Vulvar cancer with Fanconi's anemia and neutropenic fever: a case report.

BACKGROUND

Fanconi's anemia (FA), an autosomal-recessive aplastic anemia, was first described in 1927. Patients usually die from complications of pancytopenia. The longer patients survive the underlying anemia, the higher the risk of other cancers, particularly leukemias, hepatocellular cancer and squamous cell tumors. This report is the sixth reported case of vulvar cancer in a young woman with FA since 1966.

METHODS

A 25-year-old woman with FA was admitted with neutropenic fever; a rapidly growing, suppurative vulvar mass; and Trichomonas vaginalis. The patient had maintained routine, preventive gynecologic care, and 4 months prior to admission she had complete removal of a benign vulvar condyloma and no evidence of genital tract cancer. We removed the vulvar mass to relieve discomfort and eliminate an infectious source. The mass was an invasive squamous cell carcinoma of the vulva arising in a vulvar condyloma. Within 2 months of the diagnosis, the patient developed bulky disease metastatic to the lungs and inguinal lymph nodes.

CONCLUSIONS

FA patients are at high risk of squamous cell tumors, and gynecologic examinations should begin at menarche. However, despite adequate screening, rapidly progressing solid tumors of the genital tract can develop in these immunosuppressed patients, who have a defect in DNA repair genes.