Three cases of paraurethral angiofibroblastoma

Three case reports of angiofibroblastoma.

Case 1

A 32 year old lady presented with an asymptomatic, but gradually enlarging vaginal lump over 3 months. On examination, there was a small 3x2 cm rubbery tissue mass arising from the left paraurethral region. An examination under anesthesia and excisional biopsy was thus organized. However she defaulted on her surgery due to anxiety, and failed to attend her follow up appointment. Six months later she returned complaining od pain and bleeding on voinding. On examination, the solitary paraurethral lump had noe enlarged to a size 8x8x4 cm, amd had become ulcerated and infected ( figure 1). There was no associated inguinal lymphaadenopathy. Due to the pain and voiding difficulties, an examination under anesthesia, cystoscopy and excision of the mass was promptly carried out. Intraoperatively, the tumour was found to be localized to the paraurethral regionand had not invaded into the urethra or bladder. It was found that the tumour was well circumscribed and was able to be ‘shelled out’ relatively easily. The patient did not require any indwelling catheter post operatively and subsequently made an uneventful recovery ( figure 2). Histopathology confirmed the mass to be a benign angiofibroblastoma.

Case 2.

A 42 year old Para 2+1,  This patient initially presented on 9.10.2008 because she noticed a mass per vaginal which had been present for 2 years, it was reducible but protruded back out immediately. The mass was firm, nodular and mobile, measuring 4x3 cm. The mass progressive got bigger and causing voiding dysfunction.  An examination under anesthesia was carried out on 226.2009. An indwelling catheter was inserted to assist in the surgery. The mass was very close to the urethra and has distorted the anatomy of the urethra. The mass was easily enucleated.  The estimated blood loss was 100mls. There were no intraoperative complications. The catheter was kept for three days. The histology was consistent with suburethral angiofibroblastoma (4x3cm).

Case 3.

This a 65 year old lady Para 6, presented with elongated and firm mass near the urethral meatus. The mass was initially small but over six year its size has increased and causing pain and difficulty in walking. She also has difficulty in micturation. She has to move the elongated mass to one side. On examination there was a 7x4x4 cm elongated mass with the tip of the distal part of the mass appeared fungating and necrosing. The proximal part of the mass had a 4cm stock/ base. She underwent examination under anaesthesia and excision of the mass. The surgery was straight forward. Check cystoscopy was normal. The histology was consistent with    Angiomyofibroblasroma.

Discussion

Angiomyofibroblastoma is a rare mesenchymal tumour of the female genital tract that was only first described in 1992(1). This tumour is predominately found in the vulval region, bu can also arise from the vagina, clitoris, labia majora and perineum. Unusual cases involving the male scrotal and inguinal regions have been reported 9(1). They have been reported in women from the age of 23-86 (mean 45.8) years. They usually appears as a painless lump that may have been present for a few weeks or up to 13 years. Clincally, this tumour can be mistaken for a bartholin gland cyst, skene’s gland cyst, urethral diverticulum or Gardner duct cyst.

Angiomyofibroblastoma is a slow growing tumour that is usually well circumscribed, and has a soft rubbery consistency with a bulging, pink, section surface. Histologically, this tumour is composed of two components: the blood vessels and stromal cells. It shows alternating hypercellular and hypicellular oedematous ares, in which numerous thin walled, small to medium sized vessels are irregularly distributed throughout. The tumour cells show immune reaction for vimentin and desmin and , more recently, it was noted to be muscle specificactin-positive or Alfa-smooth muscle actin-positive (2,3,4). It is typically benign in nature. Only one case of a malignant transformation of an angiomyofibroblastoma ( ‘angiomyofibrosarcoma’) has been reported(3).

Angiomyofibroblasroma may have been reported as an aggressive angiomyxoma. Unlike Angiomyofibroblasroma, aggressive angiomyxoma affects deeper tissues with infiltrative margins, and tends to recur (4). The pathogenesis of Angiomyofibroblasromais still unclear, although it has been proposed that it may originated from an immature mesenchymal cell in the sub epithetial myxoid zone of the lower female genital tract, or in perivascular areas. The outcome in these patients were good, and is always favorable with simple excision of the tumour mass.

References

1.      Fetchers CDM, Tsang WY, Fisher C, lee KC & Chan JK. Angiomyofibroblasromaof the vulva. A benign neoplasm distinct from aggressive angiomyxoma. Am J Surg Patho 1992; 16;373-82.

1. 2.   Hiroshi K, Noriomi M, Yoshikazu S, Masanori M, Taiji T & Takashi s. Angiomyofibroblasroma of the female urethra. Int J Urol 1999; 6:268-270

1. 3.   Nielsen GP,Young RH, Dickersin GR & Rosenberg AE. Angiomyofibroblasromaof the vulva with sarcomatous transformation (‘Angiomyofibrosarcoma)’. Am J surg Pathol 1997;30:3-10

1. 4.     Steeper TA & Rosai J. Aggressive angiomyxoma of the female pelvis and perineium. Report of nine cases of a distinctive type of gynaecologis soft-tissue neoplasma. Am J Surg Pathol 1983; 7:463-465.

1. 5.  Fukunaga M, Nomura K, Matsumoto K, Doi K, Endo Y & Ushigome S. Vulval Angiomyofibroblasroma: Clinicopathological analyisi of six cases. Am J clin Pathol 1997; 6:45-51