A longstanding deep-seated nuchal tumor

ArticleinInternational journal of dermatology 51(8):910-2 · August 2012with2 Reads
DOI: 10.1111/j.1365-4632.2011.05364.x · Source: PubMed
  • [Show abstract] [Hide abstract] ABSTRACT: Desmoid tumors are associated with a variable and unpredictable clinical course. Surgery is the therapeutic mainstay, but there has been much discussion of late regarding its proper application. Little is known regarding the molecular determinates of desmoid tumor behavior. Some recent work has focused on the role of beta-catenin in desmoid tumor biology. Given the variable clinical course of desmoid tumors, the interpretation of factors classically associated with recurrence such as microscopic status of margins appears more nuanced that previously thought. The application of multidisciplinary assessment with multimodality treatment, including surgery, radiation and systemic therapies may underlie these changes and now form the basis of care for this tumor. The precise CTNNB1 mutation present appears to be strongly predictive of recurrence after initial resection in one large, retrospective, multivariate analysis. Establishing the population benefiting most from various treatment modalities and combinations is critical for progress in this disease. Assessment and treatment of individual patients in a multidisciplinary setting is critical to achieve the most favorable outcome. Additional study of the molecular determinates of desmoid behavior is needed to guide therapeutic selection.
    Article · Jun 2009
  • [Show abstract] [Hide abstract] ABSTRACT: Previous reports even large studies discussing the prognosis of desmoids have included tumors from intra- and extra-abdominal sites as well as incomplete resection. The purpose of this study was to explore prognostic factors associated with the recurrence free survival (RFS) rate in surgically treated extra-abdominal and abdominal wall desmoids. A total of 198 consecutive desmoid patients were treated with surgery over a 20-year period at a single institution. Of these, 151 patients with extra-abdominal and abdominal wall tumors were retrospectively reviewed. One hundred thirteen patients were referred for the primary tumor and the other 38 for recurrent disease initially treated elsewhere. All patients underwent a macroscopically complete resection. The median follow-up interval was 102 months. Thirty-one patients (20.5%) had a local recurrence (LR). No patients died of the disease. The 5- and 10-year RFS was 79.7% and 78.5%, respectively. Admission status, gender, tumor size, margin status, location, and number, were predictors of LR in univariate analysis. Tumor size and margin status were independent prognostic factors in multivariate analysis. Positive margins were predictive of recurrence of primary disease, and also showed a trend for recurrent disease, which was not statistically significant. The selective use of adjuvant radiation did not show significant benefit over local control. Regardless of primary or recurrent disease, microscopically negative margins should always be the goal for extra-abdominal desmoids surgery, if no cosmetic defects or function demolition is encountered. Extra-abdominal desmoids deserve more attention and should be treated more aggressively, especially when leaving positive margins.
    Article · Dec 2009
  • [Show abstract] [Hide abstract] ABSTRACT: Desmoid tumour (DT) is an uncommon locally invasive non-metastasizing neoplastic lesion. The aetiology of this tumour is unknown and its treatment is controversial. Twelve cases of DT are presented and the literature is reviewed. Twelve cases of DT treated at our institution during a 3.5-year period are analysed and the literature reviewed. Ten patients were referred with a primary tumour, one with local recurrence and one patient with a second primary desmoid tumour. One patient had multiple mesenteric DT (familial adenomatous polyposis coli-FAP), and in the remaining 11 patients the tumour was located in the abdominal wall in four, at an extremity in three, in the upper back in two patients, in the pelvis in one and retroperitoneally in one. The largest mesenteric DT was marginally excised en bloc with total jejunectomy. In the remaining 11 DT, complete excision to microscopically tumour-free margins was possible in nine cases and to microscopically involved margins in two cases. At a mean follow-up of 22 months (range 7-38 months), one patient was alive with stable disease (Gardner's syndrome), 10 patients were alive and free of recurrence and one patient (9%) developed local recurrence which was re-excised-she is disease-free 10 months later. Complete excision is the main modality of treatment for primary and recurrent DT. This is feasible in most cases except for tumours involving the base of the bowel mesentery. Surgical resection alone achieved local control of the tumour in most of the patients in this series (92%).
    Article · Nov 1999
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