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

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

Adenoma versus carcinoid tumor of the middle ear: a study of 48 cases and review of the literature.

Torske KR, Thompson LD.
Mod Pathol. 2002 May;15(5):543-55.
Carcinoid tumors and adenomas of the middle ear are rare neoplasms of indeterminate relationship to one another. Indeed, the literature is devoid of a large comprehensive series that evaluates the clinical, histologic, and immunophenotypic features of these tumors and their potential relationship. Forty-eight cases of middle ear adenoma between 1970 and 1995 were identified in the files of the Armed Forces Institute of Pathology. All cases were evaluated for cytomorphology and architectural pattern, in addition to their reactivity with various immunohistochemical reagents. Clinical follow-up was also obtained. A comprehensive review of the literature was performed with an eye toward correlating any distinct differences or similarities between carcinoid tumors and adenomas of the middle ear. The patients included 21 women and 27 men, aged 20 to 80 years (mean, 45.0 y). Patients experienced hearing loss, mass, and/or pain for a mean duration of 1.7 years. The mean tumor size was 0.8 cm, with six tumors extending beyond the middle ear. Histologically, the tumors were moderately cellular and unencapsulated, arranged in glandular, trabecular, and solid patterns composed of small cells with ‘salt and pepper’ nuclear chromatin distribution. The tumor cells were immunoreactive with keratin, keratin 7, chromogranin, and human pancreatic polypeptide. All patients had surgery. No patients died with their disease (mean follow-up, 15.7 y). Eight patients developed recurrences that were treated surgically and were without evidence of disease at last follow-up (mean, 15.1 y). Our study and the review of the literature showed adenomas and carcinoid tumors of the middle ear to be essentially indistinguishable benign tumors. Middle ear adenoma most correctly describes their morphologic features and clinical behavior, although neuroendocrine adenoma of the middle ear may be a more accurate designation.
PubMed ID: 12011260
Article Size: 3 MB

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

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

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
Article Size: <1 MB

Update From the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Tumours of the Ear.

Thompson LD.
Head Neck Pathol. 2017 Mar;11(1):78-87. doi: 10.1007/s12105-017-0790-5. Epub 2017 Feb 28.
The 2017 fourth edition of the World Health Organization Classification of Tumours, specifically as it relates to the ear (Chap. 9), has several changes. Importantly, the number of entities has been significantly reduced by omitting tumors or lesions if they do not occur exclusively or predominantly at this site or if they are discussed in detail elsewhere in the book. These entities include: embryonal rhabdomyosarcoma, osteoma, exostosis, angiolymphoid hyperplasia with eosinophilia, Schneiderian papilloma, inverted papilloma, lipoma of the internal auditory canal, hemangioma, hematolymphoid tumors, and secondary tumors. Paraganglioma was included in the neck chapter. New entries include otosclerosis and cholesteatoma, while refinements to nomenclature, classification and criteria were incorporated into the ceruminous gland tumors and epithelial tumors of the middle and inner ear. Specifically, the middle and inner ear were combined, as practical limitations of origin and imaging make a definitive separation artificial. The classification reflects the state of current understanding for these uncommon entities, with this update only highlighting selected entities that were the most significantly changed.
PubMed ID: 28247225
Article Size: 4.6 MB

A clinicopathological study of 15 patients with neuroglial heterotopias and encephaloceles of the middle ear and mastoid region.

Gyure KA, Thompson LD, Morrison AL.
Laryngoscope. 2000 Oct;110(10 Pt 1):1731-5.
OBJECTIVES: Heterotopic masses of neuroglial tissue involving non-midline structures, specifically, the middle ear region, are exceptional. The pathogenesis of these lesions and, in particular, their relation to encephaloceles, is uncertain.
STUDY DESIGN AND METHODS: H&E-stained sections from 15 lesions diagnosed as neuroglial heterotopias or encephaloceles involving the middle ear region were reviewed. Radiographic or operative evidence of a central nervous system (CNS) relation and clinical factors possibly related to pathogenesis were analyzed.
RESULTS: All 15 lesions (from six men and nine women; mean age, 49 y; range, 16-67 y), regardless of their relation to the CNS, were composed of varying proportions of neurons and glia with associated chronic inflammatory cells and reactive gliosis. No significant ependymal or choroid plexus component was present. Operative findings revealed that two lesions had definite CNS connections and two were unrelated to the CNS; this relation could not be determined in the remaining cases. Seven of 10 patients for whom clinical information was available had a history of chronic otitis media or mastoiditis or both; four of these seven patients also had a history of previous trauma or surgery. Three patients, including both patients whose lesions had no demonstrable CNS attachment, had no predisposing factors.
CONCLUSIONS: Most neuroglial heterotopias of the middle ear are probably acquired encephaloceles. These lesions occur in older patients than do their midline counterparts. Determination of the relation of these lesions to adjacent CNS structures must be done radiographically or using operative findings, because histology alone cannot be reliably used to render an accurate diagnosis.
PubMed ID: 11037835
Article Size: 1 MB

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

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

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

Ear fetal rhabdomyoma.

