Ceruminous adenocarcinoma is a malignant neoplasm derived from

ceruminous glands Ceruminous glands are specialized sweat glands located subcutaneously in the external auditory canal, in the outer third. Ceruminous glands are simple, coiled, tubular glands made up of an inner secretory layer of cells and an outer myoepithelia ...

of the

external auditory canal The ear canal (external acoustic meatus, external auditory meatus, EAM) is a pathway running from the outer ear to the middle ear. The adult human ear canal extends from the auricle to the eardrum and is about in length and in diameter. Stru ...

. 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 Mucoepidermoid carcinoma (MEC) is the most common type of minor salivary gland malignancy in adults. Mucoepidermoid carcinoma can also be found in other organs, such as bronchi, lacrimal sac, and thyroid gland. Mucicarmine staining is one stain us ...

Classification

This tumor only affects the outer 1/3 to 1/2 of the

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 Pain is a distressing feeling often caused by intense or damaging Stimulus (physiology), stimuli. The International Association for the Study of Pain defines pain as "an unpleasant sense, sensory and emotional experience associated with, or res ...

is the most common symptom, followed by either sensorineural or conductive

hearing loss Hearing loss is a partial or total inability to hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to acquire spo ...

tinnitus Tinnitus is a condition when a person hears a ringing sound or a different variety of sound when no corresponding external sound is present and other people cannot hear it. Nearly everyone experiences faint "normal tinnitus" in a completely ...

or drainage (discharge). A mass lesion may be present, but it is often slow growing.

Diagnosis

Imaging

Imaging studies are used to define the extent of the tumor and to exclude direct extension from the

parotid gland 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 m ...

or nasopharynx. 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. The tumors are separated into three main histologic or microscopic types: * Ceruminous adenocarcinoma, NOS * Ceruminous adenoid cystic carcinoma * Ceruminous mucoepidermoid carcinoma 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 In pathology, perineural invasion, abbreviated PNI, refers to the invasion of cancer to the space surrounding a nerve. It is common in head and neck cancer, prostate cancer and colorectal cancer. Unlike perineural spread (PNS), which is defined a ...

. 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.

Immunohistochemistry

Immunohistochemistry will help to show the biphasic appearance of the tumor, highlighting the basal or the luminal cells: * Luminal cells: positive with CK7 and

CD117 Proto-oncogene c-KIT is the gene encoding the receptor tyrosine kinase protein known as tyrosine-protein kinase KIT, CD117 ( cluster of differentiation 117) or mast/stem cell growth factor receptor (SCFR). Multiple transcript variants encoding d ...

* Basal cells: positive with p63,

S100 protein The S100 proteins are a family of low molecular-weight proteins found in vertebrates characterized by two calcium-binding sites that have helix-loop-helix ("EF-hand-type") conformation. At least 21 different S100 proteins are known. They are enc ...

and CK5/6

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 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 alon ...

(a benign ceruminous gland tumor) or a neuroendocrine adenoma of the middle ear (middle ear adenoma).

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 In physics, radiation is the emission or transmission of energy in the form of waves or particles through space or a material medium. This includes: * ''electromagnetic radiation'' consisting of photons, such as radio waves, microwaves, infr ...

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.

Epidemiology

This is a very rare neoplasm accounting for approximately 0.0003% of all tumors and about 2.5% of all

external ear The outer ear, external ear, or auris externa is the external part of the ear, which consists of the auricle (anatomy), auricle (also pinna) and the ear canal. It gathers sound energy and focuses it on the eardrum (tympanic membrane). Structur ...

neoplasms. 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. There is no statistical difference between being male and female and developing the tumor and there is no racial inclination. The typical treatment of CA is through surgery and radiation. The type of cut is important with the recurrence rate. Long-term outcomes are better when having the wide cut near the tumor. If the tumor reoccurs there is an 83% chance of mortality and if it does not reoccur there is a 9% chance of mortality. Older people have a lower survival rate than younger ones. The local reoccurrence rate is 49% and the distant metastasis rate is 13%. With local reoccurrence being at 49%, follow-up is very important.

References

External links

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