Desmoid-type fibromatoses are neoplasms of fibroblastic origin, occurring sporadically or associated with familial adenomatous polyposis (FAP) coli. By comparing sporadic and FAP-associated desmoid-type fibromatoses, we tried to identify clinical characteristics, which may indicate FAP. Histopathology data of all Dutch patients with desmoid-type fibromatoses diagnosed between 1999 and 2009 were retrieved from PALGA, the nation-wide network and registry of histopathology in the Netherlands. For calculation of incidence rates, person-years from the general matched population were used. Based on polyp counts in pathological records, the cohort was divided into a FAP group and a non-FAP group. Patient-and tumor characteristics were compared between the two groups. A total number of 519 patients older than 10 years with a confirmed diagnosis of desmoid-type fibromatoses were included. Thirty-nine (7.5%) desmoid patients were documented of having FAP. The incidences of sporadic and FAP-related desmoid-type fibromatoses were 3.42 and 2,784 per million person-years, respectively. The majority of FAP patients developed desmoid-type fibromatoses after the diagnosis of FAP. Having FAP was associated with male gender [odds ratio (OR) 2.0, p 5 0.034], desmoid diagnosis at an earlier age (mean 36 vs. 42 years, p 5 0.031), and desmoid localization intra-abdominally (OR 18.9, p 0.001) or in the abdominal wall (OR 4.8, p 0.001), compared to extra-abdominal desmoid localization. In conclusion, patients with desmoid-type fibromatoses are at risk of underlying FAP. Especially cases with desmoid localization intra-abdominal or in the abdominal wall, and all patients younger than 60 years, have a substantial increased risk and should be referred for colonoscopy.Desmoid-type fibromatoses, previously known as aggressive fibromatoses, musculoaponeurotic fibromatoses or desmoid tumors, is a rare neoplastic disorder with an unpredictable disease course. Histologically, these neoplasms are benign fibroblastic tumors consisting of spindle cells and fibroblasts with a low mitotic rate. Differentiation from other soft tissue tumors, for example low grade fibromyxoid sarcomas, can be difficult. 1 Clinically, desmoid-type fibromatoses either presents as a solid tumor, located in muscles, or as diffuse fibromatoses in the mesenterium. Despite the histological bland morphology, desmoid-type fibromatoses show locally aggressive features such as infiltration in surrounding tissue, and a tendency to recur after resection. Desmoids lack metastatic potential. The etiology of desmoid-type fibromatoses is thought to be multifactorial. 1 Supposed risk factors are exposure to estrogens, trauma (associated with surgery or pregnancy), and a positive family history of desmoid-type fibromatoses. [2][3][4][5] Desmoid-type fibromatoses are rare in the general population with estimated incidence rates of two to four per million individuals per year. 6 However, in patients with familial adenomatous polyposis (FAP), desmoid-type fibromatoses are reported in 10-30%...
Intragastric balloons are associated with (1) early period intolerance, (2) diminished effect within 3–4 months, and (3) bowel obstruction risk mandating removal at 6 months. The introduction of an adjustable balloon could improve comfort and offer greater efficacy. A migration prevention function, safely enabling prolonged implantation, could improve efficacy and weight maintenance post-extraction. The first implantations of an adjustable balloon with an attached migration prevention anchor are reported. The primary endpoint was the absence of bowel perforation, obstruction, or hemorrhage. Eighteen patients with mean BMI of 37.3 were implanted with the Spatz Adjustable Balloon system (ABS) for 12 months. Balloon volumes were adjusted for intolerance or weight loss plateau. Mean weight loss at 24 weeks was 15.6 kg with 26.4% EWL (percent of excess weight loss) and 24.4 kg with 48.8% EWL at 52 weeks. Sixteen adjustments were successfully performed. Six downward adjustments alleviated intolerance, yielding additional mean weight loss of 4.6 kg. Ten upward adjustments for weight loss plateau yielded a mean additional weight loss of 7 kg. Seven balloons were removed prematurely. Complications necessitating early removal included valve malfunction (1), gastritis (1), Mallory–Weiss tear (1), NSAID (2× dose/2 weeks) perforating ulcer (1), and balloon deflation (1). Two incidents of catheter shear from the chain: one passed uneventfully and one caused an esophageal laceration without perforation during extraction. The Spatz ABS has been successfully implanted in 18 patients. (1) Upward adjustments yielded additional weight loss. (2) Downward adjustments alleviated intolerance, with continued weight loss. (3) Preliminary 1-year implantation results are encouraging.
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