Early-stage small cell carcinoma of the ovary hypercalcemic type: autologous stem cell transplantation as standard primary treatment

Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) represents less than 0.01% of all ovarian malignancies which affects mostly young women. Most cases are caused by a pathogenic variant (PV) in SMARCA4 gene. Prognosis correlates directly with FIGO stage and multi-modal treatment including optimal cytoreductive surgery plus multi-agent platinum-based chemotherapy is recommended. A case of a 20-year-old female diagnosed with SCCOHT is presented. After surgery, she underwent six cycles of a platinum-based chemotherapy regimen consolidated with an autologous stem cell transplant. She was the first patient diagnosed with SCCOHT to receive a bone marrow transplant in Portugal and, 28 months after the initial diagnosis, has no evidence of relapse. The authors discuss the management of this clinical case in parallel with an updated literature review, and the most recent International SCCOHT Consortium (ISC) guidelines, with emphasis on the role of autologous stem cell transplantation in the early stages of this disease.