Cellular angiofibroma

Cellular angiofibroma
SpecialtyGynecology, Urology, Dermatology, Pathology, General surgery
SymptomsGrowth of a painless, soft tissue tumor in vulva-vaginal, inguinal-scrotal, or other areas
ComplicationsExtremely rare recurrence after surgical removal
Usual onsetAdults >30 years old
TypesBenign tumor
CausesUnknown
TreatmentSurgical resection
PrognosisExcellent
FrequencyRare
DeathsNone

Cellular angiofibroma (CAF) is a rare, benign tumor of superficial soft tissues that was first described by M. R. Nucci et al. in 1997.[1] These tumors occur predominantly in the distal parts of the female and male reproductive systems, i.e. in the vulva-vaginal and inguinal-scrotal areas, respectively, or, less commonly, in various other superficial soft tissue areas throughout the body.[2] CAF tumors develop exclusively in adults who typically are more than 30 years old.[3]

CAF tumors are composed of bland mesenchymal[4] spindle-shaped cells in an edematous (i.e. abnormally swollen with fluid) to fiber-laded connective tissue background.[2] Mesenchymal cells are cells in the cell lineage that includes fibroblasts, adipocytes (i.e. fat cells), macrophages, mast cells, leucocytes, and the precursor cells which mature into these cell types. In 2020, the World Health Organization classified cellular angiofibroma tumors in the category of benign fibroblastic and myofibroblastic tumors.[5]

CAF tumors rarely recur after surgical removal[3] and do not metastasize to distant tissues.[5] Accordingly, surgical resection is the commonly performed and current standard for treating these tumors.[6]

  1. ^ Nucci MR, Granter SR, Fletcher CD (June 1997). "Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma". The American Journal of Surgical Pathology. 21 (6): 636–44. doi:10.1097/00000478-199706000-00002. PMID 9199640.
  2. ^ a b Mandato VD, Santagni S, Cavazza A, Aguzzoli L, Abrate M, La Sala GB (July 2015). "Cellular angiofibroma in women: a review of the literature". Diagnostic Pathology. 10: 114. doi:10.1186/s13000-015-0361-6. PMC 4506619. PMID 26187500.
  3. ^ a b Libbrecht S, Van Dorpe J, Creytens D (March 2021). "The Rapidly Expanding Group of RB1-Deleted Soft Tissue Tumors: An Updated Review". Diagnostics (Basel, Switzerland). 11 (3): 430. doi:10.3390/diagnostics11030430. PMC 8000249. PMID 33802620.
  4. ^ Chapel DB, Cipriani NA, Bennett JA (January 2021). "Mesenchymal lesions of the vulva". Seminars in Diagnostic Pathology. 38 (1): 85–98. doi:10.1053/j.semdp.2020.09.003. PMID 32958293. S2CID 221842800.
  5. ^ a b Sbaraglia M, Bellan E, Dei Tos AP (April 2021). "The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives". Pathologica. 113 (2): 70–84. doi:10.32074/1591-951X-213. PMC 8167394. PMID 33179614.
  6. ^ Bloom J, Jordan E, Baratta VM, Zhang X, Saha A, Yavorek G, Kurbatov V (November 2020). "Cellular Angiofibroma Presenting as a Subepithelial Rectal Mass". ACG Case Reports Journal. 7 (11): e00471. doi:10.14309/crj.0000000000000471. PMC 7678802. PMID 33235884.