Intestinal-type sinonasal adenocarcinoma.

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

Intracranial mucocele formation in the context of longstanding chronic rhinosinusitis: A clinicopathologic series and literature review.

Lee JT, Brunworth J, Garg R, Shibuya T, Keschner DB, Vanefsky M, Lin T, Choi S, Stea R3 Thompson LD.
Allergy Rhinol (Providence). 2013 Fall;4(3):e166-75.
Chronic rhinosinusitis (CRS) can lead to serious long-term adverse sequelae, particularly if left untreated. The aim of this study was to describe a series of intracranial mucoceles (ICMs) that arose in the context of longstanding CRS combined with a review of the pertinent literature. A retrospective chart review was performed on all patients who developed ICMs in association with CRS between 2003 and 2012. The clinical presentation, radiographic features, surgical approach, intraoperative findings, and patient outcome were examined in the context of a literature review. Sixty-five cases of mucoceles were identified in patients with a history of CRS, of which seven (10.8%) were intracranial. Five patients were men and two were women with a mean age of 42.1 years. Headache, facial pressure, retro-orbital pain, and visual disturbances were the most common presenting symptoms. Five of the seven had previously undergone sinonasal surgery. Imaging studies showed ICMs involving the anterior cranial fossa, two of which were bilateral. Latency between onset of CRS and ICM detection ranged from 3 to 19 years (mean, 9.4 years). All patients underwent endoscopic transnasal drainage with three also requiring a concurrent, open neurosurgical procedure to access the intracranial component. There were no postoperative complications, and no recurrences were observed after a mean follow-up of 2.7 years. ICMs presenting as delayed complications of CRS are uncommon and constitute a surgical challenge. Open, external skull base approaches used in conjunction with transnasal endoscopic drainage procedures may be necessary to achieve successful management of this rare condition.
PubMed ID: 24498523
Article Size: 2.5 MB
 

Invited Review: An update on salivary gland pathology.

Müller S, Thompson LD.
Head Neck Pathol. 2013 Jul;7 Suppl 1:1-2.
FIRST PARAGRAPH: Over the past few decades, salivary gland tumor pathology has evolved. This includes recognition of newly defined entities as well as reclassification of other salivary gland tumors [1]. The development of genetic tests have shown that some salivary gland tumors have genetic abnormalities which are specific to a histologic type such as MECT1/ MAML2 gene fusion in mucoepidermoid carcinoma and PLAG1 or HMGA2 gene translocation in pleomorphic adenoma. Immunohistochemical studies have aided in both the diagnosis and prognosis of salivary gland tumors. High Ki67 is correlated with poor overall survival in mucoepidermoid carcinoma, acinic cell carcinoma and adenoid cystic carcinoma. High MUC1 in mucoepidermoid carcinoma is associated with higher grade and high recurrence while MUC4 is associated with a lower grade tumor and longer disease free survival. The finding of androgen receptors in salivary duct carcinoma has led to new therapies as these tumors are shown to be responsive to androgen deprivation therapy. Newly described salivary gland entities in the past 20 years include both benign (sclerosing polycystic adenosis, sialolipoma) and malignant (cribriform adenocarcinoma of the tongue, mammary analogue secretory carcinoma) tumors [2]. Newly recognized histologic variants of well-known salivary gland tumors have been reported, to include salivary-duct carcinoma, acinic cell carcinoma and epi-myoepithelial carcinoma.
PubMed ID: 23821215
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Kaposi sarcoma of major salivary gland origin: A clinicopathologic series of six cases.