Thompson LD.
Ear Nose Throat J. 2017 Sep;96(9):358.
FIRST PARAGRAPH: Rhabdomyomas are a group of benign tumors that show skeletal muscle differentiation. They are divided into cardiac and extracardiac types with the extracardiac type divided into adult, fetal, and genital types. The fetal type is separated into myxoid and intermediate types. There is a predominance in the head and neck of both the fetal and adult types. The fetal type is commonly seen in newborns and early childhood, with a moderate male predominance (2:1).
PubMed ID: 28931187
Article Size: <1 MB

Angiolymphoid hyperplasia with eosinophilia.

Thompson LD.
Ear Nose Throat J. 2015 Oct-Nov;94(10-11):443-4.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular tumor that features immature blood vessels lined by epithelioid endothelial cells with a prominent inflammatory infiltrate, frequently showing a conspicuous eosinophil component.

FIRST PARAGRAPH: Angiolymphoid hyperplasia with eosinophilia (ALHE), sometimes called epithelioid hemangioma, is a benign vascular tumor. It features immature blood vessels lined by epithelioid endothelial cells with a prominent inflammatory infiltrate, frequently showing a conspicuous eosinophil component. There is controversy about whether this lesion is a reactive or benign neoplastic condition.

PubMed ID: 26535818
Article Size: <1 MB

Endolymphatic sac tumor.

Thompson LD.
Ear Nose Throat J. 2013 Apr-May;92(4-5):184-8.
FIRST PARAGRAPH: Endolymphatic sac tumor (ELST) is a papillary epithelial neoplasm arising within the endolymphatic sac/duct that shows a high association with von Hippel-Lindau disease (VHL). There is usually a VHL tumor suppressor gene germline mutation with an autosomal dominant inheritance pattern. Approximately 1 in 35,000 to 40,000 people have VHL, of which approximately 10 to 15% have endolymphatic sac tumors. There is a wide age range at presentation, although most patients are between 30 and 40 years; there is no gender predilection.
PubMed ID: 23599098
Article Size: <1 MB

Chondrodermatitis nodularis helicis.

Thompson LDR.
Ear Nose Throat J. 2007 Dec;86(12):734-5.
FIRST PARAGRAPH: Chondrodermatitis nodularis helicis (CDNH) is a non-neoplastic inflammatory and degenerative process of the external ear. It is characterized by necrobiotic changes in the dermis that extend down to the perichondrium; associated alterations are seen in the cartilaginous plate. The dermal injury is thought to be attributable to a combination of factors: local trauma, actinic damage, and the relatively tenuous vascularity of the auricle. The necrobiotic dermal collagen—and in some cases the cartilaginous matrix—is extruded through a crater-like defect in the epidermis; thus, CDNH is considered to be one of the transepidermal elimination disorders.
PubMed ID: 18217375
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
Article Size: <1 MB

Ear ceruminous adenoma.

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

Cystic chondromalacia of the ear.

Nelson BL, Thompson LD.
Ear Nose Throat J. 2003 Feb;82(2):104-5.
FIRST PARAGRAPH: Idiopathic cystic chondromalacia (endochondral pseudocyst of the auricle) is a benign cystic degenerative lesion of the auricular cartilage. The lesion appears as a painless unilateral swelling along the upper half of the ear, usually in the area of the scaphoid or triangular fossae adjacent to the helix. The disorder affects young and middle-aged men more often than it does women. Trauma is an associated, although not proven, etiologic factor.
PubMed ID: 12619465
Article Size: <1 MB

Relapsing polychondritis.

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

Embryonal rhabdomyosarcoma of the ear.

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


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

Temporal bone schwannoma.

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


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

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
Article Size: <1 MB

Ear and temporal bone meningioma

Thompson, LD.
Ear Nose Throat J. 2016 Apr-May;95(4-5):146.
FIRST PARAGRAPH: Meningiomas account for 30% of all intracranial neoplasms, but primary extracranial (ectopic) meningiomas of the ear and temporal bone are less common, accounting for about 10% of all ear and temporal bone tumors. Meningiomas are derived from the arachnoid cap cells, also called pacchionian bodies.
PubMed ID: 27140011
Article Size: <1 MB