This uncommon tumor accounts for less than 2% of all ear tumors. While patients present with symptoms related to the middle ear cavity location of the tumor, the tumor may expand into the adjacent structures (external auditory canal, mastoid bone, and eustachian tube). Patients come to clinical attention with unilateral (one sided) hearing loss, usually associated with decreased auditory acuity, and particularly conductive hearing loss if the ossicular bone chain (middle ear bones) is involved. Tinnitus (ringing), otitis media, pressure or occasionally ear discharge are seen. At the time of otoscopic exam, the tympanic membrane is usually intact, with a fluid level or mass noted behind the ear drum. Even though this is a "neuroendocrine" type tumor, there is almost never evidence of neuroendocrine function clinically or by laboratory examination.

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.

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.

Neuroendocrine adenoma of the middle ear has gone by several different names, including middle ear adenoma, carcinoid tumor, amphicrine adenoma, adenocarcinoid, and adenomatoid tumor of middle ear. The various names have created some confusion about this uncommon middle ear tumor. Regardless of the name applied, the "middle ear" anatomic site must be known or confirmed.

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.

The signs and symptoms are similar to other malignant salivary gland tumours; however, it may have been preceded by an appreciable mass that was long-standing and did not appear to be growing.

Findings that suggest a malignant salivary gland tumour include rapid growth, facial weakness (due to facial nerve compression), pain, skin ulceration, fixation of the mastoid tip

and parasthesias.

Warthin's tumor primarily affects older individuals (age 60–70 years). There is a slight male predilection according to recent studies. The tumor is slow growing, painless, and usually appears in the tail of the parotid gland near the angle of the mandible. In 5–14% of cases, Warthin's tumor is bilateral, but the two masses usually are at different times. Warthin's tumor is highly unlikely to become malignant.

Most patients present clinically with progressive, one sided hearing loss, much more often of the sensorineural rather than conductive type. Patients may also experience tinnitus, vertigo, and loss of vestibular function (ataxia). Symptoms are usually present for a long time, which supports the slow growth of these tumors. Patients may also present with other symptoms related to von Hippel-Lindau syndrome in other anatomic sites, which will result in imaging evaluation of the head.

The tumor is usually solitary and presents as a slow growing, painless, firm single nodular mass. Isolated nodules are generally outgrowths of the main nodule rather than a multinodular presentation. It is usually mobile unless found in the palate and can cause atrophy of the mandibular ramus when located in the parotid gland. When found in the parotid tail, it may present as an eversion of the ear lobe. Though it is classified as a benign tumor, pleomorphic adenomas have the capacity to grow to large proportions and may undergo malignant transformation, to form carcinoma ex-pleomorphic adenoma, a risk that increases with time (9.5% chance to convert into malignancy in 15 years). Although it is "benign" the tumor is aneuploid, it can recur after resection, it invades normal adjacent tissue and distant metastases have been reported after long (+10 years) time intervals.

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 (variable appearance) seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which describes its pleomorphic appearance as opposed to its dual origin from epithelial and myoepithelial elements.

The gland most likely affected is the parotid gland. In fact, it is the only tumor virtually restricted to the parotid gland. Though much less likely to occur than pleomorphic adenoma, Warthin's tumor is the second most common benign parotid tumor.

Carcinoma ex pleomorphic adenoma, abbreviated ca ex PA, is a type of cancer typically found in the parotid gland. It arises from the benign tumour pleomorphic adenoma.

Its prognosis depends on the stage. Early tumour have essentially a benign behaviour.

A ceruminous adenoma (also known as adenoma of the ceruminous gland and ceruminoma) 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.

Salivary gland tumours usually present as a lump or swelling in the affected gland which may or may not have been present for a long time. The lump may be accompanied by symptoms of duct blockage (e.g. xerostomia). Usually, in their early stages it is not possible to distinguish a benign tumour from a malignant one. One of the key differentiating symptoms of a malignant growth is nerve involvement. For example signs of facial nerve damage (e.g facial palsy) are associated with malignant parotid tumours. Facial pain, and paraesthesia are also very often associated with a malignant tumours. Other red flag symptoms which may suggest malignancy and warrant further investigation are fixation of the lump to the overlying skin, ulceration and induration of the mucosa.

