Fibromatosis (desmoid tumor) in the urogenital system

Abstract

Fibromatosis refers to a group of rare tumors characterized by benign proliferation of fibroblasts [1]. The unique feature of fibromatosis is the often poorly circumscribed nature of the growth and infiltration of surrounding organs, often leading to incomplete removal. This report describes two such cases of pelvic fibromatosis. A 16-year-old girl presented with a history of marked lower urinary tract symptoms of 1-year duration, which culminated in acute urinary retention. A general physical examination was unremarkable. Abdominal examination revealed vague fullness in the suprapubic region. Digital rectal examination revealed a hard mass in the right paravaginal area, which was also visible in the right gluteal region. Her routine biochemical and hematological tests were normal. Ultrasound of the abdomen revealed a pelvic mass. Magnetic resonance imaging (MRI) showed a large mass occupying the pelvis (Fig. 1); the plane between the mass and the lateral pelvic wall was ill defined. Extension of the mass was seen in the right gluteal region through the sciatic foramen and right ischiorectal fossa. The mass was compressing the rectum and displacing the uterus anteriorly. The urinary bladder was also compressed. Fine-needle aspiration cytology of the mass suggested a spindle cell tumor, and debulking surgery was planned. During surgery the mass was found to occupy the whole pelvic region and removal was done piece by piece, leading to near total excision of the mass. A 40-year-old woman presented with a history of lower urinary tract symptoms and dyspareunia. Routine tests were ⁎ Corresponding author. Tel.: +91 172 2756321 (O); fax: +91 172 2744401. E-mail address: santoshsp1967jaimatadi@yahoo.co.in (S. Kumar).