2023 Volume 41 Issue 3 Pages 264-269
Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare tumor that occurs mainly in young women and has a very poor prognosis. We report a case of SCCOHT that developed in the contralateral ovary 16 years after initial treatment during childhood; therefore, it was considered metachronous. When the patient was 14 years old, she underwent left adnexectomy and adjuvant chemotherapy (vincristine + cyclophosphamide + etoposide + cisplatin) for ovarian primitive neuroectodermal tumor (PNET). She had been without recurrence until the age of 30, when she presented with abdominal pain and constipation. An 11-cm right ovarian tumor was found, suggesting the recurrence of PNET. Debulking surgery was performed, but less than 1 cm of dissemination still remained in the abdominal cavity. The pathological diagnosis was SCCOHT, elucidated by the SMARCA4/BRG1 immunostaining. The histopathological findings were similar to those from her childhood, suggesting multiple metachronous tumors. Although two courses of adjuvant chemotherapy with cisplatin and etoposide were administered, the tumor progressed, and the patient died 3.5 months after surgery. This case suggests that SCCOHT should be included in the differential diagnosis of childhood-onset malignant ovarian tumors that require long-term continuous observation due to the risk of metachronous recurrence.