Castle JT, Thompson LD.
Cancer. 2000 Jan 1;88(1):15-23.
BACKGROUND: Kaposi sarcoma (KS), one of the defining tumors of acquired immune deficiency syndrome (AIDS), is rarely identified in the major salivary glands. To the authors’ knowledge, no previous published series has evaluated the clinicopathologic aspects of this tumor in major salivary glands.
METHODS: Six cases of salivary gland KS, diagnosed between 1970 and 1998, were retrieved from the files of the Oral and Maxillofacial Pathology Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed and special stains, immunohistochemical studies, and in situ hybridization were performed (n = 4). Patient follow-up data were obtained.
RESULTS: The patients included 6 men ages 20-73 years (average, 53.0 years). Patients presented clinically with a mass in the submandibular (n = 4) or parotid (n = 2) gland region. Symptoms were present for a mean of 13.7 months. The tumors measured 1-4 cm (average, 2.5 cm) in greatest dimension. Histologically, the tumors exhibited the usual features of KS: a spindle cell vascular proliferation arranged in fasciculated bundles, variable nuclear pleomorphism, mitotic figures, extravasated erythrocytes, and hyaline globules. Five patients were serologically positive for human immunodeficiency virus (HIV) (three homosexual males, one infected by a contaminated blood transfusion, and one with an unknown risk factor). Human herpesvirus-8 (HHV-8) was present in all cases tested (n = 4). Patients were treated with surgical excision (n = 6), followed by chemotherapy (n = 1) for the single patient with other foci of KS (rectal). Three patients died of AIDS-related infectious complications and one of congestive heart failure, whereas the remaining patients are alive with AIDS but free of salivary gland KS.
CONCLUSIONS: Salivary gland enlargement is frequently identified in HIV positive or AIDS patients. Although rare, it is important to consider KS in the differential diagnosis of other AIDS-related salivary gland manifestations (infections and tumors). Copyright 2000 American Cancer Society.
PubMed ID: 10618601
Article Size: 1 MB
 

Keratocystic odontogenic tumor.

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
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Kimura disease.

Chen H, Thompson LD.
Ear Nose Throat J. 2003 Oct;82(10):763.
FIRST PARAGRAPH: Kimura disease is a rare, chronic inflammatory disorder that involves subcutaneous tissues, predominantly those in the head and neck region. Its etiology is unknown, but it is frequently associated with regional lymphade-nopathy and/or salivary gland involvement. Kimura disease has a predilection for males of Asian descent. Clinically, it can simulate a neoplasm, and most patients have peripheral blood eosinophilia and elevated serum immunoglobulin E (IgE) levels.
PubMed ID: 14606173
Article Size: <1 MB

Kimura disease: a clinicopathologic study of 21 cases.

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
 

Laryngeal angiosarcoma: a clinicopathologic study of five cases with a review of the literature.

Loos BM, Wieneke JA, Thompson LD.
Laryngoscope. 2001 Jul;111(7):1197-202.
OBJECTIVE: Primary laryngeal angiosarcoma (LA) is rare without a reported series evaluating these tumors.
STUDY DESIGN/METHODS: Five patients with LA were retrospectively retrieved from the Otorhinolaryngic Registry of the Armed Forces Institute of Pathology.
RESULTS: Three men and 2 women, aged 29 to 71 years, presented with hoarseness (n = 4) and hemoptysis (n = 1). Two patients had previous neck radiation. The tumors involved the supraglottis (n = 4) with a mean size of 3.1 cm. Histologically, all tumors had anastomosing vascular channels lined by remarkably atypical endothelial cells protruding into the lumen, frequent atypical mitotic figures, and hemorrhage. All cases tested (n = 4) demonstrated immunoreactivity with antibodies to Factor VIII-RA and CD34. All patients had surgery followed by postoperative radiation (n = 3 patients). Three patients died with disease (mean, 17 mo), whereas one patient is alive with no evidence of disease at 18 years.
CONCLUSIONS: LA is a rare tumor, frequently associated with previous radiation, usually involving the supraglottis with characteristic histomorphologic and immunophenotypic features. LA has a poor prognosis, making appropriate separation from other conditions important.
PubMed ID: 11568541
Article Size: <1 MB
 

Laryngeal granular cell tumor.

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

Laryngeal spindle cell squamous cell carcinoma.

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

Laryngeal squamous papilloma.

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

Larynx amyloidosis.

Akst LM, Thompson LD.
Ear Nose Throat J. 2003 Nov;82(11):844-5.
FIRST PARAGRAPH: Amyloidosis is a benign accumulation of extracellular, insoluble, fibrillar protein. In general, the deposition of amyloid may be either localized or systemic and either primary or secondary. Laryngeal amyloidosis is rare, accounting for less than 1% of all benign laryngeal tumors. When it does occur, it is commonly localized and primary. Multifocal disease is present in up to 15% of patients. The disease typically manifests as hoarseness or vocal changes in the fifth to sixth decades, and it affects men and women equally.
PubMed ID: 14661432
Article Size: <1 MB

Larynx contact ulcer.

