COMPASSIONATE ALLOWANCE INFORMATION

SMALL-CELL CANCER OF THE FEMALE GENITAL TRACT

DESCRIPTION

Small-Cell Cancer (SCC) of the Female Genital Tract (FGT) occurs most frequently in the cervix, but can also occur in the endometrium, ovary, fallopian tube, vagina, and vulva. It is an extremely aggressive cancer with a biological behavior that is similar to small-cell cancer of the lung. Neuroendocrine tumors of the FGT, of which SCC FGT are a subset are rare cancers that account for less than 2% of gynecological cancers.

ALTERNATE NAMES

Small-Cell Carcinoma of the Uterus; Small-Cell Carcinoma of the Vagina; Small-Cell Carcinoma of the Vulva; Small-Cell Carcinoma of the Endometrium; Small-Cell Carcinoma of the Fallopian Tube; Small-Cell Cancer of the Ovary; Small-Cell Cancer of the Cervix; Vaginal Small-Cell Carcinoma; Vaginal Small-Cell Cancer; Primary Vaginal Small-Cell Carcinoma; Poorly Differentiated Neuroendocrine Cancer of the Vaginal Tract

DIAGNOSTIC TESTING AND CODING

Diagnostic testing: CT Scan of female genital tract, brain, chest, abdomen, and pelvis; and bone marrow testing.

Physical findings: (see onset and progression).

ICD 9: 179.;180.1;180.8; 183.0; 183.2; 183.3; 183.5; 183.8; 183.9;184.4; 184.8; 184.9

TREATMENT

There is no standard treatment for SCC FGT. Treatment is symptom specific and generally consists of a combination of surgery; radiation; chemotherapy; and immunotherapy.

PROGRESSION

Small-Cell cancer of the uterus or cervix is a highly aggressive tumor that often metastasizes early and widely by both lymphatic and haematogenous routes and involves regional and distant lymph nodes, lung, bone and brain and liver. The disease-free interval is usually less than two years with an almost 70% rate of recurrence within 12 months of diagnosis. Long term survival is possible for individuals with cancers that are clinically localized to the cervix but individuals with more advanced stage of the disease is poor with very few long term survivors.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

• Pathology report of biopsy of surgical specimen of the cervix; endometrium; ovary; fallopian tube; vagina; vulva; or cell histology showing small-cell cancer of the female genital tract.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.23 A or B

13.23F

Small-cell cancer of the female genital tract meets listing 13.23F upon confirmed diagnosis alone.

Medical Equals

* 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: 9/10/15

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