Uterus - Endometrial stromal tumors

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Uterus (excludes Cervix)

Stromal tumors

Endometrial stromal tumors - general

Reviewer: Mohamed Mokhtar Desouki, MD, PhD (see Reviewers page)
Revised: 21 August 2011, last major update August 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

General
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● May be benign (stromal nodule with pushing margins) or malignant (infiltrating margins, low or high grade)
● May contain separate areas of epithelium-like formations in the form of solid masses, glandular structures, or cords resembling sex-cord structures (also known as uterine tumors resembling sex-cord structures, which tend to behave in benign manner)
● Tumors with smooth muscle and stromal features should be classified and treated as stromal sarcomas (Mod Pathol 2000;13:328)
● Difficult to diagnose as nodule vs. sarcoma based on curettings because invasion cannot be assessed; but consider tumor of some kind if endometrial stroma is present without glands

Terminology
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● Includes endometrial stromal nodule, low-grade, endometrial stromal sarcoma, undifferentiated endometrial sarcoma

Epidemiology
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● Average age 45 years, present with vaginal bleeding

Prognostic factors
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● Endometrial stromal sarcomas are indolent tumors with a favorable prognosis
● Tumors are characterized by late recurrences commonly in the pelvis and abdomen, and less frequently in the lung and vagina
● Surgical stage is the most important prognostic parameter

Treatment
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● Hysterectomy and bilateral salpingo-oophorectomy

Gross description
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● Soft, yellow-orange

Micro description
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● Haphazard arrangement (not fascicular) of uniform small cells resembling endometrial stroma (blunt, spindled to oval with scant cytoplasm and small, uniform nuclei), individually enveloped by reticulin fibers
● Stromal cells circle small / thin vessels that resemble spiral arterioles; also hyalinization and foamy cells
● May have prominent vasculature resembling hemangiopericytoma

Positive stains
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● ER, PR, vimentin, actin
● Variable keratin

Negative stains
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● S100, h-caldesmon (Am J Surg Pathol 2001;25:455), desmin

Molecular description
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● Endometrial stromal sarcomas often carry the translocation t(7;17) with involvement of two zinc finger genes: JAZF1 and JJAZ1

End of Uterus > Stromal tumors > Endometrial stromal tumors - general

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