Management of desmoid tumours: A large national database of familial adenomatous patients shows a link to colectomy modalities and low efficacy of medical treatments

Our aims were to study the factors associated with the development of desmoid tumours in familial adenomatous polyposis patients, and to describe presentation and management of desmoid tumours. We reviewed all patients with familial adenomatous polyposis followed at our institution between 1965-2013, with either identified adenomatous polyposis coli gene mutation, or a personal and family history suggesting adenomatous polyposis coli-related polyposis. The only factor significantly associated with occurrence of desmoid tumours was the type of surgery: 12 (12%) desmoid tumours in 104 patients treated by colectomy, versus 19 (25%) desmoid tumours in 76 patients treated by proctocolectomy, p_=_0.027. If confirmed, colectomy (versus proctocolectomy) should be performed in adenomatous polyposis coli-related familial adenomatous polyposis patients to avoid desmoid tumours. We show that there is a high prevalence of post-surgical recurrence and the low efficacy of available medical treatments for desmoid tumours.

This study aimed to evaluate the radiologic and symptomatic course of the disease in patients initially managed with active surveillance. Patients with a primary desmoid tumor at any anatomic location diagnosed between 1998 and 2016 were identified in a prospectively maintained database from a single sarcoma reference center in the United Kingdom. The study identified 168 patients with a primary desmoid tumor initially managed with active surveillance. The tumors were located in the abdominal wall (n?=?61, 36%), an extremity (n?=?51, 30%), chest wall (n?=?30, 18%), intra-abdominal site (n?=?15, 9%), or elsewhere (n?=?11, 6%). Of all the patients, 36% experienced radiologic progressive disease, 36% had stable disease, and 27% regressed. The patients younger than 50 years were more likely to progress (p?=?0.046), whereas the patients with chest wall or upper-extremity tumors reported significantly more pain (p?=?0.01). Eventually, 46% of the patients proceeded to treatment. The median time to start of treatment after initial surveillance was 31 months, whereas the median follow-up time for the patients not receiving any treatment was 40.5 months. The indications for initiation of treatment were pain (32%), progression (31%), or both (13%). Patients with desmoid tumors can be managed with initial active surveillance, although almost half of patients may eventually need treatment. Pain, tumor progression, or both are the most common indications for the initiation of treatment.

The present retrospective analysis aimed to determine prognostic factors leading to recurrence. Between 2000 and 2014, 114 patients with aggressive fibromatosis were treated surgically at BG-University Hospital Bergmannsheil (Bochum, Germany). The rates of recurrence-free survival (RFS) after 5 years were 68.8% [95% confidence interval (CI), 53.5-79.9%] in patients with R0-resected primary tumors and 34.1% (95% CI, 19.9-48.9%) in patients with R1/R2-status (P=0.001). The current results suggest that the attainment of microscopically negative surgical margins at the initial surgical treatment is associated with a significantly improved prognosis.

Desmoid type fibromatosis (DTF) is a rare, locally invasive, non-metastasizing soft tissue tumor. We report an interesting case of DTF involving the pancreatic head of a 54-year-old woman. She presented with intermittent dysphagia and significant weight loss within a 3-mo period. Laboratory findings showed mild elevation of transaminases, significant elevation of alkaline phosphatase and direct hyperbilirubinemia, indicating obstructive jaundice. Computerized tomography of the abdomen revealed a mass in the head of the pancreas, dilated common bile duct, and dilated pancreatic duct. Endoscopic retrograde cholangiopancreatography and endoscopic ultrasound showed a large hypoechoic mass in the head of the pancreas causing extrahepatic biliary obstruction and pancreatic ductal dilation. The patient underwent a successful partial pancreatico-duodenectomy and cholecystectomy. She received no additional therapy after surgery, and liver function tests were normalized within nine days after surgery. Currently, surgical resection is the recommended first line treatment. The patient will be followed for any recurrence.

We report on a case of multicentric fibromatosis originating from the retroperitoneal space and submandibular triangle, in an 18-year-old lady. Computed tomography revealed a retroperitoneal abdominopelvic tumor extending from the left sub-diaphragmatic space to the pelvic inlet which had enveloped the solid viscera in the left upper quadrant with a displaced celiac axis. She also had a recurrent resectable fibromatosis in left submandibular gland. Histopathological evaluation revealed fibromatosis. Preoperatively, Vinblastin-, Methotrexate-, Tamoxifen-based systemic chemotherapy was offered for 12 weeks in an attempt to downsize the mass. An en bloc complete resection of tumor with multi-visceral resection was performed to achieve negative margins and since then, the patient has remained asymptomatic without any signs of tumor recurrence during the 12th month follow-up visit. Complete resection with negative margins is the treatment of choice and majority of the lesions are amenable for surgical resections. Adjuvant therapy using non-steroidal anti-inflammatory agents, tamoxifen, interferon, anti-neoplastic agents, and radiotherapy, either alone or in combination finds application for un-resectable or recurrent cases.

Desmoid tumor (DT) is a rare and locally invasive proliferative disease. Although, the absence of metastatic potential, it has the propensity for locally invasive growth and recur. Chemotherapy may be considered in inoperable and/or recurrent disease. Patients with histological diagnosis of DT and treated with weekly low-dose chemotherapy with vinblastine and methotrexate between January 1998 and December 2015 were identified and their medical records were analyzed. Of 23 patients analyzed, most of them were women (female-to-male ratio 2.8:1). The median age at presentation was 29 years (range, 18-59 years). Tumors location was: thoracoabdominal wall (n: 11, 47.8%), extremities (n: 7, 30.4%), abdominal cavity (n: 1; 4%), and head and neck (n: 4, 17.3%). Tumor sizes were documented in 18 cases and ranged from 3 to 20_cm…