Small Cell Carcinoma of Ovary, Hypercalcemic Type: Cytologic, Histopathologic, and Immunohistochemical Landscapes of a Rare Case
Small cell carcinoma of ovary, hypercalcemia type (SCCOHT), also known as the malignant rhabdoid tumor of the ovary, is a rare and highly aggressive malignancy affecting younger women. The pathogenesis involves mutations in SWI/SNF-related, matrix-associated, actin-dependent regulator chromatin group A4 (SMARCA4)/Brahma-related gene 1 (BRG1) and/or SWI/SNF-related, matrix-associated, actin-dependent regulator chromatin group A2 (SMARCA2)/Brahma homolog (BRM). A 10-year-old girl presented with lower abdominal pain and a mass for the past 2 weeks. She underwent ultrasound-guided fine needle aspiration and core needle biopsy from the pelvic mass followed by surgery. On the basis of the characteristic morphologic and immunohistochemical features, a diagnosis of SCCOHT was rendered. Chemotherapy was started, however, she succumbed to the disease.