. Masses ranged in size from 1 to 7 cm in greatest dimension (mean, 2.4 cm). Histologically, the masses were composed of astrocytes (including gemistocytic type) and neuroglial fibers intermixed with a fibrovascular connective tissue stroma. Neurons and ependymal cells were noted in two cases. Focal calcifications and inflammatory cells were identified occasionally. Masson trichrome stains the collagen intensely blue, while the neural population stains magenta. Immunohistochemical reactivity with glial fibrillary acidic protein and S-100 protein will help to confirm the histologic diagnosis, while collagen type IV and laminin can highlight the reactive fibrosis. All cases were managed by surgery. All patients were alive without complications at last follow-up (mean, 26.8 years), except for the single fetus included in the study. Nasal glial heterotopia typically involves the nasal cavity and usually presents perinatally, although three patients presented in adulthood. The subtle glial component on routine microscopy can be accentuated with a trichrome stain or by immunoreactivity with glial fibrillary acidic protein and S-100 protein. Imaging studies must be performed before surgery to exclude an encephalocele, which requires different surgery. Complete surgical excision of nasal glial heterotopias is curative.
PubMed ID: 15018118
Article Size: <1 MB
 

Canalicular adenoma.

Head & Neck, Salivary Gland
Penner CR, Thompson L.
Ear Nose Throat J. 2005 Mar;84(3):132.
FIRST PARAGRAPH: Canalicular adenomas are benign neoplasms with a unique predilection for the upper lip (~80% of cases). They account for 1% of all salivary gland neoplasms. Their incidence peaks during the seventh decade of life; they are distinctly uncommon in patients younger than 50 years of age. The female-to-male predominance is approximately 2:1.
PubMed ID: 15871577
Article Size: <1 MB

Neuroendocrine adenoma of the middle ear.

Ear & Temporal Bone, Head & Neck
Thompson LD.
Ear Nose Throat J. 2005 Sep;84(9):560-1.
FIRST PARAGRAPH: Neuroendocrine adenomas of the middle ear, also known as middle ear adenomas and carcinoids, are rare neoplasms. These tumors occur equally in the genders, and they usually affect middle-aged patients. Patients present clinically with hearing loss and pain; tinnitus, equilibrium changes, and nerve paralysis might also be identified. Tumors are usually smaller than 1 cm in their greatest dimension, and they occasionally extend into the external or internal auditory canal.
PubMed ID: 16261754
Article Size: <1 MB

Metastatic cystic squamous cell carcinoma.

Head & Neck, Oropharynx
Thompson LD.
Ear Nose Throat J. 2005 May;84(5):272-3.
FIRST PARAGRAPH: Metastatic disease to the lymph nodes of the neck is an important clinical and pathologic consideration. When there is no known primary, the pathologist and radiologist must provide additional input to the clinician during the work-up. This installment of PATHOLOGY CLINIC focuses on cervical cystic squamous cell carcinoma (cSCC), which is commonly misdiagnosed as squamous cell carcinoma arising in a branchiogenic cyst or as a branchiogenic carcinoma.
PubMed ID: 15971745
Article Size: <1 MB

Ceruminous adenomas: a clinicopathologic study of 41 cases with a review of the literature.

