Ovarian neoplasms composed of small round cells: a review

Ovarian neoplasms composed predominantly or exclusively of small round cells with scant cytoplasm are relatively rare. However, there is a wide differential, and pathologists often struggle to make a correct diagnosis because of overlapping histologic features. Perhaps the best known of these neoplasms is ovarian small cell carcinoma of hypercalcemic type (OSCCHT), a tumor of unknown histogenesis. This may be confused with a wide range of neoplasms ranging from sex cord-stromal tumors (some of which may exhibit a small cell phenotype) to neoplasms in the family of small round blue cell tumors to various undifferentiated malignancies. A neuroendocrine small cell carcinoma, so-called small cell carcinoma of pulmonary type, may also arise within the ovary, and this may be a component of a typical ovarian surface epithelial-stromal tumor. In addition to the well-known family of small round blue cell tumors of childhood, other small cell neoplasms that may arise within the ovary or involve the ovary include intra-abdominal desmoplastic small round cell tumor, metastatic small cell carcinoma, peripheral and central primitive neuroectodermal tumor, and endometrial stromal sarcoma. Malignant melanoma, undifferentiated carcinoma, and various germ cell tumors, especially dysgerminoma and immature teratoma, also on occasion enter into the differential diagnosis of an ovarian small cell neoplasm. In this review, the morphologic features of some of these neoplasms are described, as is the value of immunohistochemistry and other ancillary techniques in establishing a diagnosis.