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

Larynx: nodules and polyps.

Thompson LD.
Ear Nose Throat J. 2013 Sep;92(9):426-8.
FIRST PARAGRAPHS: Nodules usually affect the anterior to middle thirds of the true vocal folds, and they are nearly always bilateral. About 1.5% of the overall population suffers from hoarseness; among the most common causes of hoarseness are vocal fold nodules and polyps. Vocal fold nodules and polyps represent reactive changes of the laryngeal mucosa and adjacent stroma that result in benign nodular or polypoid growths. Their etiology is multifactorial, but vocal misuse is one of the most common causes, followed by infection, smoking, and hypothyroidism. Extroverts are more likely to develop nodules and polyps.
PubMed ID: 24057901
Article Size: <1 MB
 

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

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
 

Lymphangioma.

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

Lymphoid changes of the nasopharyngeal and palatine tonsils that are indicative of human immunodeficiency virus infection. A clinicopathologic study of 12 cases.

Wenig BM, Thompson LD, Frankel SS, Burke AP, Abbondanzo SL, Sesterhenn I, Heffner DK.
Am J Surg Pathol. 1996 May;20(5):572-87.
We report 12 cases in which the histomorphologic changes of the nasopharyngeal tonsils (adenoids) or palatine tonsils suggest infection with the human immunodeficiency virus (HIV). The patients included 10 men and two women, aged 20 to 42 years (median, 33 years). The clinical presentation included airway obstruction, pharyngitis, fever, and a tonsillar or adenoidal mass lesion. Histologic evaluation of the excised adenoids or tonsils in 10 of the cases demonstrated a spectrum of changes including florid follicular hyperplasia, follicle lysis, attenuated mantle zone, and the presence of multinucleated giant cells (MGC). The latter characteristically localized adjacent to the surface or tonsillar crypt epithelium. Two of the 12 cases showed marked lymphoid depletion with absent germinal centers, plasmacytosis, and stromal vascular proliferation. Immunohistochemical evaluation for HIV p24 core protein showed reactivity in 10 of 12 cases localized to follicular dendritic cell network (FDC), the MGC, scattered interfollicular lymphoid cells, and cells identified within the surface or crypt epithelium. Localization of viral RNA by in situ hybridization paralleled the HIV p24 immunohistochemical findings. Additional significant findings included the presence of both CD-68 and S-100 protein in the MGC and the presence of S-100 protein in dendritic cells. Other than HIV, no microorganisms were identified. At the time of presentation, eight patients were not known to be a risk for HIV infection, nor were they known to be HIV infected or suffering from AIDS. In these patients, HIV infection was suspected on the basis of the histologic changes seen in the resected tonsillar and adenoidal tissue. Serologic evaluation (by enzyme-linked immunosorbent assay), confirmed the presence of HIV infection. Our findings suggest the possibility of HIV dissemination through the upper aero-digestive tract mucosa via target cells, such as intraepithelial dendritic cells, submucosal macrophages, and T-lymphocytes. Subsequent presentation of viral antigens to the tonsillar and adenoidal lymphoid tissues results in enlargement of these structures that clinically may simulate a neoplastic proliferation but causes histomorphologic changes that are highly suspicious for HIV infection even in asymptomatic HIV-positive patients.
PubMed ID: 8619422
Article Size: 4 MB
 

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

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
 

Malignant giant cell tumor of the sphenoid.

