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Ceruminous adenoma (ceruminoma) of the external auditory meatus is a rare neoplasm with benign clinical behavior.

 Visit: Ear Pathology Online ; Ceruminous Adenocarcinoma of External Ear

This tumour is mainly composed of the ceruminous gland cells (modified apocrine sweat glands).

These glands are localized deep in the skin mostly in the cartilaginous part of the external auditory meatus.

Clinically, the patients present with a painless mass. The most common symptom of the ceruminous adenoma of external auditory meatus is the unilateral conductive hearing loss. Occasionally symptoms of this tumour (pain, otorrhoea) can result from an otitis externa secondary to meatus obstruction.

Gross feature: Circumscribed polypoid masses, range in size from 0.4 to 2 cm in greatest dimension.

Microscopic features:  Histologically, the tumours demonstrate glands and small cysts lined by a tubuloglandular proliferation of inner ceruminous cells -(cerumen-secreting epithelium with decapitation secretion) and outer spindled myoepithelial cells with hyperchromatic nuclei. The inner cells are cuboidal or columnar cells with eosinophilic cytoplasm and apical snouts and contain yellow-brown granular cerumen pigment. A hyalinized stroma created an infiltrative pattern of growth and surface involvement was seen in some cases.

Cerumen pigment, CK7, and p63 can help to distinguish this tumour from other neoplasms that occur in the region.

Ceruminous adenoma should be radical excised with adequate margins of the normal tissues. Complete surgical excision results in an excellent long-term clinical outcome.

Differential Diagnosis: Ceruminal adenocarcinoma is characterized by more infiltrative pattern , perineural invasion, irregular gland formation, pleomorphism with prominent nucleoli, increased mitotic figures including atypical mitotic figures, tumour necrosis. Usually no ceruminous granules are noted.

                  

Expansion of an ceruminous adenoma into the middle ear. Laryngorhinootologie. 2006 Jun;85(6):444-7.

A 37-year-old female presented for surgery with central perforation of the eardrum with granulation. Mastoidectomy had been performed 18 years ago following chronic mastoiditis. As the clinical picture now suggested a suspected cholesteatoma, radiological imaging was performed. The CT scan revealed specification of the mastoid and the tympanic cavity. In addition, MRI scan showed signal enhancement in the same areas. However, the suspected cholesteatoma could not be confirmed intraoperatively. Pathohistology revealed a ceruminal gland adenoma. They are a rare phenomenon and should be distinguished from middle ear adenomas, pleomorph ceruminal gland adenomas, ceruminal gland adenocarcinomas and cylindromas of the ceruminal glands. Owing to a high recurrence rate, complete surgical removal is necessary. Despite its rare occurrence, a ceruminal gland adenoma must be taken into consideration in the differential diagnosis of individual cholesteatoma cases.

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

Ceruminous gland adenoma of the external auditory canal: a case report.Otolaryngol Pol. 2003;57(5):755-9.

Ceruminous adenoma (ceruminoma) of the external auditory meatus is a rare neoplasm with benign clinical behavior. This tumor is mainly composed of the ceruminous gland cells (modified apocrine sweat glands). These glands are localized deep in the skin mostly in the cartilaginous part of the external auditory meatus. The most often symptom of the ceruminous adenoma of external auditory meatus is the unilateral conductive hearing loss. Occasionally symptoms of this tumor (pain, otorrhoea) can result from an otitis externa secondary to meatus obstruction. Ceruminous adenoma should be radical excised with adequate margins of the normal tissues. The prognosis of these tumors is good. The difficulties of nomenclature, histological structure of the auditory external meatus ceruminoma, as well as symptomatology, treatment and clinical behavior are discussed on the basis of the literature. A case of histologically confirmed ceruminoma (ceruminous adenoma) of the external auditory meatus in a surgically treated 53 years old men is presented.

Ultrastructural morphology of a middle ear ceruminoma.ORL J Otorhinolaryngol Relat Spec. 2002 Sep-Oct;64(5):358-63.

The ultrastructural morphology of a ceruminous gland adenoma in the middle ear was examined electron microscopically. The epithelial tumor cells displayed apocrine caps, microvilli, cell junctions, secretory granules, vacuoles, lipid droplets and siderosomes, which are the characteristic ultrastructural features of apocrine glands. Concentric membranous bodies of the endoplasmic reticulum, phagocytic activity of the tumor cells, intracytoplasmic lumina, ciliated cells and also spiny collagen in the tumor stroma could be seen. The myoepithelial cells are an important tumor marker in the differential diagnosis between ceruminomas and adenomas of the middle ear. The ectopic origin in the modified apocrine ceruminous glands, the specific localization, the clinical features and the extremely rare occurrence of the ceruminoma makes this tumor a unique neoplastic entity.

Ceruminoma revisited. Am J Otol. 1987 Nov;8(6):485-8.

Ceruminoma is a catch-all term that has caused much confusion both in the literature and in clinical practice in regard to the specific histologic diagnosis and proper treatment for tumors arising from the ceruminous glands of the external ear canal. The term ceruminoma has been used in the past to refer to both benign and malignant lesions. To clarify the terminology and better determine appropriate treatment, two cases of benign adenoma of the ceruminous glands along with their histopathologic findings will be presented. The specific characterization of the individual types of ceruminous gland neoplasms, their clinical manifestations, histopathology, and recommended treatment will then be discussed. Finally, suggestions for the appropriate nomenclature for these rare tumors will be reviewed.

                     

 
September 2009

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