Pathology Outlines - Sex cord stromal tumors

Ovary tumor
Sex cord stromal tumors
General

Authors: Shahrzad Ehdaivand, M.D. (see Authors page)

Revised: 3 June 2016, last major update December 2012

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed search: Ovarian sex cord stromal tumor

Table of Contents

Definition / General | Positive Stains | Negative Stains

Definition / General

5% of ovarian neoplasms, 7% of malignant ovarian neoplasms
Derived from ovarian stroma, itself derived from sex cords of embryonic gonad (predecessor of Sertoli, Leydig, granulosa and theca cells)
Theca cells produce estrogens; Leydig cells produce androgens
Morphology and clinically evident hormonal activity may go together, but classify based on morphology

Positive Stains

Alpha-inhibin, mullerian inhibiting substance, alpha-inhibin (> 95%), calretinin (more sensitive, less specific than inhibin, Am J Surg Pathol 2002;26:1477), CD99 (Arch Pathol Lab Med 2000;124:563), MART1 / MelanA and WT-1

Negative Stains

EMA, PLAP and CEA
Note: anti-Mullerian hormone staining is present in only a small percentage of granulosa or Sertoli tumor cells, but is specific for these tumors (Hum Pathol 2000;31:1202), particularly compared to endometrioid tumors (Hum Pathol 1998;29:840)
Strong alpha-inhibin staining is relatively specific for ovarian sex-cord stromal proliferations, although luteinized cells from other tumors are also immunoreactiveb (Hum Pathol 1997;28:1387, Am J Surg Pathol 1997;21:583)