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Sex Cord-Stromal Tumors of the Ovary

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Gynecologic and Obstetric Pathology, Volume 2

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Abstract

Sex cord-stromal tumors (SCSTs ) are rare neoplasms derived from, or display differentiation towards primitive sex cords or stromal cells. The tumors in this category exhibit a great diversity of patterns with a long array of mimickers, and accordingly may pose a diagnostic challenge. Some of the derivative cells are involved in hormone production under physiologic conditions; therefore, many tumors under this category produce excess hormones, which may lead to the development of hormone-mediated syndromes. There are clear age-related differences in the incidence of several tumors in this category, with juvenile granulosa cell tumors, for example, occurring primarily in the <20-year age group, Sertoli-Leydig cell tumors predominating between menarche and 25 years, and adult granulosa cell tumors most commonly occurring between ages 25–50 years. The correct diagnosis of an ovarian tumor is important in all patients, but is arguably particularly so for this category, since young patients represent a significant proportion of patients, and issues of fertility preservation abound. This makes awareness of the diagnostic criteria and the differential diagnosis of the SCSTs an important task. The 2014 World Health Organization classification of SCSTs categorized this group under three main categories: pure stromal tumors, pure sex cord tumors, and mixed SCSTs. This chapter highlights all SCSTs, with an emphasis on the clinicopathologic characteristics and advances in molecular features of each entity, as well as a detailed discussion of differential diagnostic considerations, and the utility and limitations of immunohistochemical markers and ancillary studies in their diagnostic work-up.

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Abbreviations

AGCT :

Adult granulosa cell tumor

EMA :

Epithelial membrane antigen

FATWO:

Female adnexal tumor of probable Wolffian origin

GIST:

Gastrointestinal stromal tumor

GST:

Glutathione S-transferase

JGCT:

Juvenile granulosa cell tumor

LTSP:

Luteinized thecoma associated with sclerosing peritonitis

MST :

Microcystic stromal tumor

NOS:

Not otherwise specified

PJS:

Peutz-Jeghers syndrome

SCCHT:

Small cell carcinoma of hypercalcemic type

SCST:

Sex cord-stromal tumor

SCTAT :

Sex cord tumor with annular tubules

SF-1:

Steroidogenic factor 1

SLCT:

Sertoli–Leydig cell tumor

SRST:

Signet ring stromal tumor

SST :

Sclerosing stromal tumor

WHO :

World Health Organization

YST:

Yolk sac tumor

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Authors and Affiliations

  1. Department of Pathology, Microbiology and Immunology, Vanderbilt University School of Medicine, Nashville, TN, USA

    Mohamed Mokhtar Desouki

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  1. Mohamed Mokhtar Desouki
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Correspondence to Mohamed Mokhtar Desouki .

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Editors and Affiliations

  1. Department of Pathology, Department of Obstetrics and Gynecology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Wenxin Zheng

  2. Department of Pathology, University of California San Diego, San Diego, CA, USA

    Oluwole Fadare

  3. Department of Pathology, College of Medicine, University of Arkansas for Medical Sciences, Little Rock, AR, USA

    Charles Matthew Quick

  4. Department of Pathology, Peking University People’s Hospital, Beijing, China

    Danhua Shen

  5. Department of Obstetrics and Gynecology, Tianjin Gynecologic and Obstetrics Central Hospital, Tianjin, China

    Donghui Guo

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Desouki, M.M. (2019). Sex Cord-Stromal Tumors of the Ovary. In: Zheng, W., Fadare, O., Quick, C., Shen, D., Guo, D. (eds) Gynecologic and Obstetric Pathology, Volume 2. Springer, Singapore. https://doi.org/10.1007/978-981-13-3019-3_9

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  • DOI: https://doi.org/10.1007/978-981-13-3019-3_9

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