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Soft tissue Tumors

Fibroblastic / myofibroblastic tumors

Angiomyofibroblastoma

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, well-circumscribed myofibroblastic tumor, usually in vulva
● May overlap with cellular angiofibroma
● First described in 1992 (Am J Surg Pathol 1992;16:373)

Clinical description
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● Uncommon slow growing painless mass, usually females of reproductive age

Sites
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● Usually vulvar, 10-15% in vagina
● Also scrotum or paratesticular soft tissue in men (median age 57 years)

Treatment
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● Excision, only rarely recurs, does not metastasize

Case reports
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● 27 year old man with inguinal tumor (Arch Pathol Lab Med 2000;124:1679)
● 48 year old woman with pedunculated vulvar mass (Acta Med Okayama 2006;60:237)
● 49 year old man with scrotal mass (Univ Oklahoma)
● 80 year old woman with sarcomatous transformation in vulva (Am J Surg Pathol 1997;21:1104)
● Recurrence in vagina (Eur J Gynaecol Oncol 2007;28:324)

Gross description
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● Well circumscribed but not encapsulated, usually 5-7 cm or less, tan-pink soft cut surface, no necrosis

Gross images
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Scrotal mass

Micro description
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● Thin fibrous pseudocapsule, alternating hyper- and hypocellular areas with prominent thin walled and ectatic vessel in edematous stroma (Mod Pathol 1996;9:284)
● Round to spindled tumor cells have eosinophilic cytoplasm and often bi- or multi-nucleation
● May have plasmacytoid or epithelioid morphology, or rarely show degenerative changes
● Tumor cells are concentrated around vessels
● Mast cells common
● 10% have mature adipose tissue
● No/rare mitotic figures, no/rare red blood cell extravasation, no atypia
● Most menopausal patients have less cellularity, less edema but more fibrosis and vessel wall hyalinization

Micro images
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Numerous capillary-like vascular channels; spindle cells with occasional multinucleated cells

               
Scrotal mass-various images


Arising from posterior perivesical space

Positive stains
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● Vimentin, desmin (strong and diffuse, may be reduced in post-menopausal patients), ER, PR, smooth muscle actin or pan-muscle actin (focal, Hum Pathol 1997;28:1046), occasional CD34

Negative stains
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● S100, keratin

Electron microscopy description
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● Myofibroblastic and fibroblastic features

Differential diagnosis
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● Aggressive angiomyxoma: not circumscribed, > 5 cm, less cellular, less vascular but vessels are large and thick walled, stromal mucin and RBC extravasation are present, infrequent plump stromal cells, usually actin negative
● Cellular angiofibroma: more cellular uniformly, perivascular hyalinization and large, thick walled vessels, usually actin/desmin negative
● Epithelioid leiomyoma: more cellular, no biphasic pattern, usually no binucleation, no/rare mast cells

Additional references
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● Ultrastruct Pathol 2006;30:193, Stanford School of Medicine

End of Soft Tissue Tumors > Fibroblastic / myofibroblastic tumors > Angiomyofibroblastoma

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