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.

This is a very rare neoplasm accounting for approximately 0.0003% of all tumors and about 2.5% of all external ear neoplasms. There is a wide age range at initial presentation, although the mean age is about 50 years of age. Females are affected slightly more often (1.5:1).

Treatment may include the following:

- Surgery with or without radiation

- Radiotherapy

Fast neutron therapy has been used successfully to treat salivary gland tumors, and has shown to be significantly more effective than photons in studies treating unresectable salivary gland tumors.

- Chemotherapy

The primary method for treatment is surgical, not medical. Radiation and chemotherapy are not needed for benign lesions and are not effective for malignant lesions.

Benign granular cell tumors have a recurrence rate of 2% to 8% when resection margins are deemed clear of tumor infiltration. When the resection margins of a benign granular cell tumor are positive for tumor infiltration the recurrence rate is increased to 20%. Malignant lesions are aggressive and difficult to eradicate with surgery and have a recurrence rate of 32%.

Large and especially giant congenital nevi are at higher risk for malignancy degeneration into melanoma. Because of the premalignant potential, it is an acceptable clinical practice to remove congenital nevi electively in all patients and relieve the nevocytic overload.

This is an uncommon lesion, usually affecting young patients (mean age, 30 years), with a male to female ratio of 2:1. The middle ear is involved, although it may extend to the external auditory canal if there is tympanic membrane perforation.

Since this lesion is usually a complication of long standing otitis media, it is important to use an appropriate antibiotic therapy regimen. If the patient fails first line antibiotics, then second-line therapies should be employed, especially after appropriate culture and sensitivity testing. Surgery may be required if there is extension into the mastoid bone, or if a concurrent cholesteatoma is identified during surgery or biopsy. In general, patients have an excellent outcome after appropriate therapy.

Surgical excision is the standard of care. Some individuals advocate the use of hair removal laser for the treatment of congenital nevi. While this is likely safe and effective for small congenital nevus, laser removal for larger lesions might pose a liability for the laser surgeon if malignancy developed from a deep (dermal) component of the nevus that is not reached by the laser. Repigmentation after laser treatment of congenital nevi or superficial curettage supports this concern.

Many are surgically removed for aesthetics and relief of psychosocial burden, but larger ones are also excised for prevention of cancer, although the benefit is impossible to assess for any individual patient. Proliferative nodules are usually biopsied and are regularly but not systematically found to be benign. Estimates of transformation into melanoma vary from 2-42% in the literature, but are most commonly considered to be at the low end of that spectrum due to early observer bias.

As metanephric adenomas are considered benign, they can be left in place, i.e. no treatment is needed.

An acanthoma is a skin neoplasm composed of squamous or epidermal cells. It is located in the prickle cell layer.

Types of acanthoma include pilar sheath acanthoma, a benign follicular tumor usually of the upper lip; clear cell acanthoma, a benign tumor found most frequently on the legs; and Degos acanthoma, often confused with but unrelated to Degos disease.

Choroid plexus papillomas are benign tumors that are usually cured by surgery; malignant progression has been rarely reported.

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.

Benign fibromas may, but need not be, removed. Removal is usually a brief outpatient procedure.

Ocular oncology is the branch of medicine dealing with tumors relating to the eye and its adnexa.

Ocular oncology takes into consideration that the primary requirement for patients is preservation of life by removal of the tumor, along with best efforts directed at preservation of useful vision, followed by cosmetic appearance. The treatment of ocular tumors is generally a multi-specialty effort, requiring coordination between the ophthalmologist, medical oncologist, radiation specialist, head & neck surgeon/ENT surgeon, pediatrician/internal medicine/hospitalist and a multidisciplinary team of support staff and nurses.

Usually the lesion is surgically removed. Primarily, there is concern that the lesion identified in a patient could be cancerous, but there is also the risk of torsion, and possibly the development of symptoms. A stable lesion, however, could be clinically followed.

Pilomatricomas have been observed in a variety of genetic disorders including Turner syndrome, myotonic dystrophy, Rubinstein-Taybi syndrome. Trisomy 9, and Gardner syndrome. It has been reported that the prevalence of pilomatricomas in Turner syndrome is 2.6%.

Hybrid cysts that are composed of epidermal inclusion cysts and pilomatricoma-like changes have been repeatedly observed in Gardner syndrome. This association has prognostic important since cutaneous findings in children with Gardner Syndrome generally precede colonic polyposis.

Internationally tumors of the choroid plexus are rare, accounting for 0.4-0.6% of all intracranial neoplasms. It most commonly affects young children under the age of 5 with a mean patient age of 5.2 years.

Sebaceous lymphadenoma is a tissue diagnosis, e.g. salivary gland biopsy.

It may be confused with a number of benign and malignant neoplasms, including Warthin tumour, mucoepidermoid carcinoma and sebaceous lymphadenocarcinoma.

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".

These lesions rarely require surgery unless they are symptomatic or the diagnosis is in question. Since these lesions do not have malignant potential, long-term observation is unnecessary. Surgery can include the removal of the head of the pancreas (a pancreaticoduodenectomy), removal of the body and tail of the pancreas (a distal pancreatectomy), or rarely removal of the entire pancreas (a total pancreatectomy). In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy.

Variants with edema can be associated with Meigs' syndrome. They may be a part of nevoid basal cell carcinoma syndrome (Gorlin syndrome).

Trichoblastomas are a cutaneous condition characterized by benign neoplasms of follicular germinative cells. Trichoblastic fibroma is a designation used to characterize small nodular trichoblastomas with conspicuous fibrocytic stroma, sometimes constituting over 50% of the lesion.

Granular cell tumor is a tumor that can develop on any skin or mucosal surface, but occurs on the tongue 40% of the time.

It is also known as Abrikossoff's tumor, Granular cell myoblastoma, Granular cell nerve sheath tumor, and Granular cell schwannoma.)

JCT often is described as benign, however one case of metastasis has been reported, so its malignant potential is uncertain. In most cases the tumor is encapsulated.

Pilomatricoma, also known as a calcifying epithelioma of Malherbe, Malherbe calcifying epithelioma, and Pilomatrixoma, is a benign skin tumor derived from the hair matrix. These neoplasms are relatively uncommon and typically occur on the scalp, face, and upper extremities. Clinically, pilomatricomas present as a subcutaneous nodule or cyst with unremarkable overlying epidermis that can range in size from 0.5-3.0 cm, but the largest reported case was 24 cm.