Ear & Temporal Bone, Head & Neck
Thompson LD, Nelson BL, Barnes EL.
Am J Surg Pathol. 2004 Mar;28(3):308-18.
BACKGROUND: Ceruminous gland neoplasms are rare neoplasms. To date, a large clinicopathologic study of benign ceruminous gland neoplasms has not been reported.
DESIGN: Forty-one cases of ceruminous gland adenomas diagnosed between 1970 and 2000 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemical analysis was performed (n = 21), and patient follow-up was obtained (n = 40).
RESULTS: The patients included 22 men and 19 women, 24 to 85 years of age (mean, 54.2 years). Patients presented clinically with a painless mass of the outer half of the external auditory canal (n = 33) or with hearing changes (n = 11). Symptoms were present for an average of 16.3 months. The polypoid masses affected the external auditory canal only and ranged in size from 0.4 to 2 cm in greatest dimension (mean, 1.1 cm). Histologically, the tumors demonstrated glands and small cysts lined by a tubuloglandular proliferation of inner ceruminous cells (cerumen-secreting epithelium with decapitation secretion) subtended by a spindled to cuboidal myoepithelial layer. A hyalinized stroma created an infiltrative pattern of growth; surface involvement (n = 8) was seen. Tumors were divided into ceruminous adenoma (n = 36), ceruminous pleomorphic adenoma (n = 4), and syringocystadenoma papilliferum (n = 1) types. The luminal cells were strongly and diffusely immunoreactive with CK7, while the basal cells were highlighted with CK5/6, S-100 protein, and p63. CD117 highlighted the luminal cells preferentially. The proliferation markers revealed a low index. Adenocarcinoma and middle ear adenoma are the principal differential consideration. Surgical excision was used in all patients. Four patients developed a recurrence due to incomplete excision. All patients were without evidence of disease at the last follow-up: alive (n = 28, mean 16.3 years) or dead (n = 12, mean 11.8 years).
CONCLUSION: Ceruminous gland adenomas are the most common external auditory canal tumors. They demonstrate a dual cell population of basal myoepithelial-type cells and luminal ceruminous (ceruminal) cells. Cerumen pigment, CK7, and p63 can help to distinguish this tumor from other neoplasms that occur in the region. Complete surgical excision results in an excellent long-term clinical outcome.
PubMed ID: 15104293
Article Size: 2 MB
 

Kimura disease: a clinicopathologic study of 21 cases.

Head & Neck, Neck
Chen H, Thompson LD, Aguilera NS, Abbondanzo SL.
Am J Surg Pathol. 2004 Apr;28(4):505-13.
Kimura disease is a rare form of chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region and frequently associated with regional lymphadenopathy and/or salivary gland involvement. This condition has a predilection for males of Asian descent and may clinically simulate a neoplasm. Kimura disease is sometimes confused with angiolymphoid hyperplasia with eosinophilia, which occurs in the superficial skin of the head and neck region. Although sporadic cases have been reported in non-Asians, there is no large, comprehensive study of Kimura disease in the United States. We report 21 cases with nodal involvement that, histologically, are consistent with Kimura disease. There were 18 males and 3 females (male/female ratio 6:1), 8 to 64 years of age (mean, 32 years), and included 7 Caucasians, 6 Blacks, 6 Asians, 1 Hispanic, and 1 Arabic. Anatomic sites of involvement included posterior auricular (n = 10), cervical (n = 6), inguinal (n = 3), and epitrochlear (n = 2) lymph nodes, with two patients having associated salivary gland involvement. Most (n = 16) cases had peripheral blood eosinophilia. Consistent histologic features were follicular hyperplasia, eosinophilic infiltrates, and proliferation of postcapillary venules. Follow-up data on 18 patients revealed that 13 were alive without disease (3 had recurrence), mean follow-up, 10.9 years; 4 were alive with disease (2 had a recurrence), mean follow-up, 8.8 years; and 1 died with disease (12.7 years). Kimura disease has been described more often in Asians, but it does occur in non-Asians with a similar clinicopathologic presentation. It is a distinctive entity with no known etiology. Kimura disease has characteristic histologic features that are important to recognize and can be used to differentiate it from hypersensitivity and drug reactions and infections.
PubMed ID: 15087670
Article Size: 4 MB
 

Endoscopic management of intratracheal ectopic thyroid.

Endocrine, Thyroid
Pritchyk KM, Thompson LD, Malekzadeh S.
Otolaryngol Head Neck Surg. 2004 May;130(5):630-2.
CASE REPORT CONCLUSIONS: ITET is a rare cause of upper airway obstruction. CT scan of the neck without contrast should be performed to evaluate the location of obstruction. MRI scan should be used to assess the extent and vascular nature of the lesion. Endoscopic laser excision is an appropriate treatment option for benign and vascular ITET tissue.
PubMed ID: 15138431
Article Size: <1 MB
 

HIV-associated Hodgkin lymphoma: a clinicopathologic and immunophenotypic study of 45 cases.

