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Uterus (excludes Cervix)

Stromal tumors

Leiomyosarcoma


Reviewer: Mohamed Mokhtar Desouki, MD, PhD (see Reviewers page)
Revised: 22 August 2011, last major update August 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

General
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● 1-2% of uterine malignancies, but most common uterine sarcoma
● Probably not derived from leiomyomas

Epidemiology
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● Peaks at ages 40-69 years; mean is 54 years

Clinical features
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● Abnormal vaginal bleeding, palpable mass and pelvic pain
● Presentation may be related to tumor rupture (hemoperitoneum) or due to extrauterine extension
● 5 year survival 40%, only 10% if anaplastic; minimal survival if extends beyond uterus

Prognostic factors
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● Very aggressive even when confined to uterus
● Recurrence rate of 53% to 71%
● Overall survival rate ranges from 15% to 25% with a median survival of only 10 months
● Tumor size, tumor gradeand stage are major prognostic parameters

Treatment
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● Total abdominal hysterectomy and debulking of tumor
● Radiotherapy may be useful
● Chemotherapy with doxorubicin or docetaxel/gemcitabine is used for advanced or recurrent disease
● Hormonal treatment in subset of patients

Gross description
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● Bulky fleshy tumor invading into myometrial wall or polypoid tumor projecting into lumen
● Often hemorrhagic or necrotic
● Grossly appears invasive/infiltrative
● Usually 5 cm or more, but NOT multiple

Micro images
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Various images

Micro description
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● Hypercellular with spindle cells resembling smooth muscle with moderate to severe pleomorphism
● Infiltrative border is most helpful feature for diagnosis
● 10+ mitotic figures per 10 high power fields (HPF) in most mitotically active area with abundant abnormal mitotic figures
● Donít interpret small pyknotic nuclei from smooth muscle cells as mitoses; use only definitive mitotic figures
● Coagulative tumor cell necrosis is common
● Rarely contains osteoclast-like giant cells
● Epithelioid and myxoid leiomyosarcomas are rare variants with mild nuclear atypia and often <3 mitotic figures/10 HPF
Smooth muscle tumors of uncertain malignant potential (STUMP): tumor cell necrosis in a typical leiomyoma; necrosis of uncertain type with ≥10 MF/10 HPFs or marked diffuse atypia; marked diffuse or focal atypia with borderline mitotic counts and necrosis difficult to classify

Micro images
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Various images


Giant cells and a few mitotic figures

           
Contributed by Dr. Jamie Shutter, George Washington University

   
With endometrioid carcinoma


H&E and CD10 (figures 1C & 1D)

Positive stains
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● Smooth muscle actin, myosin, desmin
● Ki-67, ER, PR, EMA
● h-caldesmon (variable, Am J Surg Pathol 2001;25:253), keratin (epithelioid tumors), p53, focal CD10, histone deacetylase 8 (HDCA8), androgen receptor (variable), and c-KIT (variable)

Negative stains
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● CD44v3 (Hum Pathol 2001;32:1190)

Molecular description
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● p53 mutations and overexpression in 25-47%, but not in leiomyomas
● p16 overexpression
● Patients with germline mutations of fumarate hydratase have increased risk for uterine leiomyosarcomas and uterine leiomyomas

Differential Diagnosis
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● Endometrial stromal sarcoma with smooth muscle metaplasia
● Mitotically active leiomyoma
● Cellular leiomyoma
● Hemorrhagic leiomyoma and hormone-induced changes
● Leiomyoma with bizarre nuclei (atypical leiomyoma)
● Myxoid leiomyoma
● Epithelioid leiomyoma
● Leiomyoma with massive lymphoid infiltration

End of Uterus > Stromal tumors > Leiomyosarcoma


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