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Soft tissue Tumors

Fibroblastic / myofibroblastic tumors

Cellular angiofibroma


Reviewer: Komal Arora, M.D. (see Reviewers page)
Revised: 19 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Benign, highly cellular tumor of vulva and scrotum/inguinal region with prominent blood vessels
● Also called angiomyofibroblastoma-like lesion
● First described in 1997 (Am J Surg Pathol 1997;21:636)
● Related to spindle cell lipoma and mammary-type myofibroblastoma, with morphological variations dependent on anatomic location (Mod Pathol 2011;24:82)
● In women, may arise from hormone receptor positive mesenchymal cells in lower female genital tract (Histopathology 2004;45:360)

Clinical features
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● Rare; usually ages 40+ years
● Extragenital cases have similar features (APMIS 2007;115:254)

Treatment
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● Excision with negative margins
● Usually does not recur (Am J Surg Pathol 2004;28:1426)

Case reports
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● 44 year old woman with leiomyomatous nodules (Virchows Arch 2009;454:595)
● 51 year old woman with coexisting Bartholinís cyst (Sao Paulo Med J 2005;123:250)
● Recurrent tumor (J Clin Pathol 2002;55:477)

Gross description
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● Vulvar lesions are usually up to 3 cm, male lesions may reach 14 cm
● Well circumscribed nodules with soft to rubbery, gray-pink-brown cut surface

Micro description
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● Well circumscribed, variable pseudocapsule
● Cellular tumor with fascicles or haphazard pattern
● Bland spindle cells with scant, lightly eosinophilic cytoplasm with ill defined borders, oval to fusiform nucleus
● May be epithelioid
● Prominent small to medium-sized vessels with hyaline fibrosis in walls
● Vessels may have degenerative changes of fibrin thrombi, intramural inflammation, hemosiderin
● Some (usually 5% or less) adipose tissue present in 50%
● Stroma is usually fine collagenous fibers
● Scattered mast cells are common
● Female lesions may have brisk mitotic activity, usually absent/rare in males
● Occasional atypia or sarcomatous transformation (Am J Surg Pathol 2010;34:707)
● No necrosis, no atypical mitotic figures

Micro images
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Various images


Fusiform cells, thick walled blood vessels and adipose tissue


Fusiform cells with clear cytoplasm and bland nuclei;

       
Occasional features


Recurrent tumor

       
Malignant cases


ER+

Virtual slides
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63 year old man with scrotal mass

Positive stains
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● ER, PR, vimentin
● Smooth muscle actin (21%), CD34 (30-60%)

Negative stains
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● S100, desmin, EMA

Cytogenetics / Molecular
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● May have 13q14 involvement (Histopathology 2007;51:410)
● Similar changes as spindle cell lipoma (Cancer Genet Cytogenet 2007;177:131)
● FISH: monoallelic deletion of RB1

Differential diagnosis
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Aggressive angiomyxoma: usually large and deep, hypocellular, infiltrative margin, desmin+
Angiomyofibroblastoma: less uniform cellularity, smaller vessels, usually desmin+
Leiomyoma: spindled cytoplasm, actin+, desmin+
● Perineurioma
● PHAT
Solitary fibrous tumor: hyper- and hypocellular areas, prominent staghorn vessels, hyalinized collagen CD34+

Additional references
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Stanford School of Medicine, Histopathology 2009;54:156

End of Soft Tissue Tumors > Fibroblastic / myofibroblastic tumors > Cellular angiofibroma


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