Head & Neck, Neck
Thompson LD, Fisher SI, Chu WS, Nelson A, Abbondanzo SL.
Am J Clin Pathol. 2004 May;121(5):727-38.
We retrospectively analyzed 45 cases of HIV-associated Hodgkin lymphoma (HIV-HL). HIV-HL generally is a disease of young white men (mean age, 40.1 years) who acquired HIV infection by homosexual or bisexual behavior (68%), intravenous drug use (24%), and/or blood transfusion (8%). The mean interval between the diagnosis of HIV and HIV-HL was 5.2 years. Morphologic classification of nodal biopsy specimens (2001 World Health Organization criteria) included 15 mixed cellularity Hodgkin lymphomas (MCHLs), 14 nodular sclerosis Hodgkin lymphomas (NSHLs), 9 lymphocyte depleted Hodgkin lymphomas (LDHLs), and 7 classic Hodgkin lymphomas, type not further categorized. The Hodgkin-Reed-Sternberg (HRS) cells expressed positive immunoreactivity with fascin (30/30 [100%]), CD30 (35/37 [95%]), CD15 (32/36 [89%]), bcl-X(L) (25/31 [81%]), bcl-2 (15/29 [52%]), CD20 (4/34 [12%]), bcl-6 (3/28 [11%]), and Epstein-Barr virus latent membrane protein-1 (32/33 [97%]) and were nonreactive for CD138/syndecan-1. CD4 and CD8 immunostaining showed an inverted CD4/CD8 ratio (<1/20) in all cases. At diagnosis, most patients (n = 27) had high-stage disease (IV(E)) associated with an aggressive course (16% 5-year survival). LDHL behaved more aggressively than MCHL and NSHL (15% vs 40%, 5-year survival, respectively), as did disease with a sarcomatoid pattern (11% 5-year survival). Chemotherapy and radiotherapy proved efficacious in a minority of these patients.
PubMed ID: 15151213
Article Size: <1 MB
 

Skin keloid.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2004 Aug;83(8):519.
FIRST PARAGRAPH: A keloid is a form of excessive scar formation that occurs in response to tissue injury. Keloids arise as a result of abnormal wound healing where there is a disruption of the balance between collagen formation and degradation with a shift toward excess synthesis. Even with injuries that are seemingly minor, the resultant scar formation is disproportionately excessive. The etiology is unknown.
PubMed ID: 15487627
Article Size: <1 MB

Management of melanotic neuroectodermal tumor of infancy.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Gaiger de Oliveira M, Thompson LD, Chaves AC, Rados PV, da Silva Lauxen I, Filho MS.
Ann Diagn Pathol. 2004 Aug;8(4):207-12.
Melanotic neuroectodermal tumor of infancy is a rare congenital neoplasm involving the head and neck in young patients. The clinical assessment, histologic diagnosis, and management is reviewed, with an emphasis on different treatment alternatives in two new case reports.
PubMed ID: 15290671
Article Size: <1 MB
 

Chondrosarcoma of the larynx.

Head & Neck, Larynx
Thompson LD.
Ear Nose Throat J. 2004 Sep;83(9):609.
FIRST PARAGRAPH: Chondrosarcoma of the larynx accounts for approximately 0.2% of all head and neck malignancies and approximately 1% of all laryngeal malignant tumors, although it is the most common nonepithelial neoplasm of the larynx. Men are affected more frequently than women (3:1 ratio), usually during the middle to later decades of life. Patients present with a variety of symptoms as a result of tumor growth, including dyspnea, dysphagia, hoarseness, airway obstruction, and/or pain.
PubMed ID: 15529643
Article Size: <1 MB

Papillary thyroid carcinoma.

Endocrine, Thyroid
Nelson BL, Thompson LD.
Ear Nose Throat J. 2004 Oct;83(10):675-6.
FIRST PARAGRAPH: Papillary thyroid carcinoma is the most common type of thyroid malignancy. The tumor occurs largely in adults, usually those between the ages of 20 and 50 years; the female-to-male ratio is 4:1. Papillary thyroid carcinoma is also the most common pediatric thyroid malignancy.
PubMed ID: 15586863
Article Size: <1 MB

Branchial cleft cyst.

Head & Neck, Neck
Thompson LD.
Ear Nose Throat J. 2004 Nov;83(11):740.
FIRST PARAGRAPH: A developmental alteration of the branchial clefts or pouches can result in cysts, sinuses, and/or fistulas. A branchial cleft cyst is a congenital abnormality usually located in the lateral neck along the anterior portion of the sternocleidomastoid muscle; it can also involve the ear and parotid salivary gland. There is no sex preference, and although the lesion usually presents clinically in young patients, older patients are occasionally affected as well. The cysts are typically nontender masses that may become secondarily inflamed or infected, which often brings them to clinical attention. Bilateral masses are associated with an increased likelihood of a syndrome.
PubMed ID: 15628626
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

Diffuse sclerosing variant of papillary thyroid carcinoma: a clinicopathologic and immunophenotypic analysis of 22 cases.

