Ceruminous adenocarcinoma

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Ceruminous adenocarcinoma
Other namesCylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), [1] [2] ceruminous mucoepidermoid carcinoma
Ceruminous gland adenoid cystic carcinoma hematoxlin and eosin.tif
A high power photomicrograph of a ceruminous type adenoid cystic carcinoma
Specialty ENT surgery

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. [3] [4] Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), [1] [2] and ceruminous mucoepidermoid carcinoma.

Contents

Classification

This tumor only affects the outer 1/3 to 1/2 of the external auditory canal as a primary site. If this area is not involved, the diagnosis should be questioned. The most common tumor type is ceruminous adenoid cystic carcinoma and ceruminous adenocarcinoma, NOS.

Signs and symptoms

Pain is the most common symptom, followed by either sensorineural or conductive hearing loss, tinnitus or drainage (discharge). A mass lesion may be present, but it is often slow growing. [3]

Diagnosis

Imaging

Imaging studies are used to define the extent of the tumor and to exclude direct extension from the parotid gland or nasopharynx. [5] The imaging findings are usually non-specific, and cannot give a specific diagnosis.

Pathology

Tumors are polypoid, identified most often in the posterior canal. It is not uncommon to have ulceration of the surface squamous epithelium. Most tumors are about 1.5 cm in greatest dimension, a limitation of the anatomic site rather than of the tumor type itself. [3] The tumors are separated into three main histologic or microscopic types:

All of the tumors are infiltrative into the soft tissue, benign ceruminous glands, and/or bone. The tumor may expand into the overlying squamous surface epithelium, but it usually does not arise from the surface epithelium. The tumors are cellular, arranged in solid, cystic, cribriform, glandular, and single cell patterns. It is uncommon to see tumor necrosis, but when it is present, it is diagnostic of cancer. The same is true of perineural invasion. Nuclear pleomorphism is usually easily to identify, with the nuclei containing prominent nucleoli. There are usually increased mitotic figures, including atypical forms. There are usually areas of stromal fibrosis. Ceroid (cerumen or ear wax) is not seen in malignancies, although it is seen in benign tumors. The specific features of each tumor type can help with the separation into adenoid cystic carcinoma or mucoepidermoid types. [3]

Immunohistochemistry

Immunohistochemistry will help to show the biphasic appearance of the tumor, highlighting the basal or the luminal cells:

Differential diagnoses

It is important to exclude a tumor which is directly extending into the ear canal from the parotid salivary gland, especially when dealing with an adenoid cystic or mucoepidermoid carcinoma. This can be eliminated by clinical or imaging studies. Otherwise, the histologic differential diagnosis includes a ceruminous adenoma (a benign ceruminous gland tumor) [7] [8] or a neuroendocrine adenoma of the middle ear (middle ear adenoma). [9]

Management

Wide, radical, complete surgical excision is the treatment of choice, with free surgical margins to achieve the best outcome and lowest chance of recurrence. Radiation is only used for palliation. In general, there is a good prognosis, although approximately 50% of patients die from disease within 3–10 years of presentation. [3]

Epidemiology

This is a very rare neoplasm accounting for approximately 0.0003% of all tumors and about 2.5% of all external ear neoplasms. [3] Ceruminous adenocarcinomas (CA) is a rare type of tumor and has a very small amount of literature on it. The ages to develop ceruminous adenocarcinomas are between 21 and 92 and being most present around 48. [10] There is no statistical difference between being male and female and developing the tumor [10] and there is no racial inclination [11] . The typical treatment of CA is through surgery and radiation. The type of cut is important with the recurrence rate. [10] Long-term outcomes are better when having the wide cut near the tumor. [10] If the tumor reoccurs there is an 83% chance of mortality and if it does not reoccur there is a 9% chance of mortality. [10] Older people have a lower survival rate than younger ones. [12] The local reoccurrence rate is 49% and the distant metastasis rate is 13%. [11] With local reoccurrence being at 49%, follow-up is very important. [11]

Related Research Articles

Adenocarcinoma Medical condition

Adenocarcinoma (AC) is a type of cancerous tumor that can occur in several parts of the body. It is defined as neoplasia of epithelial tissue that has glandular origin, glandular characteristics, or both. Adenocarcinomas are part of the larger grouping of carcinomas, but are also sometimes called by more precise terms omitting the word, where these exist. Thus invasive ductal carcinoma, the most common form of breast cancer, is adenocarcinoma but does not use the term in its name—however, esophageal adenocarcinoma does to distinguish it from the other common type of esophageal cancer, esophageal squamous cell carcinoma. Several of the most common forms of cancer are adenocarcinomas, and the various sorts of adenocarcinoma vary greatly in all their aspects, so that few useful generalizations can be made about them.

