The treatment of small cell carcinoma of the ovary hypercalcemic type

Abstract

Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) is an exceedingly rare and poorly characterized tumor with an unknown cell of origin. SCCOHT typically occurs in adolescents and young women with a peak incidence in the third decade of life. The initial description of SCCOHT noted a dismal outcome among patients, particularly those with advanced disease. However, recently published studies indicate that SCCOHT is often amenable to therapy and even in advanced stages potentially curable. Surgery paired with multiagent chemotherapy including platinum-based agents and etoposide are often cited in patients who achieve a complete remission. While reports of successfully treated patients have emerged, the scarcity of prospective clinical trials has slowed the validation of effective treatment regimens. The value of extensive surgery, radiation therapy, and high-dose chemotherapy with autologous stem cell rescue remain uncertain. Insight into the biology underlying SCCOHT is desperately needed to guide the implementation of novel therapeutics in SCCOHT.