Endocrine, Thyroid
Thompson LD, Wieneke JA, Heffess CS.
Endocr Pathol. 2005 Winter;16(4):331-48.
BACKGROUND: The diffuse sclerosing variant of papillary thyroid carcinoma (DSV-PTC) is an uncommon tumor making up about 2% of all papillary thyroid carcinomas. Previous studies have not comprehensively evaluated these tumors in a large series of patients.
DESIGN: Twenty-two cases of DSV-PTC diagnosed between 1970 and 2000 were identified in the files of the AFIP. Histologic and immunohistochemical features were evaluated and patient follow-up was obtained.
RESULTS: The tumors affected 14 females and 8 males, aged 6 to 49 yr (mean, 18 yr), with males presenting at a mean older age than females (24 vs 14 yr). Symptoms included an enlarging mass in the thyroid, present for a mean of 9.5 mo. While a dominant tumor was identified in a single lobe, bilateral disease was common (n = 16). The dominant mass ranged in size from 1.7 to 5.8 cm in diameter (mean, 3.8 cm). Histologically, all cases demonstrated a papillary carcinoma (conventional, solid, or follicular pattern) diffusely involving the gland. Extrathyroidal extension, lymphocytic thyroiditis, squamous metaplasia, increased fibrosis/sclerosis, and psammoma bodies were present to a variable degree. Both the papillary carcinoma and squamous metaplasia cells were strongly immunoreactive with CK19, thyroglobulin, and TTF-1. An increased number of S-100 protein immunoreactive dendritic cells were recognized. p53 was increased (>15%) in the tumor cells in 12 patients, while Ki-67 was increased in the tumor cells in two patients. Perithyroidal and cervical lymph node metastasis occurred in 18 (82%) patients. All metastases demonstrated histologic features similar to the primary. Complete resection (thyroidectomy in 18 patients) with lymph node dissection, yielded a 95% 5-yr survival without evidence of disease. One patient died of disease after a malignant transformation of the squamous metaplasia into squamous cell carcinoma.
CONCLUSIONS: The recognition of DSV-PTC can be made with the following features: classic to solid foci of PTC, lymphocytic thyroiditis, squamous metaplasia, increased fibrosis, and innumerable psammoma bodies. DSV-PTC is more biologically aggressive than conventional PTC, but the patients’ survival is not significantly different. This diagnosis should lead the clinician to aggressively manage these patients (thyroidectomy and lymph node dissection) in an effort to achieve an excellent long-term clinical outcome.
PubMed ID: 16627920
Article Size: <1 MB
 

Embryonal rhabdomyosarcoma of the ear.

Ear & Temporal Bone, Head & Neck
Sautter NB, Thompson LD.
Ear Nose Throat J. 2004 May;83(5):316-7.
FIRST PARAGRAPH: Rhabdomyosarcoma is the most common soft-tissue malignancy in the pediatric population. It is generally classified into embryonal, alveolar, pleomorphic, and mixed histologic subtypes. Embryonal rhabdomyosarcoma is the most common histologic variant seen in childhood; a large proportion of them arise in the head and neck—most commonly in the orbit, the nasopharynx, and the ear.
PubMed ID: 15195874
Article Size: <1 MB

Dyshormonogenetic goiter of the thyroid gland.

Endocrine, Thyroid
Thompson LD.
Ear Nose Throat J. 2005 Apr;84(4):200.
FIRST PARAGRAPH: Dyshormonogenetic goiter is the name given to a family of inborn errors of metabolism that lead to defects in the synthesis of thyroid hormone. The prevalence of this disease is 1 in 30,000 to 50,000 live births, and it is the second most common cause (10 to 15%) of permanent congenital hypothyroidism.
PubMed ID: 15929313
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

Salivary gland lymphoepithelial cysts.