Due to the diverse nature of salivary gland tumours, many different terms and classification systems have been used. Perhaps the most widely used currently is that system proposed by the World Health Organization in 2004, which classifies salivary neoplasms as primary or secondary, benign or malignant, and also by tissue of origin. This system defines five broad categories of salivary gland neoplasms:

Benign epithelial tumors

- Pleomorphic adenoma

- Warthin's tumor

- Myoepithelioma

- Basal cell adenoma

- Oncocytoma

- Canalicular adenoma

- Lymphadenoma

- "Sebaceous lymphadenoma"

- "Nonsebaceous lymphadenoma"

- Ductal papilloma

- "Inverted ductal papilloma"

- "Intraductal papilloma"

- "Sialadenoma papilliferum"

- Cystadenoma

- Malignant epithelial tumors

- Acinic cell carcinoma

- Mucoepidermoid carcinoma

- Adenoid cystic carcinoma

- Polymorphous low-grade adenocarcinoma

- Epithelial-myoepithelial carcinoma

- Clear cell carcinoma, not otherwise specified

- Basal cell adenocarcinoma

- Sebaceous carcinoma

- Sebaceous lymphadenocarcinoma

- Cystadenocarcinoma

- Low-grade cribriform cystadenocarcinoma

- Mucinous adenocarcinoma

- Oncocytic carcinoma

- Salivary duct carcinoma

- Salivary duct carcinoma, not otherwise specified

- Adenocarcinoma, not otherwise specified

- Myoepithelial carcinoma

- Carcinoma ex pleomorphic adenoma

- Mammary analogue secretory carcinoma

- Carcinosarcoma

- Metastasizing pleomorphic adenoma

- Squamous cell carcinoma

- Large cell carcinoma

- Lymphoepithelial carcinoma

- Sialoblastoma

- Soft tissue tumors

- Hemangioma

- Hematolymphoid tumors

- Hodgkin lymphoma

- Diffuse large B-cell lymphoma

- Extranodal marginal zone B cell lymphoma

- Secondary tumors (i.e. a tumor which has metastasized to the salivary gland from a distant location)

Others, not included in the WHO classification above, include:

- Intraosseous (central) salivary gland tumors

- Hybrid tumors (i.e. a tumor displaying combined forms of histologic tumor types)

- Hybrid carcinoma

- Others

- Others

- Keratocystoma

- Sialolipoma

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

The name of the tumor derives from the fact that:

- It has a varied microscopic architectural appearance, i.e. it is "polymorphous".

- It is non-aggressive when compared to other oral cavity tumors, i.e. it is a "low-grade" tumor.

- It forms glands, i.e. it is an "adenocarcinoma".

It affects the minor salivary glands in the area between the hard and the soft palate. Male to female ratio is 3:1, and the average age is 56 years.

Imaging studies help to identify the tumor and the specific anatomic site of involvement. Magnetic resonance images show a hyperintensity (hypervascularity) of a heterogeneous mass by T1 weighted images. Computed tomography shows a multilocular, lytic destructive temporal bone mass, centered within the endolymphatic sac (between internal auditory canal and sigmoid sinus).

Myoepitheliomas are diagnosed from an examination of the tissue by a pathologist.

An oncocytoma is an epithelial tumor composed of oncocytes, large eosinophilic cells having small, round, benign-appearing nuclei with large nucleoli.

Oncocytoma can arise in a number of organs.

Epithelial-myoepithelial carcinoma, abbreviated 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.

PLGAs consist of a monomorphous cell population that has a varied histologic morphology.

Microscopically, its histology can be confused with an adenoid cystic carcinoma and a pleomorphic adenoma.

Myoepithelioma of the head and neck, also myoepithelioma, is a salivary gland tumour of the head and neck that is usually benign.

As the name suggests, it consists of myoepithelial cells. Classically, they are found in the parotid gland or palate. A similar tumor type may be found in the tongue, referred to as ectomesenchymal chondromyxoid tumor.

Salivary gland oncocytomas are most common in ages 70–80, females, the parotid gland (85–90%), and are firm, slowly growing, painless masses of less than 4 cm. They may be bilateral.

The symptoms may be similar to those classically associated with renal cell carcinoma, and may include polycythemia, abdominal pain, hematuria and a palpable mass. Mean age at onset is around 40 years with a range of 5 to 83 years and the mean size of the tumour is 5.5 cm with a range 0.3 to 15 cm (1). Polycythemia is more frequent in MA than in any other type of renal tumour. Of further relevance is that this tumour is more commonly calcified than any other kidney neoplasm. Surgery is curative and no other treatment is recommended. There is so far no evidence of metastases or local recurrence.

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

It should not be confused with the pathologically unrelated, yet similar sounding, "mesonephric adenoma".