Parotid gland Major salivary gland in many animals

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.

Adenoid cystic carcinoma Medical condition

Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, bartholin gland, trachea, and the paranasal sinuses.

The International Classification of Diseases for Oncology (ICD-O) is a domain-specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. This classification is widely used by cancer registries.

Canalicular adenoma Benign salivary gland tumor

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.

Myoepithelial cells are cells usually found in glandular epithelium as a thin layer above the basement membrane but generally beneath the luminal cells. These may be positive for alpha smooth muscle actin and can contract and expel the secretions of exocrine glands. They are found in the sweat glands, mammary glands, lacrimal glands, and salivary glands. Myoepithelial cells in these cases constitute the basal cell layer of an epithelium that harbors the epithelial progenitor. In the case of wound healing, myoepithelial cells reactively proliferate. Presence of myoepithelial cells in a hyperplastic tissue proves the benignity of the gland and, when absent, indicates cancer. Only rare cancers like adenoid cystic carcinomas contains myoepithelial cells as one of the malignant component.

Glandular odontogenic cyst Human jaw cyst

A glandular odontogenic cyst (GOC) is a rare and usually benign odontogenic cyst developed at the odontogenic epithelium of the mandible or maxilla. Originally, the cyst was labeled as "sialo-odontogenic cyst" in 1987. However, the World Health Organization (WHO) decided to adopt the medical expression "glandular odontogenic cyst". Following the initial classification, only 60 medically documented cases were present in the population by 2003. GOC was established as its own biological growth after differentiation from other jaw cysts such as the "central mucoepidermoid carcinoma (MEC)", a popular type of neoplasm at the salivary glands. GOC is usually misdiagnosed with other lesions developed at the glandular and salivary gland due to the shared clinical signs. The presence of osteodentin supports the concept of an odontogenic pathway. This odontogenic cyst is commonly described to be a slow and aggressive development. The inclination of GOC to be large and multilocular is associated with a greater chance of remission. GOC is an infrequent manifestation with a 0.2% diagnosis in jaw lesion cases. Reported cases show that GOC mainly impacts the mandible and male individuals. The presentation of GOC at the maxilla has a very low rate of incidence. The GOC development is more common in adults in their fifth and sixth decades.

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 tumour Medical condition

Salivary gland tumours also known as mucous gland adenomas 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. Patients with these types of tumours may be asymptomatic.

Mucinous cystadenoma Medical condition

Mucinous cystadenoma is a benign cystic tumor lined by a mucinous epithelium. It is a type of cystic adenoma (cystadenoma).

Eccrine carcinoma Medical condition

Eccrine carcinoma is a rare skin condition characterized by a plaque or nodule on the scalp, trunk, or extremities. It originates from the eccrine sweat glands of the skin, accounting for less than 0.01% of diagnosed cutaneous malignancies. Eccrine carcinoma tumors are locally aggressive, with a high rate of recurrence. Lack of reliable immunohistochemical markers and similarity to other common tumors has made identification of eccrine carcinoma difficult.

Epithelial-myoepithelial carcinoma of the lung is a very rare histologic form of malignant epithelial neoplasm ("carcinoma") arising from lung tissue.

Mucinous cystadenocarcinoma of the lung (MCACL) is a very rare malignant mucus-producing neoplasm arising from the uncontrolled growth of transformed epithelial cells originating in lung tissue.

Odontogenic cyst are a group of jaw cysts that are formed from tissues involved in odontogenesis. Odontogenic cysts are closed sacs, and have a distinct membrane derived from rests of odontogenic epithelium. It may contain air, fluids, or semi-solid material. Intra-bony cysts are most common in the jaws, because the mandible and maxilla are the only bones with epithelial components. That odontogenic epithelium is critical in normal tooth development. However, epithelial rests may be the origin for the cyst lining later. Not all oral cysts are odontogenic cyst. For example, mucous cyst of the oral mucosa and nasolabial duct cyst are not of odontogenic origin.
In addition, there are several conditions with so-called (radiographic) 'pseudocystic appearance' in jaws; ranging from anatomic variants such as Stafne static bone cyst, to the aggressive aneurysmal bone cyst.

Epithelial-myoepithelial carcinoma Medical condition

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.

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.

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.

Endolymphatic sac tumor

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).