Head & Neck, Salivary Gland
Varnholt H, Thompson L, Pantanowitz L.
Ear Nose Throat J. 2007 May;86(5):265
FIRST PARAGRAPH: Lymphoepithelial cysts are benign, slowly growing unilocular or multilocular lesions that may appear in the head and neck. Among the reported head and neck sites are the salivary glands (typically the parotid gland) and the oral cavity (usually the floor of the mouth). These cysts are usually seen in adults and only occasionally in children. They range in size from 0.5 to 5.0 cm, and they can cause considerable cosmetic deformity and physical discomfort.
PubMed ID: 17580800
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
 

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

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
 

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
 

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
 

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

Melanoma.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2007 Sep;86(9):542, 545
FIRST PARAGRAPH: Melanoma is a malignancy of melanocytes that show a series of molecular events that result in the melanocytes going through a stepwise progression from dysplasia to invasion to metastasis. Melanomas account for approximately 4.4% of all malignancies. Approximately 62,000 new cases of melanoma are reported annually in the United States, and they are responsible for about 7,900 deaths. The incidence of melanoma worldwide has been increasing steadily.
PubMed ID: 17970142
Article Size: <1 MB

Ewing sarcoma and primitive neuroectodermal tumor.

Head & Neck, Sinonasal Tract
Thompson LDR.
Ear Nose Throat J. 2007 Feb;86(2):79-80.
FIRST PARAGRAPH: Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are closely related, high-grade, round-cell tumors with a neuroectodermal phenotype. These tumors are histologically considered on a morphologic spectrum, and they express similar genetic alterations. ES usually develops in bone and is more undifferentiated, while PNET tends to involve soft tissue and demonstrates more pronounced neuroendocrine features.
PubMed ID: 17385610
Article Size: <1 MB

Neoplasms metastatic to the thyroid gland.

Endocrine, Thyroid
Thompson L.
Ear Nose Throat J. 2006 Aug;85(8):480, 483.
FIRST PARAGRAPH: Tumors that occur in the thyroid gland as a result of lymph or vascular spread from distant sites are considered to represent metastatic disease rather than a direct extension of a primary from an adjacent organ. Metastatic deposits are identified at a higher frequency in abnormal glands—that is, those with adenomatoid nodules, thyroiditis, and follicular neoplasms. Further, metastatic deposits may be found within primary thyroid tumors, such as a renal cell carcinoma metastatic to a thyroid papillary carcinoma. Although a thyroid gland mass may be the presenting clinical sign, it is more often the underlying thyroid gland disease (e.g., thyroiditis, adenomatoid nodules) that prompts clinical evaluation. The thyroid gland metastatic deposit is the initial presentation of an occult primary tumor in as many as 40% of patients. Carcinomas are the most common metastatic tumors from (in order of frequency) the kidney (figure 1), lung, breast (figure 2), and stomach; melanoma is less common.
PubMed ID: 16999049
Article Size: <1 MB

Mucinous cystic neoplasms of the pancreas: radiologic-pathologic correlation.

Endocrine, Pancreas
Buetow PC, Rao P, Thompson LD.
Radiographics. 1998 Mar-Apr;18(2):433-49.
Mucinous cystic neoplasms of the pancreas are rare primary tumors. They have pathologic and clinical similarities to biliary cystadenomas of the liver and mucinous cystic tumors of the ovary. Mucinous cystic neoplasms of the pancreas typically affect middle-aged women and arise in the tail of the pancreas. Gross pathologic and imaging features usually are those of a large, multilocular cystic mass. There is, however, a spectrum of radiologic findings that overlaps with those of other entities including pancreatic pseudocyst, other primary epithelial and nonepithelial tumors of the pancreas, and metastases. In most cases, ultrasound and computed tomography are the mainstays for radiologic evaluation, with magnetic resonance imaging having a complementary role. All mucinous cystic neoplasms should be considered as mucinous cystadenocarcinomas of low-grade malignant potential. Complete surgical excision alone results in an excellent clinical outcome and disease-free survival, irrespective of histologic or radiologic parameters in over 90% of cases studied.
PubMed ID: 9536488
Article Size: 1.5 MB
 

Larynx contact ulcer.

Head & Neck, Larynx
Thompson LD.
Ear Nose Throat J. 2005 Jun;84(6):340.
FIRST PARAGRAPH: A larynx contact ulcer, also known as a pyogenic granuloma, is a benign lesion that is most common among adult men. Patients present with hoarseness and/or throat pain, and they often experience chronic throat-clearing or habitual coughing. Contact ulcer is associated with vocal abuse, intubation, and acid regurgitation. Intubation-induced contact ulcer is more common among females, especially in an emergent setting when an inappropriately sized endotracheal tube has been placed. Gastrolaryngeal reflux or gastroesophageal reflux disease (GERD) is frequently missed because physicians do not correlate the nonspecific gastric symptoms with the laryngeal symptoms. A hiatal hernia, peptic esophagitis, or gastritis can cause acid reflux, usually during sleep, thereby leading to the development of a contact ulcer without the cause being obvious to the patient.
PubMed ID: 16075851
Article Size: <1 MB

Nasopharyngeal carcinoma.

