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.

Practical roles for molecular diagnostic testing in ovarian adult granulosa cell tumour, Sertoli–Leydig cell tumour, microcystic stromal tumour and their mimics

“…The classic morphological and immunohistochemical features of AGCT have been extensively reviewed recently and will not be repeated here . Given the broad spectrum of growth patterns and cytological effects of luteinisation in AGCTs (Figure A–C), these tumours may mimic a diverse array of epithelial, germ cell and other sex cord–stromal tumours; this has also been recently reviewed .…”

“…The classic morphological and immunohistochemical features of AGCT have been extensively reviewed recently and will not be repeated here. [31][32][33] Given the broad spectrum of growth patterns and cytological effects of luteinisation in AGCTs ( Figure 1A-C), these tumours may mimic a diverse array of epithelial, germ cell and other sex cord-stromal tumours; this has also been recently reviewed. 33,34 Therefore, we recommend immunohistochemical confirmation of all cases of AGCT by the use of sex cord-stromal markers (FOXL2, SF-1, calretinin, and or inhibin) and, as appropriate, additional markers relevant to the differential diagnosis.…”

Non-Peutz–Jeghers syndrome-associated ovarian sex cord tumor with annular tubules treated by radiotherapy: a case report and literature review

“…Most lesions are clinically benign, with some patients (20%) showing low-grade malignancy. 3–6 Our patient was confirmed to have non-PJS SCTAT presenting with multiple lesions. The Ki-67 concentration was relatively low, but the degree of malignancy was high with multiple recurrences and metastases.…”

Ovarian microcystic stromal tumor with significant bizarre nuclei

“…The histopathological morphology of this tumor varies with the relative prominence of 3 components: microcysts (dominant in 60% of cases), solid cellular regions, and hyalinized fibrous stroma. [ 2 ] Based on personal experience of Young, [ 3 ] the tumor cells usually have lightly eosinophilic cytoplasm and bland nuclei, but bizarre nuclei are present in 60% of the cases. Microcystic stromal tumors have a distinctive immunoprofile.…”

“…They are typically negative for inhibin, calretinin, but diffuse positive for beta-catenin, cyclin D1, WT-1, FOXL2, and SF-1. [ 4 ] Some pathologists think that this tumor may not be truly related to ovarian stromal origin but is, for now, placed in the stromal family as the “best fit.” [ 3 ] However, there is no specific description about the degree and area of bizarre nuclei in MCST in the English literatures. Here, we report a case of ovarian MCST with significant bizarre nuclei accounting for about 50% of the tumor cells.…”