Chan J, Gannon FH, Thompson LD.
Ann Diagn Pathol. 2003 Apr;7(2):100-5.
Malignant giant cell tumors (MGCTs) of the sphenoid sinus are extremely rare neoplasms. They are challenging to diagnose and difficult to treat because of their skull base location. To the best of our knowledge, we report the first case of a primary MGCT of the sphenoid arising in a patient with Paget’s disease. A 77-year-old man presented with epistaxis and a history of Paget’s disease. There was normal cranial nerve function although radiographic images disclosed a large mass centered in the sphenoid sinus and extending into the ethmoid and maxillary sinuses. Excisional biopsy revealed a MGCT composed of a cellular stroma with increased mitotic activity and necrosis with giant cells present throughout. Additional therapy was declined and the patient died with disease 7 months later. Because of their rarity, no treatment guidelines exist for the management of MGCTs of the sphenoid. We discuss both the diagnostic and therapeutic considerations based on a review of the pertinent literature.
PubMed ID: 12715335
Article Size: <1 MB
 

Management of melanotic neuroectodermal tumor of infancy.

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
 

Melanotic neuroectodermal tumor of infancy.

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

Mesenchymal chondrosarcoma of the sinonasal tract: a clinicopathological study of 13 cases with a review of the literature.

Knott PD, Gannon FH, Thompson LD.
Laryngoscope. 2003 May;113(5):783-90.
OBJECTIVES/HYPOTHESIS: Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Isolated cases have been reported in the English literature, with no large series evaluating the clinicopathological aspects of these tumors.
STUDY DESIGN: Retrospective review. METHODS: Thirteen patients with sinonasal mesenchymal chondrosarcoma were retrieved from the Otorhinolaryngologic-Head and Neck Registry of the Armed Forces Institute of Pathology.
RESULTS: Nine women and 4 men (age range, 11 to 83 y; mean age, 38.8 y) presented with nasal obstruction (n = 8), epistaxis (n = 7), or mass effect (n = 4), or a combination of these. No patients reported prior head and neck irradiation. The maxillary sinus was the most common site of involvement (n = 9), followed by the ethmoid sinuses (n = 7) and the nasal cavity (n = 5). Tumors had an overall mean size of 5.1 cm. Microscopically, the tumors displayed a small, blue, round cell morphology appearance arranged in a hemangiopericytoma-like pattern with foci of cartilaginous matrix. All cases were managed by surgery with adjuvant radiation therapy (n = 4) and/or chemotherapy (n = 3). The overall mean survival was 12.1 years, although five of six patients who developed local recurrences died of disease (mean survival, 6.5 y). Six patients were alive and disease free (mean survival, 17.3 y), and two patients were lost to follow-up.
CONCLUSIONS: Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.
PubMed ID: 12792311
Article Size: 2 MB
 

Metastatic cystic squamous cell carcinoma.

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

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

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
 

Molecular Characterization of Apocrine Salivary Duct Carcinoma

Chiosea SI, Williams L, Griffith CC, Thompson LD, Weinreb I, Bauman JE, Luvison A, Roy S, Seethala RR, Nikiforova MN.
Am J Surg Pathol. 2015 Jun;39(6):744-52.
Contemporary classification and treatment of salivary duct carcinoma (SDC) require its thorough molecular characterization. Thirty apocrine SDCs were analyzed by the Ion Ampliseq Cancer HotSpot panel v2 for mutations in 50 cancer-related genes. Mutational findings were corroborated by immunohistochemistry (eg, TP53, BRAF, ?-catenin, estrogen, and androgen receptors) or Sanger sequencing/SNaPshot polymerase chain reaction. ERBB2 (HER2), PTEN, FGFR1, CDKN2A/P16, CMET, EGFR, MDM2, and PIK3CA copy number changes were studied by fluorescence in situ hybridization. TP53 mutations (15/27, 56%), PTEN loss (11/29, 38%, including 2 cases with PTEN mutation), PIK3CA hotspot mutations (10/30, 33%), HRAS hotspot mutations (10/29; 34%), and ERBB2 amplification (9/29, 31%, including 1 case with mutation) represented the 5 most common abnormalities. There was no correlation between genetic changes and clinicopathologic parameters. There was substantial overlap between genetic changes: 8 of 9 cases with ERBB2 amplification also harbored a PIK3CA, HRAS, and TP53 mutation and/or PTEN loss. Six of 10 cases with PIK3CA mutation also had an HRAS mutation. These findings provide a molecular rationale for dual targeting of mitogen-activated protein kinase and phosphoinositide 3-kinase pathways in SDC. FGFR1 amplification (3/29, 10%) represents a new potential target. On the basis of studies of breast carcinomas, the efficacy of anti-ERBB2 therapy will likely be decreased in SDC with ERBB2 amplification co-occurring with PIK3CA mutation or PTEN loss. Therefore, isolated ERBB2 testing is insufficient for theranostic stratification of apocrine SDC. On the basis of the prevalence and type of genetic changes, apocrine SDC appears to resemble one subtype of breast carcinoma-‘luminal androgen receptor positive/molecular apocrine.’
PubMed ID: 25723113
Article Size: <1 MB
 
 
 
 

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

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
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Mucocele: Retention and extravasation types.

