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29 July 2011 – Case of the Week #211
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Case of the Week #211
A 57 year old woman presented with post-menopausal bleeding. An endometrial polyp was removed piecemeal at hysteroscopy, the largest fragment was 18 mm.
Inhibin (left) and Melan-A (right)
ER (left), PR (middle), CD10 (right)
What is your diagnosis?
Uterine tumour resembling ovarian sex cord tumors (UTROSCT)
Tumors of the uterus resembling ovarian sex cord tumors were first described by Clement and Scully (Am J Clin Pathol 1976;66:512). They are divided into 2 groups: group 1 - endometrial stromal tumors with sex cord-like elements (ESTSCLE), and group 2 - mural uterine tumors. In group 1 tumors, the sex cord component constitutes a minor portion of an endometrial stromal neoplasm. In group 2 tumors, it is the predominant or exclusive component of a uterine wall lesion composed of various mesenchymal elements.
UTROSCT are polypoid or nodular neoplasms, which generally arise in the fourth to sixth decade of life. Grossly, they are polypoid or nodular, typically in the myometrium. Histologically, they are well circumscribed, polyphenotypic neoplasms, with a predominant, if not exclusive, pattern of cords, nests and trabeculae containing epitheloid cells resembling granulosa or Sertoli cell tumors of the ovary. True Leydig cell differentiation is rare (Mod Pathol 2006;19:17).
These tumors typically express at least two “sex cord markers” of calretinin, inhibin, CD99 and Melan A (Int J Gynecol Pathol 2008;27:229), although a recent study substituting WT1 for CD99 found less frequent immunoreactivity (Am J Surg Pathol 2010;34:1749). They also express myoid markers, epithelial markers and hormone receptors.
The origin of these tumors is uncertain. They may derive from an uncommitted cell with the capacity to differentiate along several lines (J Clin Pathol 2007;60:1148). Although these tumors often express smooth muscle markers, there are no ultrastructural features of smooth muscle differentiation (Ultrastruct Pathol 2010;34:16). Although they resemble ESTSCLE, they lack the JAZF1-JJAZ1 translocation frequently associated with endometrial stromal tumors (Am J Surg Pathol 2009;33:1206).
The differential diagnosis includes metastatic ovarian sex cord tumors (different clinical history) and mixed mullerian tumors (histologic evidence of malignancy). If one inappropriately focuses on immunohistochemistry results, an erroneous diagnosis of a myoid tumor (such as vascular plexiform leiomyoma, Ann Diagn Pathol 2010;14:355) or an epithelial tumor may occur.
Excision by hysterectomy is usually curative, although some recommend fertility-sparing surgery in select patients, with careful follow up. Tumors of the uterus resembling ovarian sex cord tumors typically have benign behavior, although metastases have rarely been reported (Gynecol Oncol 2009;114:140, Int J Gynecol Pathol 2008;27:58).
Nat Pernick, M.D., President
and Liz Parker, B.A., Associate Medical Editor
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Alternate email: NatPernick@gmail.com