Uterine tumors resembling ovarian sex cord tumors
Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 6 November 2011, last major update October 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.
● Rare tumors of uncertain etiology
● Most are benign with uniform cells, no infiltration and low mitotic index
● Malignant variants behave similar to low grade stromal sarcoma (may recur or metastasize)
● 62 year old woman with recurrent postmenopausal bleeding and uterine polyp (Cases J 2009;2:55)
● More cellular than endometrial stromal nodules, mature smooth muscle often present
● Sex cord-like elements are cords, tubules or sheets resembling epithelial cells, but with scanty cytoplasm, indistinct cell margins and round nuclei
● May have abundant eosinophilic or clear cytoplasm
● No prominent arterioles
Tumor cells resemble granulosa cells
● Often co-express sex cord (inhibin, calretinin, WT1, melanA), epithelial (KL1, EMA) and smooth muscle markers (smooth muscle actin, desmin, histone deacetylase 8), but variable staining for all markers (Am J Surg Pathol 2010;34:1749, Mod Pathol 2006;19:17)
● Also variable staining for CD10, CD117
● Polyphenotypic neoplasms with focal epithelial and variable sex cord-like differentiation, but no smooth muscle differentiation (Ultrastruct Pathol 2010;34:16)
● Endometrial stromal tumor: 60% have JAZF1-JJAZ1 gene fusion, not present in UTROSCT (Am J Surg Pathol 2009;33:1206)
● Vascular leiomyoma with plexiform pattern (Ann Diagn Pathol 2010;14:355) or leiomyoma with tubules (Int Semin Surg Oncol 2008;5:15)
End of Uterus > Stromal tumors > Uterine tumors resembling ovarian sex cord tumors
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