Head & Neck, Sinonasal Tract
Thompson L.
Ear Nose Throat J. 2005 Jul;84(7):404-5.
FIRST PARAGRAPH: The most common type of nasopharyngeal tumor is a carcinoma. The etiology of nasopharyngeal carcinoma (NPC) is multifactorial; race, genetics, Epstein-Barr virus (EBV) infection, and the environment all play a role. NPC is rare in white populations, but it is one of the most common cancers among Chinese. EBV is almost always present in NPC, indicating that this virus plays an oncogenic role. The viral titer can be used to monitor therapy or possibly as a diagnostic tool in the evaluation of patients who present with a metastasis from an unknown primary. Exposure to environmental carcinogens, especially high levels of volatile nitrosamines (specifically, those in Cantonese-style salted fish), has been implicated in this complicated disorder; carcinogens related to smoking, formaldehyde exposure, and radiation have also been implicated.
PubMed ID: 16813025
Article Size: <1 MB

Myeloid sarcoma.

Hematopathology, Other Anatomic Sites
Pantanowitz L, Thompson L.
Ear Nose Throat J. 2005 Aug;84(8):470-1.
FIRST PARAGRAPH: Myeloid sarcoma (MS) is an extramedullary myeloid tumor (granulocytic sarcoma) that can occur in one of three clinical settings: (1) in patients who have a history of acute myeloid leukemia (AML), during active disease or a recurrence; (2) in patients with chronic myeloproliferative disorder or myelodysplastic syndromes, who are at increased risk of blast transformation or acute leukemia; or (3) in patients with no history of hematologic disease, although it commonly predates the development of leukemia, often within 1 year.
PubMed ID: 16220848
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

Lymphangioma.

Head & Neck, Neck
Thompson L.
Ear Nose Throat J. 2006 Jan;85(1):18-9.
FIRST PARAGRAPH: Lymphangiomas are rare congenital tumors, with up to 70% reported in the head and neck. They are separated into three types: cystic (cystic hygroma), capillary, and cavernous. Lymphangiomas account for approximately 25% of all vascular neoplasms in children and adolescents. About 25% of cervical cysts are lymphangiomas.
PubMed ID: 16509236
Article Size: <1 MB

Herpes simplex virus laryngitis.

Head & Neck, Larynx
Thompson L.
Ear Nose Throat J. 2006 May;85(5):304.
FIRST PARAGRAPH: Both infectious and inflammatory conditions can result in laryngitis, which is divided into acute and chronic forms. Some conditions are more frequent in adults, while others are more common in children. Laryngitis (‘croup,’ epiglottitis, and laryngotracheobronchitis) has many causes, including viral, bacterial, mycotic, or mycobacterial infections, trauma, neoplasms, vascular compromise during surgery, iatrogenic injury from feeding tubes or tracheostomy tubes, foreign-body impaction, and radiation therapy. A number of etiologies may be present synchronously, such that an infection may develop in association with radiation therapy, for example. Therefore, multiple etiologies may need to be addressed.
PubMed ID: 16771019
Article Size: <1 MB

Olfactory neuroblastoma.

Head & Neck, Sinonasal Tract
Thompson L.
Ear Nose Throat J. 2006 Sep;85(9):569-70.
FIRST PARAGRAPH: Olfactory neuroblastoma (esthesioneuroblastoma) is an uncommon malignant neuroectodermal nasal tumor that accounts for approximately 5% of all malignant neoplasms. Olfactory neuroblastomas are thought to arise from the specialized sensory neuroepithelial (neuroectodermal) olfactory cells that are normally found in the upper part of the nasal cavity, usually including the cribriform plate of the ethmoid sinus. These tumors affect both sexes equally. A bimodal age distribution (the 2nd and 6th decades of life) has been documented, although patients of all ages can be affected. Patients present with nonspecific symptoms of nasal obstruction (70% of cases) and epistaxis (50%); less common symptoms include headache, pain, visual disturbances, and anosmia (<5%). Owing to the nonspecific nature of the presenting symptoms, patients often have a long history prior to diagnosis.
PubMed ID: 17044420
Article Size: <1 MB

Follicular lymphoma.

