Soft tissue
Fibroblastic / myofibroblastic
Angiomyofibroblastoma
Author: Komal Arora, M.D.
Topic Completed: 1 July 2014
Minor changes: 20 November 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: Angiomyofibroblastoma
Table of Contents
Definition / general | Clinical features | Sites | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Arora K. Angiomyofibroblastoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueangiomyofibroblastoma.html. Accessed January 16th, 2021.
Definition / general
- 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 features
- Uncommon slow growing painless mass, usually females of reproductive age
Sites
- Usually vulvar, 10 - 15% in vagina
- Also scrotum or paratesticular soft tissue in men (median age 57 years)
Case reports
- 27 year old man with angiomyofibroblastoma of the male inguinal region (Arch Pathol Lab Med 2000;124:1679)
- 48 year old woman with angiomyofibroblastoma of the vulva (Acta Med Okayama 2006;60:237)
- 80 year old woman with angiomyofibroblastoma of the vulva with sarcomatous transformation (Am J Surg Pathol 1997;21:1104)
- Recurrent angiomyofibroblastoma of the vagina (Eur J Gynaecol Oncol 2007;28:324)
Treatment
- Excision, only rarely recurs, does not metastasize
Gross description
- Well circumscribed but not encapsulated, usually 5 - 7 cm or less, tan-pink soft cut surface, no necrosis
Gross images
Contributed by Mark R. Wick, M.D.
Scrotum
Microscopic (histologic) description
- 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
Microscopic (histologic) images
Contributed by Mark R. Wick, M.D.
Scrotum
Vulva
Images hosted on other servers:
Arising from posterior perivesical space
Numerous capillary-like vascular channels
Positive stains
- 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
Electron microscopy description
- Myofibroblastic and fibroblastic features
Differential diagnosis
- 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
