Ceruminous adenoma

Ceruminous adenoma
Other names	Adenoma of the ceruminous gland and ceruminoma

Last updated January 15, 2021

A ceruminous adenoma is a benign glandular neoplasm which arises from the ceruminous glands located within the external auditory canal. These glands are found within the outer one third to one half of the external auditory canal, more common along the posterior surface; therefore, the tumor develops within a very specific location.^[2]^[3]^[4]

Signs and symptoms

Ceruminous adenoma are rare tumors, accounting for less than 1% of all external ear tumors. The patients will present with a mass, perhaps associated pain, and may have changes in hearing (usually a sensorineural or a conductive hearing loss). Some patients have tinnitus. Nerve paralysis is very uncommon.^[2]

Pathology findings

The tumors are usually small, with a mean size of about 1.2 cm.^[2] The tumors are separated into three histologic types based on the dominant findings:

Ceruminous adenoma^[2]
Ceruminous pleomorphic adenoma^[2]^[5]
Ceruminous syringocystadenoma papilliferum^[2]

All of the tumors are unencapsulated, but are usually well defined or circumscribed. The overlying surface epithelium is not involved. The tumor shows a dual or biphasic appearance, with glandular or cystic spaces showing inner luminal secretory cells with abundant granular, eosinophilic cytoplasm subtended by basal, myoepithelial cells at the periphery, adjacent to the basement membrane. The luminal cells will often have decapitation (apocrine) secretions and will also have yellow-brown, ceroid, lipofuscin-like (cerumen) pigment granules. There is no pleomorphic, limited mitoses, and no necrosis.^[2]

Immunohistochemistry can be performed to confirm the biphasic nature of the tumor. All cells are positive with pancytokeratin and epithelial membrane antigen; only the luminal cells are positive with CK7; only the basal cells are positive with CK5/6, p63, S100 protein. CD117 can be positive in either population. The cells are negative with chromogranin, synaptophysin and CK20.^[2]

Diagnosis

The major diagnosis from which to separate ceruminous adenoma is ceruminous adenocarcinoma, which shows an infiltrative growth, pleomorphism, mitoses, necrosis, and lacks ceroid pigment granules.^[6] Other tumors which need to be excluded include a neuroendocrine adenoma of the middle ear (middle ear adenoma),^[7] paraganglioma, and endolymphatic sac tumor.^[2]^[8]

Management

The tumors are usually removed in small pieces due to the anatomic confines of the area.^[2]

Prognosis

Patients treated with complete surgical excision can expect an excellent long term outcome without any problems. Recurrences may be seen in tumors which are incompletely excised.^[2]

Epidemiology

While there is a wide age range at clinical presentation (12–85 years), most patients come to clinical attention at 55 years (mean). There is no gender difference.^[2]

Related Research Articles

The parotid gland is a major salivary gland in many animals. In humans, the two parotid glands are present on either side of the mouth and in front of both ears. They are the largest of the salivary glands. Each parotid is wrapped around the mandibular ramus, and secretes serous saliva through the parotid duct into the mouth, to facilitate mastication and swallowing and to begin the digestion of starches. There are also two other types of salivary glands; they are submandibular and sublingual glands. Sometimes accessory parotid glands are found close to the main parotid glands.

An adenoma is a benign tumor of epithelial tissue with glandular origin, glandular characteristics, or both. Adenomas can grow from many glandular organs, including the adrenal glands, pituitary gland, thyroid, prostate, and others. Some adenomas grow from epithelial tissue in nonglandular areas but express glandular tissue structure. Although adenomas are benign, they should be treated as pre-cancerous. Over time adenomas may transform to become malignant, at which point they are called adenocarcinomas. Most adenomas do not transform. However, even though benign, they have the potential to cause serious health complications by compressing other structures and by producing large amounts of hormones in an unregulated, non-feedback-dependent manner. Some adenomas are too small to be seen macroscopically but can still cause clinical symptoms.

Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of parenchymatous glandular cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural Pleomorphism seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which refers to its dual origin from epithelial and myoepithelial elements as opposed to its pleomorphic appearance.

Canalicular adenoma is a benign, epithelial salivary gland neoplasm arranged in interconnecting cords of columnar cells. This is a very rare benign neoplasm, that makes up about 1% of all salivary gland tumors, or about 4% of all benign salivary gland tumors.

Polymorphous low-grade adenocarcinoma (PLGA) is a rare, asymptomatic, slow-growing malignant salivary gland tumor. It is most commonly found in the palate.

Ceruminous glands are specialized sudoriferous glands located subcutaneously in the external auditory canal, in the outer 1/3. Ceruminous glands are simple, coiled, tubular glands made up of an inner secretory layer of cells and an outer myoepithelial layer of cells. They are classed as apocrine glands. The glands drain into larger ducts, which then drain into the guard hairs that reside in the external auditory canal. Here they produce cerumen, or earwax, by mixing their secretion with sebum and dead epidermal cells. Cerumen keeps the eardrum pliable, lubricates and cleans the external auditory canal, waterproofs the canal, kills bacteria, and serves as a barrier to trap foreign particles by coating the guard hairs of the ear, making them sticky.

Salivary gland tumours or neoplasms are tumours that form in the tissues of salivary glands. The salivary glands are classified as major or minor. The major salivary glands consist of the parotid, submandibular, and sublingual glands. The minor salivary glands consist of 800-1000 small mucus-secreting glands located throughout the lining of the oral cavity.

