Small Cell Carcinoma of the Ovary, Pulmonary Type, With a Germline BRCA2 Mutation: A Report of a Rare Case.

Small cell carcinoma of the ovary, pulmonary type (SCCOPT), is a rare and aggressive malignancy with a poor prognosis. We report a rare case of this cancer in a patient with a germline BRCA2 (gBRCA2) mutation. A 66-year-old female patient presented with abdominal distention, underwent a laparotomy, and was diagnosed with stage IVB SCCOPT. Histopathological examination revealed no other ovarian tumor components. Adjuvant platinum-based chemotherapy was administered, followed by maintenance therapy with poly(ADP-ribose) polymerase inhibitors (PARPi) because of the gBRCA2 mutation. Despite expectations of a favorable outcome with the targeted maintenance therapy, disease progression was noted five months after starting maintenance therapy, and the patient died 12 months after surgery. The disease course suggests that maintenance therapy may have limited efficacy against this cancer, even in the presence of gBRCA2 mutations. Further research is necessary to investigate the role of PARPi and establish more effective oncological treatment for this cancer.