Sclerosing stromal tumor: an important differential diagnosis of ovarian neoplasms in childhood and adolescence

Sclerosing stromal tumors are an uncommon type of benign ovarian sex cord-stromal tumor. Histologically, they are characterized by a cellular pseudolobular pattern, composed of fibroblasts and round cells separated by densely collagenous or markedly edematous hypocellular tissue and prominent vascularity. Although the usual age of presentation is in the 2nd and 3rd decades, sclerosing stromal tumor can occur in adolescence or premenarchal girls. Clinical symptoms include premature menarche, menstrual irregularities, abdominal discomfort, and rarely, ascites. Imaging studies frequently reveal solid or complex cystic adnexal masses with marked vascularity raising concern for germ cell tumors and, especially in the absence of elevated tumor markers, surface epithelial neoplasms. The differential diagnosis of a benign sclerosing stromal tumor is seldom entertained. We present the clinicopathological findings in 4 adolescent patients, presenting with unilateral adnexal masses, in which the preoperative clinical suspicion for malignancy was very high. We stress the importance of being familiar with sclerosing stromal tumors when evaluating ovarian neoplasms in children and adolescents in order to contribute to the appropriate clinical management preventing extensive and unnecessary surgery, and preserving fertility.