Cellular angiofibromas are rare tumors found in the adult population. There is no gender predominance. The tumors show a peak incidence in the 5th decade of life in women, whereas men are usually affected in the 7th decade 1-3.
Usually, the tumor manifests as a slowly progressive well-defined indolent mass 1.
In men, cellular angiofibromas can be associated with hydrocele testis or inguinal hernias 1.
Cellular angiofibromas are richly vascularized cellular fibroblastic tumors consistent of multiple thick-walled vessels usually arising in the vulvovaginal or inguinal and scrotal regions 1-4.
The etiology of cellular angiofibroma is unknown 1.
Cellular angiofibromas are typically found in the superficial soft tissues of the genital region namely the vulvovaginal area in women and the paratesticular and inguinal areas in men, with rare examples in other body locations 1-6.
The gross appearance of cellular angiofibromas is that of a well-defined round to oval or lobulated tumor with grey-pinkish to brown-yellowish color and a solid soft to rubbery consistency 1-3.
Microscopically cellular angiofibromas show the following features 1-3:
- moderately to highly cellular tumor with an unorganized random or short fascicular pattern
- small uniform short spindle-shaped cells with pale eosinophilic cytoplasm and ill-defined borders
- edematous fibrous stroma containing short wispy collagen bundles and with focal myxoid changes or hyalinized background in a part of the cases
- prominent small to medium-sized thick-walled vessels with intramural hyaline fibrosis
- no necrosis
- no atypical mitotic activity
Immunohistochemistry stains express estrogen receptor, progesterone receptor in many cases and CD34 in about 30-60%. A minority of cases show variable expressions of desmin and smooth muscle actin (SMA) 1.
Cellular angiofibromas frequently feature a deletion of the RB1 gene on chromosome 13q14 1.
Cellular angiofibromas are well defined highly vascularized lesions in the genital region 4.
Ultrasound findings have been described as well-marginated with hypoechoic or heterogeneous architecture 5.
Cellular angiofibromas have been reported as heterogeneously enhancing soft tissue mass after contrast administration 4,7.
Cellular angiofibromas have been described to appear as solid well-circumscribed variably heterogeneous soft tissue lesions 4-6.
- T1: low to intermediate signal intensity
- T2: heterogeneous intermediate to high signal intensity
- T2FS/STIR: intermediate to high signal intensity
- T1 C+ (Gd): avid heterogeneous enhancement
The radiological report should include a description of the following:
- location and size of the tumor
- relation to other organs
- relation to soft tissue structures structures
Treatment and prognosis
Cellular angiofibromas are benign tumors and the diagnosis is often established after excision. Local recurrences are very infrequent. Sarcomatous transformation or cell atypia exists but there are apparently no known cases, where this has changed prognosis or led to recurrence 1.
History and etymology
Cellular angiofibroma was first described by MR Nucci and colleagues in 1997 8.
The differential diagnosis of cellular angiofibroma includes the following tumors 1-4:
- 1. W.H.O. Classification WHO Classification of Tumours Editorial Board, Who Classification of Tumours Editorial. Soft Tissue and Bone Tumours. (2020) ISBN: 9789283245025
- 2. Flucke U, van Krieken JH, Mentzel T. Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. (2011) Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc. 24 (1): 82-9. doi:10.1038/modpathol.2010.170 - Pubmed
- 3. Mandato VD, Santagni S, Cavazza A, Aguzzoli L, Abrate M, La Sala GB. Cellular angiofibroma in women: a review of the literature. (2015) Diagnostic pathology. 10: 114. doi:10.1186/s13000-015-0361-6 - Pubmed
- 4. Kamitani R, Matsumoto K, Fujiwara S, Akita H, Mikami S, Kameyama K, Jinzaki M, Oya M. A case of inguinal cellular angiofibroma. (2020) IJU case reports. 3 (2): 69-71. doi:10.1002/iju5.12147 - Pubmed
- 5. Van Mulders S, Faes E, Broeckx G, Jacquemyn Y. Cervicovaginal cellular angiofibroma. (2020) BMJ case reports. doi:10.1136/bcr-2020-235241 - Pubmed
- 6. Ntorkou AA, Tsili AC, Giannakis D, Batistatou A, Stavrou S, Sofikitis N, Argyropoulou MI. Magnetic resonance imaging findings of cellular angiofibroma of the tunica vaginalis of the testis: a case report. (2016) Journal of medical case reports. 10: 71. doi:10.1186/s13256-016-0861-3 - Pubmed
- 7. Chun KH, Inn FX, Hing EY, Hong GE. Imaging modalities for an uncommon inguinal scrotal pathology: A case report and literature review. (2017) Urology annals. 9 (4): 387-389. doi:10.4103/UA.UA_69_17 - Pubmed
- 8. Nucci MR, Granter SR, Fletcher CD. Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma. (1997) The American journal of surgical pathology. 21 (6): 636-44. doi:10.1097/00000478-199706000-00002 - Pubmed