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Uterus
Stromal tumors
Adenosarcoma
Reviewer: Sanjay Logani, M.D., InCyte Pathology (see Reviewers page)
Revised: 21 September 2012, last major update June 2010
Copyright: (c) 2002-2012, PathologyOutlines.com, Inc.
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● A rare, low grade, biphasic, malignant mixed müllerian tumor (MMMT), usually in elderly, with benign epithelial and sarcomatous mesenchymal components
● First described in 1974 (Cancer 1974;34:1138)
Terminology
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● Also called mullerian adenosarcoma
Epidemiology
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● 5-7% of uterine sarcomas
● Median age 58 years, range 13-89 years (Hum Pathol 1990;21:363)
Sites
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● Usually uterus or cervix, also elsewhere in gynecologic tract
● Rare cases reported involving the intestine
Etiology
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● Rosai considers these tumors to be a variant of endometrial stromal sarcoma with the capacity to form glands, including sex-cord-like differentiation
Clinical features
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● Often presents with abnormal vaginal bleeding, enlarged uterus and tissue protruding from external os
● May occasionally present as low grade endometrial polyps, with subtle low grade sarcomatous features (J Clin Pathol 2006;59:801) or at biopsy with no sarcomatous features (diagnosis requires re-excision)
● Usually stage IA or IB (Am J Surg Pathol 2009;33:278)
● Younger patients are often misdiagnosed (Aust N Z J Obstet Gynaecol 2008;48:596)
Prognostic factors
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● Poor prognostic factors are high grade sarcomatous overgrowth, myometrial invasion or high grade tumor
Case reports
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● 42 year old woman with uterine mass post tamoxifen for breast cancer (contributed by Dr. Najla Fakruddin)
● 45 year old woman with lymphovascular emboli (Cases J 2009 May 18;2:7515)
Treatment
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● Hysterectomy is usually curative
● May recur in pelvis or vagina; rarely metastasizes
Gross description (Macroscopy)
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● Bulky, polypoid and multicystic neoplasm arising from endometrium and filling endometrial cavity
● May be fleshy
● Mean 6 cm, but up to 20 cm
● Less hemorrhage and necrosis than MMMT
Gross images
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Large polypoid mass fills endometrial cavity
Micro description (Histopathology)
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● Epithelial and stromal elements with stromal hypercellularity
● Epithelial component appears benign; glands are usually large and dilated with periglandular stromal cuffing, 80% have cambium layer (stromal condensation) beneath surface epithelium and adjacent to glands (most characteristic histologic feature); mitotic activity and cytologic atypia are more common in this zone
● Epithelium is usually endometrioid but also ciliated, mucinous and even squamous
● Stroma has polypoid or leaf-like projections into glandular lumina, resembling phyllodes tumor of breast
● Stromal elements have mild or occasionally moderate atypia; resemble low grade endometrial stromal sarcoma but less bizarre and less undifferentiated
● 33% have sarcomatous overgrowth
● 25-47% are myoinvasive
● 20% have multinucleated giant cells and heterologous elements (skeletal muscle)
● May also have extensive stromal fibrosis
● Occasionally sex cord like elements (Am J Clin Pathol 1989;91:664)
● Usually 1+ mitotic figure/10 HPF in stromal cells
Micro images
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Left: leaf-like appearance similar to phyllodes tumor; stroma is more cellular than adenofibroma
Right: cuffing of stroma around compressed slit-like gland
Dilated glands surrounded Cambium layer
by cuff of stomal cells
AFIP #192: Tumor arises in endometrial polyp. AFIP #194-The cellular stroma forms
One compressed gland is cuffed by hypercellular cuffs surrounding the benign
hypercellular and atypical stroma. The other appearing, mostly slit-like glands. Foci
glands, some dilated, are separated by the of squamous metaplasia are present.
usual stroma of an endometrial polyp
AFIP #196: Several benign appearing glands AFIP #197: Foam cells form sex-cord like
show eosinophilic cell change. Cuffing by structures in the stroma.
hypercellular stroma is apparent.
AFIP #198: A hypercellular cuff of stroma AFIP #199: A large focus of well-differentiated
surrounds the single benign gland, and chondrosarcoma is present, as well as a foci
another hypercellular band of stroma shows of squamous metaplasia and of hypercellular
rhabdomyosarcomatous differentiation. stromal cuffs around the glandular component.
Other images: epithelial glands show proliferation, but no obvious atypia; cambium layer with marked atypia; benign epithelial cells and malignant stromal cells with mitotic figure; malignant stromal cells
Cytology images
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Other images: dense clusters of polymorphic tumor cells with elongated hyperchromatic nuclei of various sizes; denuded malignant cells with variable sizes; long oval, hyperchromatic nuclei with 1+ nucleoli (Pap); tumor cells have variable sizes; partly denuded tumor cells with fragile cytoplasm
Positive stains
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Mesenchymal component, in areas without sarcomatous overgrowth:
● ER, PR, WT1, CD10 (Am J Surg Pathol 2008;32:1013)
● Variable muscle markers, keratin (epithelial component), androgen receptor
Electron microscopy descriptions
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● Features of proliferative endometrium; stroma resembles endometrial stromal sarcoma or mixed müllerian tumor (Am J Clin Pathol 1979;71:112)
Differential Diagnosis
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● Adenofibroma: may be extremely difficult to distinguish on a curettage specimen; no hypercellular stroma, no cambium layer, low mitotic index (< 2/10 HPF), at most mild stromal nuclear atypia
● Adenomyomatous polyp: attenuated surface endometrium on 3 sides; cystic change; large, thick walled blood vessels, no stromal hypercellularity, no cuffing, no atypia
● Carcinosarcoma: malignant epithelial (at least focally) and sarcomatous elements
● Embryonal rhabdomyosarcoma (children): evidence of skeletal muscle differentiation (histology, immunohistochemistry or EM)
● Endometrial stromal sarcoma: often intravascular growth, tongue-like infiltration, arborizing vessels; may have glands, but they are smaller and not dilated; no periglandular stromal condensation, no intraluminal polypoid protrusions
● Endometriosis, polypoid: resembles adenosarcoma particularly when occurring in cervix or peritoneum
Additional references
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● Mod Path 2000;13:328, Adv Anat Pathol 2010;17:122
End of Uterus > Stromal tumors > Adenosarcoma
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