TN 1 (10-08)

DI 23022.290 Salivary Tumors

COMPASSIONATE ALLOWANCE INFORMATION

SALIVARY TUMORS

DESCRIPTION

Malignant Salivary Tumors is a rare cancer that forms in tissues of salivary glands in the floor of the mouth and throughout the oropharynx, the parotid glands and the submandibular glands. Cancer of the salivary glands commonly presents with one of several different histologies: mucoepidermoid, adenoid cystic, acinic cell, malignant mixed, squamous or adenocarcinoma. There are two rare histologies which have much worse prognosis than the standard pathological diagnoses: Anaplastic small cell and adenosquamous carcinoma of the salivary glands.
Anaplastic Small Cell Carcinoma of the Salivary Glands is a neuroendocrine tumor which displays very aggressive metastatic behavior. Microscopically, the tumor cells have oval, hyperchromatic nuclei and scant amount of cytoplasm and are organized in sheets, strands, and nests. At time of diagnosis, distant metastatic disease is almost always present.
Adenosquamous Carcinoma of the Salivary Glands is an extremely rare malignant neoplasm that simultaneously arises from surface mucosal epithelium and salivary gland ductal epithelium. The carcinoma shows histopathologic features of both squamous cell carcinoma and adenocarcinoma. In addition to swelling, adenosquamous carcinoma produces visible changes in the mucosa including erythema, ulceration, and induration. Pain frequently accompanies ulceration. This carcinoma behaves aggressively with extensive infiltrating local disease as well as distant metastatic disease.
Symptoms include: a lump (usually painless) in the area of the ear, cheek, jaw, lip, or inside the mouth; fluid draining from the ear; trouble swallowing or opening the mouth widely; numbness or weakness in the face and pain in the face that does not go away.

ALTERNATE NAMES

Salivary Glands Cancer, Anaplastic Small Cell Carcinoma of the Salivary Glands, Adenosquamous Carcinoma of the Salivary Glands, Anaplastic Small Cell Carcinoma, Adenosquamos Carcinoma

DIAGNOSTIC TESTING AND CODING

Small cell cancer of the salivary gland and adenosquamous cancer of the salivary gland can be determined only by pathologic evaluation of tissue obtained by needle biopsy or surgery.
Additional diagnostic tests may include: MRI, CT scan, PET scan, ultrasound, endoscopy and fine needle aspiration biopsy. After salivary gland cancer has been diagnosed, tests are done to find out if cancer cells have spread within the salivary gland or to other parts of the body.

TREATMENT

Treatment can consist of surgery, radiation, and chemotherapy in various combinations depending on the clinical circumstances. Unfortunately, treatment for small cell carcinoma or adenosquamous carcinoma of the salivary gland is rarely curative.

PROGRESSION

Progression can be with local recurrence or distant metastases.
Anaplastic Small Cell Carcinoma - Neuroendocrine carcinomas are frequently found in the minor salivary glands. Individuals with this type of cancer have a better survival rate compared to those with small cell carcinomas of the lung.
Adenosquamous Carcinoma - Limited data indicate that this is a highly aggressive neoplasm with a poor prognosis.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation: A pathology report on needle biopsy or surgical specimen showing small cell cancer or adenosquamous cancer.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

 

 

Medical Equals

13.14 B

Small cell carcinoma of the lung and small cell carcinoma of the salivary gland behave similarly with early metastatic progression

13.14 B

Adenosquamous carcinoma of a salivary gland equals 13.14 B because its poor prognosis is similar to small cell carcinoma of the lung as it is based on histology alone.

* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

Last Updated: 9/10/08

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DI 23022.290 - Salivary Tumors - 10/24/2008
Batch run: 01/27/2009
Rev:10/24/2008