Head & Neck, Neck
Pantanowitz L, Thompson LD.
Ear Nose Throat J. 2006 Oct;85(10):636-7.
FIRST PARAGRAPH: Follicular lymphoma is defined as a neoplasm of follicle center B lymphocytes (centrocytes and centroblasts) that has at least a partially follicular growth pattern. It is a low-grade lymphoma, and its course is indolent. Most patients present during the sixth decade of life; the neoplasm is very rare in patients younger than 20 years of age. The female-to-male ratio is 1.7:1. Lymph node enlargement is the usual presentation; only about 20% of patients have B symptoms (i.e., fever, chills, night sweats, and weight loss). The disease is usually systemic at presentation (stage III or IV). In the head and neck region, follicular lymphoma may involve lymph nodes, Waldeyer’s ring, and/or skin.
PubMed ID: 17124929
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

Monoclonal origins of malignant mixed tumors (carcinosarcomas). Evidence for a divergent histogenesis.

Endocrine, Pancreas
Thompson L, Chang B, Barsky SH.
Am J Surg Pathol. 1996 Mar;20(3):277-85.
Malignant mixed tumors (carcinosarcomas) are examples of unusual neoplasms whose occurrences have been observed in increasingly diverse sites but whose pathogenesis remains a complete mystery. Two antithetical hypotheses that have been advanced to explain the histogenesis of these tumors include the convergence hypothesis, which proposes an origin from two or more stem cells (multiclonal hypothesis), and the divergence hypothesis, which proposes an origin from a single totipotential stem cell that differentiates into separate epithelial and mesenchymal directions (monoclonal hypothesis). To test these hypotheses, a novel strategy for the determination of clonality from as few as 100 tumor cells obtained by enzymatic digestion of either fresh or formalin-fixed, paraffin-embedded tissues and cell sorting was used that exhibited the polymerase chain reaction (PCR) in amplifying a 511-bp region located within the first intron of the human hypoxanthine phosphoribosyl transferase gene, a site that contains inactive X chromosomal obligately methylated HpaII/MspI sites and single-base allelic polymorphisms in 5% females. Carcinoma cells gated on the basis of fluorescein isothiocyanate (FITC)-anti-cytokeratin and sarcoma cells gated on the basis of FITC-antivimentin or FITC-anti-desmin were sorted to homogeneity on FACSTAR and then subjected to genomic DNA extraction and Hpa II digestion before PCR amplification and subsequent analysis of the product on denaturing gradient gel electrophoresis. The comigrations of the single homoduplexes generated from both the carcinoma cells and sarcoma cells in six different malignant mixed tumors obtained from four different organs indicated clonal identity and monoclonality in all cases. These findings of monoclonality were confirmed independently by two other methods of clonality determination. The findings of a monoclonal origin of carcinosarcomas support the single totipotential stem-cell-divergence hypothesis.
PubMed ID: 8772780
Article Size: 2.5 MB
 

Parathyroiditis.

Endocrine, Parathyroid
Thompson LD.
Ear Nose Throat J. 2005 Oct;84(10):636.
FIRST PARAGRAPH: Parathyroiditis is a rare and poorly understood condition. Chronic parathyroiditis may occur in patients with hypoparathyroidism, as well as in those with primary chief-cell hyperplasia. Most patients are asymptomatic. Antibodies to parathyroid tissue are seen in only a few cases of parathyroiditis. It is thought that parathyroiditis represents an autoimmune process similar to thyroid gland Hashimoto’s disease. The presence of seronegative cases of parathyroiditis does not rule against an autoimmune etiology, because a similar phenomenon is observed in Hashimoto’s thyroiditis. Based on this assumption, it is believed that the lymphocytic infiltration is an ongoing destructive process.
PubMed ID: 16382744
Article Size: <1 MB

Mucinous cystic neoplasm (mucinous cystadenocarcinoma of low-grade malignant potential) of the pancreas: a clinicopathologic study of 130 cases.

