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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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#1

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
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● Also known as endolymphatic stromal myosis
● 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

Case reports
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● 53 year old woman with vaginal bleeding (Case of Week #246)

Treatment
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● Adjuvant therapy may reduce recurrence (Ann Oncol 2010;21:2102)

Gross description
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● Polypoid mass extending into broad ligament, ovaries and fallopian tubes
● Lymphatic tumor plugs may appear as yellow, ropy or ball-like masses

Gross images
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Endometrial stromal sarcoma

#1, #2, #3

Micro description
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● Monotonous ovoid to spindly cells with minimal cytoplasm intimately associated with prominent arterioles, closely resembles proliferative endometrial stroma
● 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

Micro images
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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+

Cytology description
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● 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
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● ER, PR
● 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)

Negative stains
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● Mucin, glycogen

Molecular
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● JAZF1-JJAZ1 fusion transcript in 50-60% (Am J Surg Pathol 2007;31:65)

Differential diagnosis
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Stromal nodule: not infiltrative, no angiolymphatic invasion
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

Additional references
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Mod Pathol 2000;13:328, Mod Pathol 2001;14:465


Undifferentiated
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● Much less common than low grade endometrial stromal sarcoma
● 5 year survival of 50% with recurrences in pelvis and metastases to lung

Gross description
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● Diffusely involves most of endometrial surface
● Frequently extends beyond uterus
● Vascular invasion not as evident as low grade stromal sarcomas

Gross images
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Diffuse infiltration of uterine serosa

Micro description
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● 10+ mitotic figures per 10 HPF, often abnormal
● 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

Micro images
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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

Positive stains
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● 50% of tumors with uniform nuclei express beta-catenin and cyclin D1 (Mod Pathol 2010;23:225)

Molecular
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● 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
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Leiomyosarcoma
Lymphoma
Undifferentiated carcinoma

End of Uterus > Stromal tumors > Endometrial stromal sarcoma


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