Ovarian sex cord–stromal tumors: Reflections on a 40-year experience with a fascinating group of tumors, including comments on the seminal observations of Robert E. Scully, MD

Abstract

Context.—This year being the 60th anniversary of the publication of the excellent book Endocrine Pathology of the Ovary by John McLean Morris, MD, and Robert E. Scully, MD, the writer reflects on that work and in particular the remarkable contributions of its second author to our knowledge in this area. Objective.—To review ovarian sex cord–stromal tumors. Data Sources.—Literature and personal experience. Conclusions.—The essay begins with remarks on the oftentimes straightforward stromal tumors of the ovary because the commonest of them, the fibroma, dominates from the viewpoint of case numbers. Then, the sclerosing stromal tumor and the peculiar so-called luteinized thecomas of the type associated with sclerosing peritonitis are discussed in greater detail and their wide spectrum is illustrated. Brief mention is made of 2 rare neoplasms: the ovarian myxoma and signet-ring stromal tumor. Discussion then turns to the more recently recognized intriguing tumor tentatively designated microcystic stromal tumor and the commonest malignant tumor in this entire family, the so-called adult granulosa cell tumor, which despite its name may occasionally be seen in young individuals. The second variant of granulosa cell tumor—that which usually, but not always, occurs in the young—the so-called juvenile granulosa cell tumor, is then discussed. In the section of Sertoli-Leydig cell tumors, particular attention is focused on unusual tumors with heterologous elements and the remarkable so-called retiform tumors, which have a predilection for the young, often have distinctive gross features, and exhibit slitlike spaces and papillae. The essay concludes with consideration of the sex cord tumor with annular tubules.