Endocrine, Pancreas
Thompson LD, Becker RC, Przygodzki RM, Adair CF, Heffess CS.
Am J Surg Pathol. 1999 Jan;23(1):1-16.
Mucinous cystic neoplasms (MCNs) of the pancreas are uncommon tumors. The classification and biologic potential of these neoplasms remain the subject of controversy. Attempts to classify these tumors in a similar manner to ovarian MCNs remains controversial, as even histologically benign-appearing pancreatic MCNs metastasize and are lethal. One hundred thirty cases of MCNs were identified in the files of the Endocrine Pathology Tumor Registry of the Armed Forces Institute of Pathology from the years 1979 to 1993. The pathologic features, including hematoxylin and eosin staining, histochemistry, immunohistochemistry (IHC), cell cycle analysis, and K-ras oncogene determination were reviewed. These findings were correlated with the clinical follow-up obtained in all cases. There were 130 women, aged 20-95 years (mean age at the outset, 44.6 years). The patients had vague abdominal pain, fullness, or abdominal masses. More than 95% of the tumors were in the pancreatic tail or body and were predominantly multilocular. The tumors ranged in size from 1.5 to 36 cm in greatest dimension, with the average tumor measuring >10 cm. A spectrum of histomorphologic changes were present within the same case and from case to case. A single layer of bland-appearing, sialomucin-producing columnar epithelium lining the cyst wall would abruptly change to a complex papillary architecture, with and without cytologic atypia, and with and without stromal invasion. Ovarian-type stroma was a characteristic and requisite feature. Focal sclerotic hyalinization of the stroma was noted. This ovarian-type stroma reacted with vimentin, smooth muscle actin, progesterone, or estrogen receptors by IHC analysis. There was no specific or unique epithelial IHC. K-ras mutations by sequence analysis were wild type in all 52 cases tested. Ninety percent of patients were alive or had died without evidence of disease (average follow-up 9.5 years), irrespective of histologic appearance; 3.8% were alive with recurrent disease (average 10 years after diagnosis); and 6.2% died of disseminated disease (average 2.5 years from diagnosis). Irrespective of the histologic appearance of the epithelial component, with or without stromal invasion, pancreatic MCNs should all be considered as mucinous cystadenocarcinomas of low-grade malignant potential. Pancreatic MCNs cannot be reliably or reproducibly separated into benign, borderline, or malignant categories.
PubMed ID: 9888699
Article Size: 4 MB
 

A clinicopathologic and immunohistochemical study of ten pancreatic lymphangiomas and a review of the literature.

Endocrine, Pancreas
Paal E, Thompson LD, Heffess CS.
Cancer. 1998 Jun 1;82(11):2150-8. — Erratum in: Cancer 1998 Aug 15;83(4):824.
BACKGROUND: Pancreatic lymphangiomas are rare benign tumors, of which only a few cases have been reported in the literature. In this study, the authors present a series of primary pancreatic lymphangiomas.
METHODS: Cases of nonepithelial pancreatic cystic tumors (lymphangiomas) diagnosed between 1966 and 1994 were retrieved from the Endocrine Pathology Registry of the Armed Forces Institute of Pathology. Histologic features (in 10 cases) as well as histochemical and immunohistochemical studies (in 6 cases) were reviewed. Long term patient follow-up data were obtained in 9 cases.
RESULTS: The patients included 8 females and 2 males ages 2-61 years (mean age, 28.9 years) at initial presentation. The tumors were circumscribed and occurred predominantly (in 6 of 10 cases) in the tail of the pancreas. The multicystic, serous, or chylous fluid-filled cystic tumors ranged from 3 to 20 cm (average, 12.7 cm) in greatest dimension. Histologically, the tumors consisted of multilocular cystic spaces of various sizes, lined by endothelial cells. The stroma contained smooth muscle and mature lymphocytes. Immunohistochemistry determined the endothelial lining cells to be factor VIII-R antigen and CD31 positive (in all cases tested) but usually CD34 negative. All patients for whom follow-up data were obtained (n=9) were alive without evidence of disease an average of 7.2 years after initial diagnosis.
CONCLUSIONS: Pancreatic lymphangiomas occur predominantly in females within a wide age range. Multilocular, fluid-filled cysts, with endothelial immunoreactivity for factor VIII-R antigen and CD31, are characteristic of these tumors. Complete surgical excision of these benign tumors resulted in excellent long term prognoses for all patients studied.
PubMed ID: 9610694
Article Size: <1 MB