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Case of the Week #246

21 June 2012 - Case of the Week #246

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Thanks to Dr. Shahrzad Ehdaivand, Women & Infants Hospital & Warren Alpert Medical School of Brown University, for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.

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Case of the Week #246

Clinical History:

A 53 year old woman had a hysterectomy for vaginal bleeding.

Micro images:



Smooth muscle myosin

What is your diagnosis?


Endometrial Stromal Sarcoma


Endometrial stromal tumors may be benign (stromal nodule with pushing margins) or malignant. They have a haphazard (not fascicular) arrangement of uniform small cells resembling endometrial stroma; i.e. blunt, spindled to oval cells with scant cytoplasm and small, uniform nuclei. Each cell is individually enveloped by reticulin fibers. The stromal cells surround small / thin vessels that resemble spiral arterioles, but the vasculature may also resemble hemangiopericytoma

Endometrial stromal sarcomas, as in this case, often have tongue-like infiltrating margins and lymphovascular invasion. Low grade tumors resemble proliferating endometrium with uniform cells intimately associated with prominent arterioles. There is usually little cytological atypia or pleomorphism, and mitoses are scanty. The JAZF1-JJAZ1 fusion transcript is present in 50-60% of cases (Am J Surg Pathol 2007;31:1277).

In contrast, undifferentiated endometrial sarcomas are frankly malignant, and lack specific differentiation and any features of normal endometrial stroma. They often have myometrial invasion, hemorrhage and necrosis, as well as marked nuclear pleomorphism and high mitotic activity. These tumors may have the YWHAE-FAM22A/B genetic rearrangement (Am J Surg Pathol 2012;36:641).

The differential diagnosis of low grade endometrial stromal sarcoma includes:
Endometrial stromal nodule: no/minimal infiltration, no angiolymphatic invasion, < 10 MF/HPF
Adenomyosis with sparse glands: this is usually an incidental finding in post-menopausal women with typical areas of adenomyosis usually present
Cellular leiomyoma: no infiltration, no angiolymphatic invasion

In low grade endometrial stromal sarcoma, even after hysterectomy, there is often slow clinical progression with repeated local recurrences in up to 50%. Death due to disease occurs in 15%, often due to lung metastases, which may occur 30 years later.

Nat Pernick, M.D., President
and Liz Parker, B.A., Associate Medical Editor
PathologyOutlines.com, Inc.
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