Thompson LD.
Ear Nose Throat J. 2013 Mar;92(3):106-8.
FIRST PARAGRAPHS: The extravasation type is the most common mucocele, more common in children and young adults, with a peak in the second decade of life. The most common non-neoplastic lesion of salivary gland tissue is the mucocele (also called sialocele and ptyalocele). A mucocele is defined as the pooling of mucin in a cystic cavity. Two types of mucoceles are recognized: (1) the retention type, in which the mucin pooling is confined within a dilated excretory duct or cyst, and (2) the extravasation type, in which mucin is spilled into the connective tissues from a ruptured or traumatized salivary gland duct.
PubMed ID: 23532645
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Mucoepidermoid carcinoma.

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
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Nasal glial heterotopia.

Penner CR, Thompson LD.
Ear Nose Throat J. 2004 Feb;83(2):92-3.
FIRST PARAGRAPH: Nasal glial heterotopia (nasal glioma) is the term used to describe a mass made up of mature brain tissue that is isolated from the cranial cavity or spinal canal. Most of these rare, benign, congenital tumors are found in the nasal region, particularly at the bridge of the nose and in the nasal cavity. Nasal glial heterotopia is frequently diagnosed in newborns; a few cases have been found in adults.
PubMed ID: 15008441
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Nasal glial heterotopia: a clinicopathologic and immunophenotypic analysis of 10 cases with a review of the literature.

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
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Nasopharyngeal carcinoma.

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
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Necrotizing sialometaplasia.

Penner CR, Thompson LD.
Ear Nose Throat J. 2003 Jul;82(7):493-4.
FIRST PARAGRAPH: Necrotizing sialometaplasia is a benign, self-limited, reactive inflammatory process that involves salivary glands. It occurs in middle-aged patients; men are affected slightly more often than women. Patients may experience an antecedent event (e.g., surgery, radiation, or trauma) up to 3 weeks before necrotizing sialometaplasia appears clinically, but many are asymptomatic. A submucosal nodular swelling will give way to an ulcerative, crater-like lesion that usually measures less than 3 cm in its greatest dimension.
PubMed ID: 12955830
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Neuroendocrine adenoma of the middle ear.

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
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Nodular fasciitis of the external ear region: a clinicopathologic study of 50 cases.

Thompson LD, Fanburg-Smith JC, Wenig BM.
Ann Diagn Pathol. 2001 Aug;5(4):191-8.
Nodular fasciitis (NF), uncommon in the auricular area, is a benign reactive myofibroblastic proliferation that may be mistaken for a neoplastic proliferation. Fifty cases of NF of the auricular region were identified in the files of the Otorhinolaryngic-Head and Neck Tumor Registry of the Armed Forces Institute of Pathology. The patients included 22 females and 28 males, aged 1 to 76 years (mean, 27.4 years). The patients usually presented clinically with a mass lesion (n = 49). Five patients recalled antecedent trauma. The lesions were dermal (n = 28) or subcutaneous (n = 11) in those cases where histologic determination was possible, measuring 1.9 cm on average. The majority of the lesions were circumscribed (n = 38), composed of spindle-shaped to stellate myofibroblasts arranged in a storiform growth pattern, juxtaposed to hypocellular myxoid tissue-culture-like areas with extravasation of erythrocytes. Dense, keloid-like collagen and occasional giant cells were seen (n = 18). Mitotic figures (without atypical forms) were readily identifiable. By immunohistochemical staining, myofibroblasts were reactive with vimentin, actins, and CD68. All patients had surgical excision. Four patients (9.3%) developed local recurrence and were alive and disease free at last follow-up. All patients with follow-up (n = 43) were alive or had died of unrelated causes, without evidence of disease an average 13.4 years after diagnosis. Nodular fasciitis of the auricular area occurs most often in young patients. Because NF is more often dermally situated than extremity NF, it may present with superficial ulceration and/or bleeding. Local recurrence is more frequent because of the difficulty in obtaining complete surgical excision around the ear.
PubMed ID: 11510001
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Nodular fasciitis.

