Small cell carcinoma of the ovary of hypercalcemic type: a case report

Abstract

The authors report a case of small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), in a mother and daughter and discuss the possibility of a heritable risk. Both mother and daughter were treated at the same institution for SCCOHT. A 23-year-old woman presented with hypercalcemia 4 months after giving birth to her daughter. She was diagnosed as having SCCOHT. Despite surgery, chemotherapy, and radiation, she died of the disease 11 months after diagnosis. Eleven years later, her daughter presented with a histologically and immunophenotypically identical SCCOHT tumor. She received postoperative chemotherapy and radiation but, eventually, relapsed and died of the disease at 27 months after the initial diagnosis. Small cell carcinoma of the ovary, hypercalcemic type, is an uncommon and aggressive malignancy that occurs in young women, which is associated with a solid ovarian tumor and hypercalcemia. Despite aggressive multimodality treatment, most patients die within 2 years of diagnosis. Genetic counseling, sonographic ovarian surveillance and serum calcium monitoring at early age, and even prophylactic oophorectomy should be considered for surviving at-risk family members.