Journal of Clinical and Diagnostic Research. 2018 Apr, Vol-12(4): XR01-XR03 1 1 DOI: 10.7860/JCDR/2018/32311.11420 Case Series Oncology Section Abdominal Aggressive Fibromatosis: Diagnostic and Therapeutic Features Desmoid tumours, also known as aggressive fibromatosis, are rare soft tissue tumours. They represent 3.5% of fibrous tumours and less than 0.03% of all tumours. They occur sporadically or as part of FAP. They are characterised by local malignancy with a high tendency to recur [1,2]. Aim of this series was to report a 10-years experience of 16 desmoid tumours and review some facts with symptoms, investigation, and therapeutic aspects of this rare entity. CASE SERIES From the hospital case record, a total of 16 consecutive desmoid tumours (8 patients) were identified over a duration of 10 years from January 2005 to December 2015 [Table/Fig-1]. The mean age of patients was 35±14.6 years (17-58 years). The male to female ratio was 3:5. All patients underwent a CT-scan [Table/Fig-2-4] and a colonoscopy. In three cases, fibromatosis was isolated. Associated FAP was found in five patients. Polyposis was discovered by colonoscopies in two patients. In the other three patients, fibromatosis appeared after surgery for FAP with an average delay of two years after surgery. The desmoid tumours were multiple in 4 (50%) cases. A total of 10 tumours occurred in the abdominal wall [Table/ Fig-5], five in the mesentery [Table/ Fig-6,7], and one was presacral tumour. The mean diameter of the tumours was 6 cm (range, 2.3-40 cm). Surgical resection with wide surgical margins was the treatment of choice whenever possible. Excised specimens were all subjected to pathological evaluation. In two cases, treatment given was tamoxifene and/or nonsteroidal anti-inflammatory drugs. The margin of surgical resection was healthy in five patients. Soft tissue reconstruction following resection was required in two cases. No patient had surgery-related complications. During follow up (4.25 years) period, two patients returned with recurrence of tumours. Because of the nonresectable character of the tumour, they had treatment with nonsteroidal anti- inflammatory drugs and tamoxifen. HOUCINE MAGHREBI 1 , AMINE MAKNI 2 , AMINE SEBAI 3 , ASMA LABIDI 4 , FERIEL KSANTINI 5 , MOHAMED JOUINI 6 , MONTASSAR KACEM 7 , ZOUBEIR BENSAFTA 8 Keywords: Desmoid tumours, Oncology, Surgery ABSTRACT Desmoid tumours are rare soft tissue tumours developed from myofibroblasts. They usually infiltrate into adjacent muscle bundles and are characterised by the absence of metastatic potential. However, a high local aggressiveness is seen with a high rate of recurrence after surgical excision. We hereby attempt to present a series of patients with desmoid tumours and review treatment options and management strategies. Ten tumours occurred in the abdominal wall, five in the mesentery, and one was presacral tumour. The mean diameter of the tumour was 6 cm (range, 2.3-40 cm). Associated Familial Adenomatous Polyposis (FAP) was found in five patients. The margin of surgical resection was healthy in five patients. During follow up period, two patients returned with recurrence of tumours. Case Age (years) Gen- der Number of tu- mours Tumour site Tumour size (cm) FAP Treat- ment Re- cur- rence 1 42 M 5 Abdominal wall (4) 5 + Tamoxifen Stable Mesenteric (1) 3 2 17 F 2 Abdominal wall 8 + NSAID+ Tamoxifen Stable Mesenteric 6 3 39 M 1 Mesenteric 5 + Surgery - 4 58 M 1 Presacal 2.3 + Tamoxifen Stable 5 30 F 1 Abdominal wall 5 - Surgery - 6 25 F 1 Mesenteric 7 - Surgery - 7 53 F 2 Abdominal wall 6 - Surgery + (4 years) Abdominal wall 5 8 23 F 3 Abdominal wall 5 + Surgery + (2 years) Abdominal wall 7 Mesenteric 40 [Table/Fig-1]: Demographic data of treated patients. F: Female; M: Male; FAP: Familial adenomatous polyposis; NSAID: Nonsteroidal anti-inflammatory drugs [Table/Fig-2]: Axial CT-scan showing abdominal desmoid tumour.