Thompson LD.
Ear Nose Throat J. 2002 Dec;81(12):830.
FIRST PARAGRAPH: Nodular fasciitis is generally regarded as a benign, reactive, tumor-like proliferation of myofibroblasts. It quite commonly occurs in the head and neck region in young patients shortly after they develop an enlarging mass (ulceration is infrequent). In some cases, nodular fasciitis is associated with antecedent trauma.
PubMed ID: 12516376
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Novel PRKD Gene Rearrangements and Variant Fusions in Cribriform Adenocarcinoma of Salivary Gland Origin

Weinreb I, Zhang L, Tirunagari LM, Sung YS, Chen CL, Perez-Ordonez B, Clarke BA, Skalova A, Chiosea SI, Seethala RR, Waggott D, Boutros PC, How C, Liu FF, Irish JC, Goldstein DP, Gilbert R, Ud Din N, Assaad A, Hornick JL, Thompson LD, Antonescu CR.
Genes Chromosomes Cancer. 2014 Oct;53(10):845-56.
Polymorphous low-grade adenocarcinoma (PLGA) and cribriform adenocarcinoma of minor salivary gland (CAMSG) are low-grade carcinomas arising most often in oral cavity and oropharynx, respectively. Controversy exists as to whether these tumors represent separate entities or variants of one spectrum, as they appear to have significant overlap, but also clinicopathologic differences. As many salivary carcinomas harbor recurrent translocations, paired-end RNA sequencing and FusionSeq data analysis was applied for novel fusion discovery on two CAMSGs and two PLGAs. Validated rearrangements were then screened by fluorescence in situ hybridization (FISH) in 60 cases. Histologic classification was performed without knowledge of fusion status and included: 21 CAMSG, 18 classic PLGA, and 21 with ‘mixed/indeterminate’ features. The RNAseq of 2 CAMSGs showed ARID1A-PRKD1 and DDX3X-PRKD1 fusions, respectively, while no fusion candidates were identified in two PLGAs. FISH for PRKD1 rearrangements identified 11 additional cases (22%), two more showing ARID1A-PRKD1 fusions. As PRKD2 and PRKD3 share similar functions with PRKD1 in the diacylglycerol and protein kinase C signal transduction pathway, we expanded the investigation for these genes by FISH. Six additional cases each showed PRKD2 and PRKD3 rearrangements. Of the 26 (43%) fusion-positive tumors, there were 16 (80%) CAMSGs and 9 (45%) indeterminate cases. A PRKD2 rearrangement was detected in one PLGA (6%). We describe novel and recurrent gene rearrangements in PRKD1-3 primarily in CAMSG, suggesting a possible pathogenetic dichotomy from ‘classic’ PLGA. However, the presence of similar genetic findings in half of the indeterminate cases and a single PLGA suggests a possible shared pathogenesis for these tumor types.
PubMed ID: 24942367
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Nuclear expression and gain-of-function β-catenin mutation in glomangiopericytoma (sinonasal-type hemangiopericytoma): insight into pathogenesis and a diagnostic marker.

