Retroperitoneal desmoid tumor with unusual appearance as a spontaneous ureteral rupture and rare association with desmoid tumor of the breast

Faouzi Mallat,Faouzi Mosbah

doi:10.5348/ijcri-201470-cr-10381

Abstract

Introduction: Desmoid tumors of retroperitoneum and breast are rare. their association is exceptional and their management is controversial. spontaneous ureteral rupture as a complication and a circumstance of discovery of the retroperitoneal desmoid tumor is a rare entity and poses management difficulties. to the best of our knowledge, this case is the first to present this exceptional association, and to report the ureteral rupture as a circumstance of discovery of the retroperitoneal desmoid tumor. case report: We present the first reported case, seen in a young girl without Gardner’s syndrome, in which upper ureteral rupture was the primary symptom of an aggressive retroperitoneal desmoid tumor. she underwent laparotomy for septic peritonitis secondary to rupture of the right ureter, in which initially right ureterocutaneostomy was done, later right nephrectomy was made; for recurrent right ureteral rupture. Imatinib (Glivec) 200 mg was prescribed. However, due to intractable diarrhea, the target therapy was discontinued. At follow-up, left breast desmoid tumor was diagnosed by mammography, sonography and magnetic resonance imaging scan; and confirmed by sonographically guided core biopsy; and large excision was made. At the last follow-up, radiological exams did not show any

Highlights

Desmoid tumors of retroperitoneum and breast are rare
Case Report: We present the first reported case, seen in a young girl without Gardner’s syndrome, in which upper ureteral rupture was the primary symptom of an aggressive retroperitoneal desmoid tumor
At follow-up, left breast desmoid tumor was diagnosed by mammography, sonography and magnetic resonance imaging scan; and confirmed by sonographically guided core biopsy; and large excision was made

Summary

Introduction

Desmoid tumors are benign myofibroblastic neoplasms originating from the muscle aponeurosis and classified as deep fibromatoses [1]. An abdominal computed tomography (CT) scan (Figure 1) was performed which revealed large, hypodense and heterogeneously enhancing mass (138×98×112 mm) with an exophytic component arising from retroperitoneal space, extending up to the mesenteric vessels and encased them, compressing the right kidney, and abutting the anterior abdominal wall. Both ureters were encased in the infiltrative tumor, with extravasation of radiocontrast from the right upper ureter at the L3 to L4 transverse process level leading to a 20-cm huge retroperitoneal urinoma over the right retroperitoneal space, extending from the perirenal space along the psoas muscle to the pelvic and peritoneal cavity. The patient changes the double-J catheter of the left ureter every six months and still refuses any medical or surgical treatment

DISCUSSION

CONCLUSION