Desmoid tumour in familial adenomatous polyposis patients: responses to treatments.

We aimed to assess the results of the different treatments used for DT in Familial Adenomatous Polyposis (FAP) patients. All patients followed for FAP who developed a DT between 1970 and 2010 were collated. Overall, 80 treatment lines were administered to patients with a progression free or regression rate of 43% (34/80). Response rates were: chemotherapy 77% (10/13); Sulindac+tamoxifen 50% (6/12); Tamoxifen 40% (6/15); Imatinib 36% (4/11); Sulindac 28% (8/29). Among the 42 surgical procedures, an R0 resection was performed in 26 (62%) allowing the absence of recurrence for 54%. After a median follow-up of 81months, 8 patients died of their DT and 6 died of other cause. Overall and DT-specific survival at 20 years were 52 and 79%, respectively. Chemotherapy was the most efficient treatment. For intra-abdominal DT, we consider it as a first choice treatment and reserve surgery when it is impossible or when DT are life threatening.

Peritoneal disease can be caused by a wide spectrum of pathologies. This article begins with an overview of peritoneal embryology and anatomy followed by a detailed description of the multimodality imaging appearance of peritoneal diseases. The uncommon diseases which cause peritoneal disease include desmoid fibromatosis, desmoplastic small round cell tumor, malignant mesothelioma, well-differentiated mesothelioma, multicystic mesothelioma, papillary serous carcinoma, leiomyomatosis, extramedullary hematopoiesis, inflammatory pseudotumor and amyloidosis. This manuscript will help the radiologist become familiar with the different peritoneal spaces, pathways of spread, multimodality imaging appearance and differential diagnoses of peritoneal diseases in order to report the essential information for surgeons and oncologists to plan treatment.

Desmoid tumors can have atypical presentation making clinical correlation challenging to unsuspecting urologists. Only a few cases have been reported in the urology literature. In this report, we present a case of desmoid tumor presenting with gross hematuria.

We perform an abdominal wall desmoid resection using ultrasound guide. This technique ensures to spare a wide wall area and to obtain a multilayer reconstruction minimizing postoperative risk. This approach allows good oncological results and better managing abdominal wall post-resection defect.

The optimal management of desmoid fibromatosis remains unclear, leading to significant variability in patient management. To assess this problem, the current approach of clinicians managing this complex condition in the UK was investigated. A hypothetical case of intramuscular limb girdle desmoid fibromatosis in a fit 65-year-old patient was devised. Surgical and non-surgical oncology members of the British Sarcoma Group were questioned on how they would manage this case in three scenarios: primary disease with function-sparing surgery possible, primary disease with neurovascular involvement and disease recurrence after a previous R0 resection. Initial management, management of symptomatic disease progression, follow-up preferences and any differences in respondents’ management choices in a younger case were investigated. The responses from 14 sarcoma surgeons and 23 oncologists (14 clinical, nine medical) were analysed. Desmoid fibromatosis management is generally shared by surgeons and oncologists within sarcoma multidisciplinary teams in the UK. Variation exists in the chosen initial management of primarydesmoid fibromatosis in the UK, with function-sparing surgery possible (observation 51%, resection 51%), primary desmoid fibromatosis with neurovascular involvement (hormone therapy with non-steroidal anti-inflammatory drugs 51%, radiotherapy 27%, observation 22%) and for cases of desmoid fibromatosisrecurrence (radiotherapy 41%, hormone therapy and non-steroidal anti-inflammatory drugs 27%, observation 24%). There was a clear preference of surgical resection of symptomatic disease progression in cases of primary desmoid fibromatosis without neurovascular involvement (60%). By contrast, radiotherapy was the preferred treatment for progression in cases with neurovascular involvement (47%) or cases of recurrence after a previous R0 resection (34%). Clinical follow-up was selected 3 months after intervention in 68% of scenarios. Follow-up imaging was selected 3 or 6 months after intervention in 57% and 21% of cases, respectively. Most respondents would not change their chosen management in younger patients. Several groups have issued formal guidelines for clinicians managing desmoid fibromatosis, including the British Sarcoma Group, the National Comprehensive Cancer Network and the European Society for Medical Oncology. However, these are in some ways contradictory and may not reflect recent publications, potentially explaining the significant variation in the management of desmoid fibromatosis in the UK shown by this survey. We propose a review of current evidence; a national consensus or a desmoid fibromatosisregistry may help to standardise desmoid fibromatosis care.

We present the case of a 34-year-old woman that developed a painless mass in the right popliteal fossa during pregnancy after intracytoplasmic sperm injection and hormonal therapy. The open biopsy was done one week after delivery, and the histology showed a desmoid tumor. We performed the resection one week later and found the common peroneal nerve completely surrounded by the tumor. The close resection due to the neurolysis was the reason why an adjuvant radiation with 56 Gy was done. The last clinical examination, 18 month later, did not show any signs of recurrence and an excellent functional outcome. This case demonstrates the possible influences of pregnancy and hormonal therapy on the evolution of desmoid tumors.