Lasota J, Felisiak-Golabek A, Aly FZ, Wang ZF, Thompson LD, Miettinen M.
Mod Pathol. 2015 May;28(5):715-20.
Glomangiopericytoma (sinonasal-type hemangiopericytoma) is a rare mesenchymal neoplasm with myoid phenotype (smooth muscle actin-positive), which distinguishes this tumor from soft tissue hemangiopericytoma/solitary fibrous tumor. Molecular genetic changes underlying the pathogenesis of glomangiopericytoma are not known. In this study, 13 well-characterized glomangiopericytomas were immunohistochemically evaluated for β-catenin expression. All analyzed tumors showed strong expression and nuclear accumulation of β-catenin. Following this observation, β-catenin glycogen serine kinase-3 beta phosphorylation region, encoded by exon 3, was PCR amplified in all cases and evaluated for mutations using Sanger sequencing. Heterozygous mutations were identified in 12 of 13 tumors. All mutations consisted of single-nucleotide substitutions: three in codon 32 (c.94G>C (n=2) and c.95A>T), four in codon 33 (two each c.98C>G and c.98C>T), two in codon 37 (c.109T>G), one in codon 41 (c.121A>G), and two in codon 45 (c.133T>C). At the protein level, these substitutions would lead to p.D32H, p.D32V, p.S33C, p.S33F, p.S37A, p.T41A, and p.S45L mutations, respectively. Previously, similar mutations have been reported in different types of cancers and shown to trigger activation of β-catenin signaling. All analyzed glomangiopericytomas showed prominent nuclear expression of cyclin D1, as previously shown for tumors with nuclear expression of β-catenin as a sign of oncogenic activation. These results demonstrate that mutational activation of β-catenin and associated cyclin D1 overexpression may be central events in the pathogenesis of glomangiopericytoma. In additon, nuclear accumulation of β-catenin is a diagnostic marker for glomangiopericytoma.
PubMed ID: 25431235
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Olfactory neuroblastoma.

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
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Olfactory neuroblastoma.

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
 
 
 
 

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

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
 

Oncocytomas of the submandibular gland. A series of 22 cases and a review of the literature.

Thompson LD, Wenig BM, Ellis GL.
Cancer. 1996 Dec 1;78(11):2281-7.
BACKGROUND: Oncocytomas are benign salivary gland neoplasms that represent approximately 1.5% of all salivary gland tumors. Oncocytomas of the submandibular gland, however, are decidedly uncommon.
METHODS: Twenty-two cases of submandibular gland oncocytomas from the files of the Oral and Otolaryngic Tumor Registries of the Armed Forces Institute of Pathology were reviewed, and analysis of the histologic criteria, histochemical and immunohistochemical reactions, and ultrastructural and clinical follow-up data was performed.
RESULTS: The patients included 11 females and 11 males, age 21-88 years, with a mean age at presentation of 58.7 years. Clinically, the tumors were generally asymptomatic masses in the submandibular gland that increased in size over a period ranging from several weeks to 20 years and were occasionally associated with pain (n = 9). The tumors ranged in greatest dimension from 0.7 to 7 cm and were circumscribed to encapsulated. Histologically, the tumors were characterized by large epithelial cells with eosinophilic, granular cytoplasm. The cytoplasm stained positively with stains used to demonstrate mitochondria (phosphotungstic acid-hematoxylin, Novelli, Cresylecht violet V, and Kluver-Barrera Luxol fast blue stains). Immunohistochemical reactions demonstrated an epithelial origin (keratin and epithelial membrane antigen), whereas markers for myoepithelial derivation (S-100 protein, actin, and glial fibrillary acidic protein) were not identified. At the time this study was conducted, all patients with submandibular oncocytomas were either alive without evidence of disease or had died without evidence of recurrent disease, with surgical resection the only treatment.
CONCLUSIONS: Submandibular gland oncocytomas are rare, benign tumors. The tumor cells are filled with mitochondria, which are easily demonstrated by histochemical reactions. Complete surgical resection is adequate therapy.
PubMed ID: 8940996
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Oral Alveolar Soft Part Sarcoma in Childhood and Adolescence: Report of Two Cases and Review of Literature.

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
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Oral cicatricial pemphigoid.

Nelson BL, Thompson LD.
Ear Nose Throat J. 2004 Jan;83(1):22.
FIRST PARAGRAPH: Cicatricial pemphigoid is a vesiculobullous disease of the skin that may be found in the oral cavity. Previously designated ‘benign mucous membrane pemphigoid,’ cicatricial pemphigoid is a chronic, blistering, autoimmune disease that affects mucous membranes. Tissue-bound autoantibodies are directed against one or more components of the basement membrane in an affected individual. Cicatricial pemphigoid initially occurs in the fifth to seventh decades of life, and it is observed more frequently in women. Patients usually describe oral pain and/or ulceration, often of many years’ duration. Clinically, the disease is characterized by the formation of bullae, which can be found anywhere in the oral cavity. The bullae rupture and produce ulceration, which may cause a scar (cicatrix) upon healing.
PubMed ID: 14986753
Article Size: <1 MB

Oral lichen planus.

