Microscopic description:

Microscopic images demonstrate bland spindle shaped cells with fusiform nuclei and pale eosinophilic cytoplasm with wispy collagen bundles in the extracellular matrix. Small to medium sized thick walled hyalinized vessels and scattered adipose tissue.

Ancillary testing:
  • Positive: Desmin and CD34
  • Negative: CD117s

Discussion:

Cellular angiofibroma(CAF) is a rare benign tumor that has been described in several case studies in children and adults. In terms of the genital and pelvic regions, it is most associated with the labium and vulvovaginal areas in females, and superficially in the scrotum and inguinal canal in males. Periurethral and pelvic CAF is rather rare with only a few case studies describing patients with symptoms and simple surgical excision (Emtage 2013, Kumar 2018). All published cases recommend close follow up of lesions and in the case of rapid increase in size, prompt surgical excision of mass with negative margins is appropriate with the goal of preventing recurrence (Arena 2016).

CAF have been described as more common in women as demonstrated by Mandata et al when they gathered 79 cases of CAF in women from 1997 to 2014. They found that 56% of these CAF cases showed tumor growth in the vulvovaginal and labium region. Similarly, most male cases have been described in analogous male genitalia such as the scrotum and inguinal regions, and only a handful have been described in the pelvic periurethral region (Emtage 2013).

CAF is usually a well circumscribed mass or with a variable pseudo-capsule and often involves superficial tissues.

Microscopic images demonstrate bland spindle cells with scant, eosinophilic cytoplasm with oval to fusiform nucleus and with wispy collagen bundles and prominent small to medium-sized vessels with hyaline fibrosis in walls.

Cellular angiofibroma has a rare recurrence and there are no standard guidelines for treatment. A recent case report from Kumar et al in 2018 described a female periurethral mass that had been enlarging to 2-3 cm over 8 years. Simple surgical excision of this mass resulted in cure with no signs of recurrence in the 2 year follow up period.

References:

Emtage JB, Parker J, Marcet J, Finan J, Lockhard J, Hernandez D. “A large cellular angiofibroma of the male pelvis presenting with obstructive voiding: A case report and review of the literature”. Can Uro Assoc J. 2013 May; v.7(5-6): E373 – E375.

Nucci MR, Granter SR, Fletcher CD. “Cellular Angiofibroma: A Benign Neoplasm Distinct from Angiomyofibroblastoma and Spindle Cell Lipoma”. Am J Surg Path. 1997 June; V.21(6): 636-644.

Qiu P, Wang Z, Li Y, and Cui G. “Giant pelvic angiomyofibroblastoma: a case report and literature review”. Diagnostic Pathology. 2014; 9:106.

Kumar P, Singh S, Kumar A, Janoria S. “A rare care of cellular angiofibroma affecting the peirurethral region in a 30 year old woman”. BMJ Case Rep. 2018 Sept.

Laskin WB, Fetsch JF, Mostofi FK. Angiomyofibroblastomalike tumor of the male genital tract: analysis of 11 cases with comparison to female angiomyofibroblastoma and spindle cell lipoma. Am J Surg Pathol. 1998 Jan; 22: 6–16.

Arena S, Barresi V, Romeo C. “Giant angiofibroma of the scrotum in a child”. Urology. 2016 July: Vol (93); e15-e16.