Papillary Cystadenoma Lymphomatosum

By: Pagulayan , Sheena May

Papillary Cystadenoma Lymphomatosum

Also known as Warthin's tumor , monomorphic adenoma or adenolymphoma. The disease was named Warthin's tumor after the pathologist who published the first two case reports in the American literature in 1929. He gave the tumor the name papillary cystadenoma lymphomatosum and regarded it as heterotopia of the mucous membrane of the accessory eustachian tube anlage.

It is a type of benign tumor of the parotid gland. Warthin's tumors most commonly present as an asymptomatic, slowly growing round or oval mass.

 The average size of Warthin's tumor at diagnosis is about 2.5 centimeter.

Warthin's tumor is highly unlikely to become malignant.

A sixty years old man with Warthin's tumor of the left parotid gland

Etiology

Its etiology is unknown, but there is a strong association with cigarette smoking. Smokers are at 8 times greater risk of developing Warthin's tumor than the non smoker. The great majority of patients with Warthin's tumor had a history of over 20 years of smoking

Age and Gender predilection

Warthin's tumor primarily affects older individuals (age 6070 years). Male are usually affected.

The tumor is generally superficial, lying just beneath the parotid capsule or protruding to it

CT scan of the neck (axial view) showing a rounded tumor in the right parotid gland.

Histologic features

The tumor is made up of two histologic components: epithelial and lymphoid tissue. there are cystic spaces surrounded by two uniform rows of cells with centrally placed pyknotic nuclei. The cystic spaces have epithelium referred to as papillary infolding that protrude into them. Additionally, the epithelium has lymphoid stroma with germinal center formation. There is frequently an eosinophilic coagulum present within the cystic spaces,which appears as a chocolatecolored fluid in gross specimen.

High magnification micrograph of a Warthin tumor showing the characteristic bilayered epithelium.

Warthin's tumor. Two histologic components are required for the diagnosis: the eosinophilic oncocytes and a lymphoid element.

Pathology

These tumors are well encapsulated lesions with cystic and solid areas. These tumors consist of an oncocytic epithelial cell component arranged in double layers, which develops cysts and papillary projections, and a variable amount of lymphoid tissue often with germinal centers. The immunoprofile of the lymphocyte subsets is similar to that in normal or reactive lymph nodes. A few Warthin's tumors (about 8%) show areas of squamous cell metaplasia and regressive changes.

Differential diagnosis

sebaceous lymphadenoma oncocytoma

Sebaceous lymphadenoma
It is a benign tumor accounting for only 0.196% of all adenomas of the parotid gland. The tumor has a benign course, unless there is a synchronous association with an aggressive malignant neoplasm.

Sebaceous lymphadenoma

Oncocytoma
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.

Micrograph of a parotid gland oncocytoma (right of image). Normal parotid gland is also present (left of image). H&E stain.

Oncocytoma of the Salivary Gland. This lesion presented as a lateral anterior neck mass. At surgery, it was found to be a soft 3.0 x 2.1 x 1.8 cm tumor of the submandibular salivary gland. The photo shows the characteristic dark color of an oncocytoma, a rare type of benign neoplasm, at the left side of the image (the normal lobulated salivary gland tissue is to the right).

Treatment

Most of these tumors are treated with surgical removal. It is mostly non recurrent . Specialists who treat Warthins tumors are Otolaryngologists.

Using a nerve monitor, the branches of the facial nerve are carefully identified and dissected before removing of the tumor.

Prognosis

Prognosis for patients with Warthins tumors is very good. If the patient does decide to remove the tumor recurrence is extremely rare, occurring in only 6% 12% of cases.