Vulvar tumors are those neoplasms of the vulva. Vulvar and vaginal neoplasms make up a small percentage (3%) of female genital cancers. They can be benign or malignant. Vulvar neoplasms are divided into cystic or solid lesions and other mixed types. Vulvar cancers are those malignant neoplasms that originate from vulvar epithelium, while vulvar sarcomas develop from non-epithelial cells such as bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Epithelial and mesenchymal tissue are the origin of vulvar tumors.

References

  1. 1 2 Dong, F.; Gidley, P. W.; Ho, T.; Luna, M. A.; Ginsberg, L. E.; Sturgis, E. M. (2008). "Adenoid Cystic Carcinoma of the External Auditory Canal". The Laryngoscope. 118 (9): 1591–1596. doi:10.1097/MLG.0b013e31817c42a8. PMID   18677277. S2CID   20216496.
  2. 1 2 Perzin, K. H.; Gullane, P.; Conley, J. (1982). "Adenoid cystic carcinoma involving the external auditory canal. A clinicopathologic study of 16 cases". Cancer. 50 (12): 2873–2883. doi:10.1002/1097-0142(19821215)50:12<2873::aid-cncr2820501230>3.0.co;2-r. PMID   6291744.
  3. 1 2 3 4 5 6 7 Crain, N.; Nelson, B. L.; Barnes, E. L.; Thompson, L. D. R. (2008). "Ceruminous Gland Carcinomas: A Clinicopathologic and Immunophenotypic Study of 17 Cases". Head and Neck Pathology. 3 (1): 1–17. doi:10.1007/s12105-008-0095-9. PMC   2807538 . PMID   20596983.
  4. ↑ Hicks, G. W. (1983). "Tumors arising from the glandular structures of the external auditory canal". The Laryngoscope. 93 (3): 326–340. doi:10.1288/00005537-198303000-00016. PMID   6300574. S2CID   31253554.
  5. ↑ Aikawa, H.; Tomonari, K.; Okino, Y.; Hori, F.; Ueyama, T.; Suenaga, S.; Bundo, J.; Tsuji, K. (1997). "Adenoid cystic carcinoma of the external auditory canal: Correlation between histological features and MRI appearances". The British Journal of Radiology. 70 (833): 530–532. doi:10.1259/bjr.70.833.9227237. PMID   9227237.
  6. ↑ Ito, K.; Ito, T.; Tsukuda, M.; Kanisawa, M. (1993). "An immunohistochemical study of adenoid cystic carcinoma of the external auditory canal". European Archives of Oto-Rhino-Laryngology. 250 (4): 240–244. doi:10.1007/bf00171533. PMID   7690238. S2CID   11946085.
  7. ↑ Thompson, L. D.; Nelson, B. L.; Barnes, E. L. (2004). "Ceruminous adenomas: A clinicopathologic study of 41 cases with a review of the literature". The American Journal of Surgical Pathology. 28 (3): 308–318. doi:10.1097/00000478-200403000-00003. PMID   15104293. S2CID   27571673.
  8. ↑ Dehner, L. P.; Chen, K. T. (1980). "Primary tumors of the external and middle ear. Benign and malignant glandular neoplasms". Archives of Otolaryngology. 106 (1): 13–19. doi:10.1001/archotol.1980.00790250015004. PMID   6243462.
  9. ↑ Torske, K. R.; Thompson, L. D. R. (2002). "Adenoma versus Carcinoid Tumor of the Middle Ear: A Study of 48 Cases and Review of the Literature". Modern Pathology. 15 (5): 543–555. doi: 10.1038/modpathol.3880561 . PMID   12011260.
  10. 1 2 3 4 5 Saunders, T. F. C.; Monksfield, P. (2014). "A Case of Conservatively Managed Invasive Ceruminoma and a Review of the Literature". Case Reports in Otolaryngology. 2014: 897540. doi:10.1155/2014/897540. ISSN   2090-6765. PMC   4129918 . PMID   25143852.
  11. 1 2 3 Wanner, Brian; Rismiller, Kyle; Carr, David R. (2021-06-23). "Treatment and survival outcomes of ceruminous carcinomas of the external auditory canal: a SEER database analysis and literature review". Archives of Dermatological Research. doi:10.1007/s00403-021-02257-4. ISSN   1432-069X.
  12. ↑ Ruhl, Douglas S.; Tolisano, Anthony M.; Swiss, Tyler P.; Littlefield, Philip D.; Golden, J. Blake (2016). "Ceruminous adenocarcinoma: An analysis of the Surveillance Epidemiology and End Results (SEER) database". American Journal of Otolaryngology. 37 (2): 70–73. doi:10.1016/j.amjoto.2015.11.005.

Further reading

Classification
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