Clinical experience of young patients with small cell ovarian carcinoma of the hypercalcemic type (OSCCHT)

Abstract

Objective: Small cell ovarian cancer of the hypercalcemic type (OSCCHT) is a very rare and highly aggressive disease which mainly affects young women, while optimal treatment guidelines have not yet been defined. The objective of this work is to present our experience with four OSCCHT patients.

Study design: We evaluated the surgical course and clinical outcome of all OSCCHT patients treated in the European Competence Center for Ovarian Cancer, Charité, University Medicine of Berlin. Pathology was reviewed by specialized gynecological pathologists of our center.

Results: Four OSCCHT patients were identified between 2008 and 2011 (median age: 24.5 years; range: 18-29) out of 845 ovarian cancer patients being operated on within this timeframe. Two patients were diagnosed at a very early tumor stage (FIGO Ia), one in FIGO IIb, and one patient presented with advanced stage disease FIGO IIIc. Treatment of choice was surgery followed by adjuvant platinum-based chemotherapy. In all patients the uterus was preserved and also the contralateral ovary in three out of the four patients. Within a median follow-up time of 22 months (range: 8-47) only the FIGO IIIC-patient relapsed twice and died 15 months after initial diagnosis. The other three patients are all alive and with no signs of relapse at 8, 29 and 47 months after initial diagnosis.

Conclusion: OSCCHT is a rare tumor entity which usually affects young women with hopes of childbearing. The clinical course varies widely and although it is associated with an overall dismal prognosis, fertility-sparing surgery followed by platinum-based adjuvant chemotherapy may be considered in early stages of the disease.