Soft tissue
Fibroblastic / myofibroblastic
Cellular angiofibroma
Author: Komal Arora, M.D.
Last author update: 1 July 2012
Last staff update: 6 February 2023 (update in progress)
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Cellular angiofibroma
Table of Contents
Definition / general | Clinical features | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Arora K. Cellular angiofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuecellularangiofibroma.html. Accessed June 10th, 2023.
Definition / general
- 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
- Rare; usually ages 40+ years
- Extragenital cases have similar features (APMIS 2007;115:254)
Case reports
- 44 year old woman with leiomyomatous nodules (Virchows Arch 2009;454:595)
- 49 year old woman with a mass in the posterior aspect of the left labia majora (J Clin Pathol 2002;55:477)
- 51 year old woman with coexisting Bartholin cyst (Sao Paulo Med J 2005;123:250)
Treatment
- Excision with negative margins
- Usually does not recur (Am J Surg Pathol 2004;28:1426)
Gross description
- 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
Microscopic (histologic) description
- 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
Microscopic (histologic) images
Images hosted on other servers:
Various images
Recurrent tumor
ER+
Occasional features
Typical features of CAF
Malignant cases
CD34+
Positive stains
- ER, PR, vimentin
- Smooth muscle actin (21%), CD34 (30 - 60%)
Molecular / cytogenetics description
- 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
- 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
