In two separate reviews, we review the time-honored but still frequently challenging features of ovarian sex cord-stromal tumors, and also emphasize new developments including unusual morphologic appearances that, despite the relative rarity of many of the tumors, result in a disproportionate number of differential diagnostic problems, variant immunohistochemical profiles, and specific molecular and syndromic associations. These neoplasms are also of historical interest as current knowledge is still based in significant part on the contributions of 2 giants of gynecologic pathology, Dr Robert Meyer and Dr Robert E. Scully. In part I, we present the major clinical, pathologic, and genomic features of the pure ovarian stromal tumors including comments on differential diagnosis and briefly note significant historical contributions. In part II we will discuss pure sex cord and sex cord-stromal tumors.
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http://dx.doi.org/10.1097/PAP.0000000000000435 | DOI Listing |
Cureus
December 2024
Medical Corps, Israel Defense Forces, Haifa, ISR.
Testicular tumors represent the most common solid organ malignancy in young and adult males. Sex cord-stromal tumors are the second-largest group of primary testicular cancers, after germ-cell tumors. Sertoli cell (SC) tumors of the testis are relatively rare, accounting for only a small fraction of testicular tumors.
View Article and Find Full Text PDFPediatr Dev Pathol
January 2025
Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, St. Louis, MO, USA.
A desmoplastic small round cell tumor (DSRCT) presented in a 13-year-old female with an acute abdomen due to torsion of a fallopian tube cyst. She was found to have an incidental 2 cm pedunculated, solid, and multicystic mass attached to the pelvic floor on laparoscopy. The neoplasm had a variably myxoid and spindle cell pattern with nests and cords of small cells, forming pseudocysts, and true cysts lined by ciliated epithelium which were PAX-8+ and ER+/PR+.
View Article and Find Full Text PDFDiagn Pathol
January 2025
Department of Pathology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Studničkova 2, Prague, 12800, Czech Republic.
Background: Juvenile granulosa cell tumor (JGCT) of the ovary is a rare tumor with distinct clinicopathological and hormonal features primarily affecting young women and children. We conducted a complex clinicopathological, immunohistochemical, and molecular analysis of five cases of JGCT.
Methods: The immunohistochemical examination was performed with 32 markers, including markers that have not been previously investigated.
Front Oncol
December 2024
Department of Gynaecologic Oncology, Center for Gynaecologic Oncology Amsterdam, Cancer Center Amsterdam, Amsterdam University Medical Centres, Amsterdam, Netherlands.
Urol Case Rep
January 2025
Department of Urology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Sertoli cell tumors are a rare type of sex-cord stromal tumor. We present a case of a thirteen-year-old male presenting with 2-3 months of fevers and twenty-pound weight loss. Evaluation revealed leukocytosis, anemia, elevated systemic inflammatory markers and a negative infectious disease evaluation.
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