Background: The clinical and prognostic evaluation of cervical and vaginal tumors other than squamous cell and adenocarcinomas is hampered by the low incidence, and clinical and epidemiological studies on these uncommon tumors are scarce. Having close affinity with the pathology laboratories, the Netherlands Cancer Registry offers a great opportunity to study frequency, stage, treatment, and survival of uncommon tumors in the cervix and vagina and separately, the clear cell adenocarcinoma of the vagina and cervix.

Methods: All invasive cervical tumors (n = 10,570) and all in situ and invasive vaginal tumors (n = 778) diagnosed in the Netherlands during 1989-2003 were selected from the Netherlands Cancer Registry. Age, stage at diagnosis, and treatment were described for each histological subgroup to find differences between common and uncommon tumors, including 5-year relative survival rates.

Results: Twenty-five patients (3%) with cervical cancer subsequently developed a vaginal tumor (during 1989-2003), and 19 of these patients underwent hysterectomy for their cervical cancer. A significantly worse prognosis was found for patients with small cell neuroendocrine cervical tumors and for patients with vaginal melanomas. Patients with clear cell adenocarcinoma of the vagina and cervix were found across all age categories.

Conclusions: The less common histological types of cervical and vaginal cancers were clearly different from squamous cell carcinomas, especially with respect to age at diagnosis and survival rates. Spreading population-based knowledge of effects of treatment of these uncommon tumors should help clinical decision making and therefore improve prognosis.