Small cell carcinoma of the ovary, hypercalcemic type

Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare and highly aggressive malignancy that predominantly affects young women in their second and third decades of life. The reported survival is only 55% for stage I tumors, and long-term survival is uncommon with more advanced disease. SCCOHT is characterized by monogenic loss-of-function mutations in SMARCA4. The mechanisms of tumorigenesis in SCCOHT remain poorly understood, the survival rate is low, and effective treatments are needed.