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Ovary-II Germ Cell Tumors and Sex Cord Tumors

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Practical Gynecologic Pathology

Part of the book series: Practical Anatomic Pathology ((PAP))

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Abstract

Ovarian primitive germ cell tumors are uncommon. The diagnosis of dysgerminoma, EC and YST and their distinction from non-germ cell tumors often requires immunohistochemical markers such as SALL4, OCT4, CD117, D2-40, CD30, AFP, and glypican-3. Immature teratoma is graded on the amount of immature neuroepithelium. The biologic behavior of struma ovarii cannot be reliably predicted by specific histologic features. Diagnosis of gonadoblastoma should prompt chromosomal analysis for the patient. Sex cord stromal tumors include pure stromal tumors, sex cord tumors and mixed sex-cord stromal tumors. Fibroma can be cellular and mitotically active but lack diffuse cytologic atypia seen in fibrosarcoma. Adult granulosa cell tumor and cellular fibroma/fibrothecoma can be distinguished with reticulin stain and FOXL2 mutation analysis. Juvenile granulosa cell tumor may mimic small cell carcinoma hypercalcemic type but without SMARCA4 (BRG1) loss. Intermediate and poorly differentiated Sertoli-Leydig cell tumors most often possess DICER1 mutations and genetic testing is advised. Sex cord tumor with annular tubules (SCTAT) is the most common sex-cord stromal tumor associated with Peutz-Jeghers syndrome.

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Abbreviations

AFP:

Alpha-fetoprotein

AGCTs:

Adult granulosa cell tumors

FOXL2:

Forkhead box L2

GFAP:

Glial fibrillary acidic protein

HDFCO:

Highly differentiated follicular carcinoma of ovarian origin

HPFs:

High power fields

JGCT:

Juvenile granulosa cell tumor

OCT4:

Octamer-binding transcription factor 4

PJS:

Peutz-Jeghers syndrome ()

SALL4:

Sal-like protein 4

SCCHT:

Small cell carcinoma, hypercalcemic type

SCTAT:

Sex cord tumor with annular tubules

SF-1:

Steroidogenic factor

SLCTs:

Sertoli-Leydig cell tumors

STK11:

Serine/Threonine Kinase 11

YST:

Yolk Sac Tumor

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Authors and Affiliations

  1. Pathology and Immunology, Washington University School of Medicine, St. Louis, MO, USA

    Dengfeng Cao

  2. Department of Pathology, Oregon Health & Science University (OHSU), Portland, OR, USA

    Olivia L. Snir

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  1. Dengfeng Cao
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  2. Olivia L. Snir
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Editors and Affiliations

  1. Department of Pathology, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA

    Jian-Jun Wei

  2. Department of Pathology, Yale School of Medicine, New Haven, CT, USA

    Pei Hui

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Cao, D., Snir, O.L. (2021). Ovary-II Germ Cell Tumors and Sex Cord Tumors. In: Wei, JJ., Hui, P. (eds) Practical Gynecologic Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-68608-6_10

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