A mixed tumor is a tumor that derives from multiple tissue types. A biplastic tumor or biphasic tumor has two tissue types.

Metanephric adenoma (MA)is a rare, benign tumour of the kidney, that can have a microscopic appearance similar to a nephroblastoma, or a papillary renal cell carcinoma.

Melanotic neuroectodermal tumor of infancy is a very rare oral cavity tumor that is seen in patients usually at or around birth. It must be removed to be cured. Definitions: A rare, biphasic, neuroblastic, and pigmented epithelial neoplasm of craniofacial sites, usually involving the oral cavity or gums.

Fetal adenocarcinoma (FA) of the lung is a rare subtype of pulmonary adenocarcinoma that exhibits tissue architecture and cell characteristics that resemble fetal lung tissue upon microscopic examination. It is currently considered a variant of solid adenocarcinoma with mucin production.

A biphasic disease is a disease which has two distinct phases or components. In clinically biphasic diseases, the phases are generally chronologically separated. In histopathologically biphasic tumors, there is neoplastic tissue which contains two different cellular elements.

Myoepithelioma of the head and neck, also myoepithelioma, is a salivary gland tumour of the head and neck that is usually benign. When malignant, which is exceedingly rare, they are known as malignant myoepithelioma or Myoepithelial carcinoma, and they account for 1% of the salivary tumors with poor prognosis.

Epithelial-myoepithelial carcinoma (EMCa) is a rare malignant tumour that typically arises in a salivary gland and consists of both an epithelial and myoepithelial component. They are predominantly found in the parotid gland and represent approximately 1% of salivary gland tumours.

A sialoblastoma is a low-grade salivary gland neoplasm that recapitulates primitive salivary gland anlage. It has previously been referred to as congenital basal cell adenoma, embryoma, or basaloid adenocarcinoma. It is an extremely rare tumor, with less than 100 cases reported worldwide.

Neuroendocrine adenoma middle ear (NAME) is a tumor which arises from a specific anatomic site: middle ear. NAME is a benign glandular neoplasm of middle ear showing histologic and immunohistochemical neuroendocrine and mucin-secreting differentiation.

An endolymphatic sac tumor (ELST) is a very uncommon papillary epithelial neoplasm arising within the endolymphatic sac or endolymphatic duct. This tumor shows a very high association with von Hippel-Lindau syndrome (VHL).

Ceruminous adenocarcinoma is a malignant neoplasm derived from ceruminous glands of the external auditory canal. This tumor is rare, with several names used in the past. Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), and ceruminous mucoepidermoid carcinoma.

Noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) is an indolent thyroid tumor that was previously classified as an encapsulated follicular variant of papillary thyroid carcinoma, necessitating a new classification as it was recognized that encapsulated tumors without invasion have an indolent behavior, and may be over-treated if classified as a type of cancer.

Ectomesenchymal chondromyxoid tumor (ECT) is a benign intraoral tumor with presumed origin from undifferentiated (ecto)mesenchymal cells. There are some who think it is a myoepithelial tumor type.

References

↑ Mills RG, Douglas-Jones T, Williams RG (Mar 1995). "'Ceruminoma'--a defunct diagnosis". J Laryngol Otol. 109 (3): 180–8. doi:10.1017/s0022215100129652. PMID 7745330.
1 2 3 4 5 6 7 8 9 10 11 12 Thompson LD, Nelson BL, Barnes EL (Mar 2004). "Ceruminous adenomas: a clinicopathologic study of 41 cases with a review of the literature". Am J Surg Pathol. 28 (3): 308–18. doi:10.1097/00000478-200403000-00003. PMID 15104293. S2CID 27571673.
↑ Durko T, Danilewicz M, Pajor A (2003). "[Glandular neoplasms of the external auditory canal--clinical and morphologic observations]". Otolaryngol Pol. 57 (1): 51–7. PMID 12741144.
↑ Hicks GW (Mar 1983). "Tumors arising from the glandular structures of the external auditory canal". Laryngoscope. 93 (3): 326–40. doi:10.1288/00005537-198303000-00016. PMID 6300574. S2CID 31253554.
↑ Markou K, Karasmanis I, Vlachtsis K, Petridis D, Nikolaou A, Vital V (March–April 2008). "Primary pleomorphic adenoma of the external ear canal. Report of a case and literature review". Am J Otolaryngol. 29 (2): 142–6. doi:10.1016/j.amjoto.2007.04.005. PMID 18314029.
↑ Crain N, Nelson BL, Barnes EL, Thompson LD (Mar 2009). "Ceruminous gland carcinomas: a clinicopathologic and immunophenotypic study of 17 cases". Head Neck Pathol. 3 (1): 1–17. doi:10.1007/s12105-008-0095-9. PMC 2807538 . PMID 20596983.
↑ Torske KR, Thompson LD (May 2002). "Adenoma versus carcinoid tumor of the middle ear: a study of 48 cases and review of the literature". Mod Pathol. 15 (5): 543–55. doi: 10.1038/modpathol.3880561 . PMID 12011260.
↑ Lassaletta L, Patrón M, Olóriz J, Pérez R, Gavilán J (Aug 2003). "Avoiding misdiagnosis in ceruminous gland tumours". Auris Nasus Larynx. 30 (3): 287–90. doi:10.1016/S0385-8146(03)00055-5. PMID 12927294.