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Uterus
Stromal tumors
Endometrial stromal sarcoma
Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 21 June 2012, last major update January 2012
Copyright: (c) 2002-2012, PathologyOutlines.com, Inc.
Note: low grade and undifferentiated variants are described below
General
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● 0.2 to 1.5% of uterine malignancies; <10% of uterine sarcomas
● Low grade or high grade
Diagrams
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Diagnostic algorithm for uterine pure mesenchymal neoplasms - need not count mitotic figures
Gross images
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Micro description
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● Resembles endometrial stromal nodules but with infiltration, defined as irregular, jagged islands or tongues of neoplastic stromal cells between smooth muscle bundles of the surrounding normal myometrium
● May have angiolymphatic invasion (clumps of tumor cells present in spaces within the myometrium)
● May have glands without cuff of hypercellular stroma seen in adenosarcoma
Positive stains
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● CD10, muscle markers in areas of smooth muscle differentiation (muscle specific actin, smooth muscle actin and desmin)
Negative staining
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● c-kit/CD117, h-caldesmon (Am J Surg Pathol 2001;25:455) Low grade
● Also known as endolymphatic stromal myosis Case reports
● 53 year old woman with vaginal bleeding (Case of Week #246) Treatment
● Adjuvant therapy may reduce recurrence (Ann Oncol 2010;21:2102) Gross description
● Polypoid mass extending into broad ligament, ovaries and fallopian tubes Gross images
Micro description
● Monotonous ovoid to spindly cells with minimal cytoplasm intimately associated with prominent arterioles, closely resembles proliferative endometrial stroma Micro images
Cytology description
● Clean background; usually moderate to marked cellularity with both single cells and stromal fragments; usually also blood vessels interspersed between stromal cell clusters; cells predominantly spindled with scant to moderate cytoplasm, round to ovoid nuclei, fine chromatin; rare mitotic figures, nuclei (Am J Clin Pathol 2007;128:265) Positive stains
● ER, PR Negative stains
● Mucin, glycogen Molecular
● JAZF1-JJAZ1 fusion transcript in 50-60% (Am J Surg Pathol 2007;31:65) Differential diagnosis
● Stromal nodule: not infiltrative, no angiolymphatic invasion Additional references
● Mod Pathol 2000;13:328, Mod Pathol 2001;14:465 Undifferentiated
● Much less common than low grade endometrial stromal sarcoma Gross description
● Diffusely involves most of endometrial surface Gross images
Micro description
● 10+ mitotic figures per 10 HPF, often abnormal Micro images
Positive stains
● 50% of tumors with uniform nuclei express beta-catenin and cyclin D1 (Mod Pathol 2010;23:225) Molecular
● YWHAE-FAM22A/B genetic rearrangement may be specific for high grade tumors (Proc Natl Acad Sci USA 2012 Jan 5 [Epub ahead of print]) Differential diagnosis
● Leiomyosarcoma
End of Uterus > Stromal tumors > Endometrial stromal sarcoma
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● Slow clinical progression with repeated local recurrences
● 50% recur (may take 10 years), 15% die of metastases (lung) but may be 20-30 years later
● Rarely arise from ovary, vagina and peritoneum
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● Lymphatic tumor plugs may appear as yellow, ropy or ball-like masses
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Endometrial stromal sarcoma
#1, #2, #3
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● Up to 10-15 mitotic figures per 10 HPF in most active areas
● Tongue-like infiltration between muscle bundles of myometrium
● Angiolymphatic invasion common
● May exhibit myxoid, epithelioid and fibrous change
● May have foam cells or hyalinization
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Resembles proliferative phase endometrium
With glands
Various images
Various images
Dilated and slit-like thin-walled vessels are prominent
Dilated vessels and sex cord-like structures
Extensive myometrial infiltration. Note also slit-like vascular or lymphatic spaces around tumor masses, which represent vascular involvement
Myometrial infiltration by uniform small tumor cells
Myometrial lymphatics are expanded and infiltrated by tumor; the lobulated contour of this intravascular tumor is unusual, resembling intravenous leiomyomatosis
Nests and sex cord-like arrangements blend with intervening stromal cells
Low grade endometrial stromal sarcoma With intravenous extension to the heart
Beta-catenin and CD10
Diffuse CD10 immunoreactivity is seen in histologic variants
H&E #1, #2, #3, #4
PR+, CD10+, smooth muscle actin+
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● Diffuse, strong nuclear immunoreactivity with beta-catenin in 67% (Mod Pathol 2008;21:756)
● AE1-AE3 in 50% (usually due to CK19+, (Hum Pathol 2008;39:1459)
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● Adenomyosis with sparse glands: usually incidental finding in post-menopausal woman with small and atrophic stromal cells, typical areas of adenomyosis usually present
● Cellular leiomyoma: no infiltration, no angiolymphatic invasion
● Menstrual endometrium within vessels: usually glands are uniformly distributed with bland stroma
● Metastatic lobular carcinoma: check clinical history, strongly keratin+
● Pseudosarcomatous changes in the stroma
● Fragmented lymphoid follicles in biopsies or curettings
● Intravascular leiomyomatosis
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● 5 year survival of 50% with recurrences in pelvis and metastases to lung
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● Frequently extends beyond uterus
● Vascular invasion not as evident as low grade stromal sarcomas
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Diffuse infiltration of uterine serosa
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● Angiolymphatic invasion common
● Infiltrative margins, usually extensive myometrial invasion
● Marked nuclear atypia, enlargement, variable pleomorphism
● May be useful to comment on nuclear uniformity or pleomorphism (Am J Surg Pathol 2008;32:1228)
● Tumor cells do NOT resemble endometrial stroma, no arborizing vasculature
● Resembles stromal component of MMMT
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The round cells resemble endometrial stromal cells but are large and pleomorphic; the regular vascular pattern of low-grade tumors is absent
Anaplastic nuclear features and mitotic figures
Immunohistochemistry of β-catenin and cyclin D1
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● Lymphoma
● Undifferentiated carcinoma
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