Successful treatment of preadolescents with small cell carcinoma of the ovary hypercalcemic type.

BACKGROUND: Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) is a rare tumor with a peak incidence in young adulthood that historically has carried a poor prognosis. OBSERVATIONS: We present 2 advanced stage cases of SCCOHT in preadolescents successfully treated with a combination of cisplatin-based chemotherapy and surgical resection. The more recent patient also underwent consolidative high-dose chemotherapy with stem cell rescue and external beam radiotherapy. Her therapy was concluded with a maintenance course of bevacizumab. The patients are now disease-free 7 years and 30 months, respectively, after diagnosis.
CONCLUSIONS: With aggressive multimodal therapy SCCOHT is curable in children.