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
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Ossifying fibroma of the jaw.

Gannon FH, Thompson L.
Ear Nose Throat J. 2004 Jul;83(7):458.
FIRST PARAGRAPH: Ossifying fibroma of the jaw is a benign, fibro-osseous lesion that is part of a larger family of fibro-osseous lesions that includes juvenile active ossifying fibroma, psammomatous ossifying fibroma, and extragnathic ossifying fibroma of the skull. Ossifying fibromas of the jaw are well-circumscribed, slowly growing lesions. They are often mentioned in the same differential diagnosis as fibrous dysplasia, but it is important to make the distinction because the former lends itself to ready enucleation, while the latter can be admixed with surrounding tissues, making surgery more complicated.
PubMed ID: 15372914
Article Size: <1 MB

Osteosarcoma.

Thompson LD.
Ear Nose Throat J. 2013 Jul;92(7):288-90.
FIRST PARAGRAPHS: Osteosarcoma affects the mandible and the maxilla differently, with mandibular tumors tending to arise from the body of the mandible while maxillary tumors arise from the alveolar ridge and sinus. Osteosarcoma is a mesenchymal malignancy in which the neoplastic cells synthesize and secrete the organic components of bone matrix. While it is the most common primary tumor of bone, it is very uncommon overall.
PubMed ID: 23904301
Article Size: <1 MB

Otic polyp.

Thompson LD.
Ear Nose Throat J. 2012 Nov;91(11):474-5.
FIRST PARAGRAPHS: An uncommon entity, otic polyp arises in response to a long-standing inflammatory or infectious process of the middle ear, most often in young boys. An otic, or aural, polyp is a benign proliferation of chronic inflammatory cells and granulation tissue that is usually lined with benign reactive epithelium. These lesions arise in response to a long-standing inflammatory or infectious process of the middle ear. Otic polyps are uncommon; when they do occur, they usually affect young patients. They are more common in males than in females.
PubMed ID: 23288791
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Papillary cystadenoma lymphomatosum (Warthin tumor).

Rizzi MD, Thompson LD.
Ear Nose Throat J. 2003 Dec;82(12):920-2.
FIRST PARAGRAPH: Papillary cystadenoma lymphomatosum (Warthin tumor, adenolymphoma) is a benign salivary gland tumor that occurs almost exclusively in the parotid gland. It represents 5 to 6% of all salivary gland tumors, and it is the second most common benign parotid neoplasm. Men are affected more often than women, usually in the fifth to seventh decades of life, although this gender proportion is changing. Warthin tumor is associated with smoking. The most common clinical manifestation is a painless, slowly growing mass in the inferior pole of the superficial lobe of the parotid gland, usually at the level of the mandibular angle. Multifocality occurs in up to 14% of cases; when two salivary gland neoplasms are present synchronously, Warthin tumor is the most common second tumor.
PubMed ID: 14702874
Article Size: <1 MB

Papillary Cystadenoma of Minor Salivary Glands: Report of 11 Cases and Review of the English Literature.Cases and Review of the English Literature.

Tjioe KC, de Lima HG, Thompson LD, Lara VS, Damante JH, de Oliveira-Santos C.
Head Neck Pathol. 2015 Sep;9(3):354-9.
Papillary cystadenoma is a rare, benign salivary gland tumor which is well-circumscribed, containing cystic cavities with intraluminal papillary projections. Only 19 cases arising within minor salivary glands (MnSG) from the oral cavity sites have been reported in the English literature (PubMed 1958-2014). We report 11 new cases of MnSG papillary cystadenomas in conjunction with a review of the literature. Demographic information, clinical and histologic features, treatment and prognosis are compiled and discussed for all 30 cases reported in the English literature.
PubMed ID: 25547059
Article Size: 1.2 MB