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      Multifocal bilateral desmoid tumour of perirenal tissues with peribiliary localization

      case-report
      Author(s): , MD, , MD, , MD, , MD, , MD
      Publication date (Electronic):
      Journal: BJR | case reports
      Publisher: The British Institute of Radiology

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          Abstract

          Desmoid tumour (DT) is an unusual, benign tumour, more frequently observed in patients with familial polyposis and pregnant females. It usually presents as a single mass lesion, more frequently showing a compressive rather than an infiltrative growth pattern. We report a case of a 70-year-old male presenting with a multifocal, bilateral infiltrative DT of the perirenal tissue, with involvement of the choledochus wall. The patient was partly treated with tamoxifen and docetaxel, but both therapies were discontinued in accordance with the patient’s decision owing to mild toxicity; however, a CT examination performed 3 months later showed an unexpected remarkable reduction of the tumour at all sites. At 1 year follow-up, new pathologic tissue was visible surrounding the right renal pelvis and the calices.

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          Desmoid tumors: clinical features and treatment options for advanced disease.

          Peter Hohenberger, Bernd Kasper, Philipp Ströbel (2010)
          Desmoid tumors describe a rare monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Although histologically benign, desmoids are locally invasive and associated with a high local recurrence rate, but lack metastatic potential. On the molecular level, desmoids are characterized by mutations in the β-catenin gene, CTNNB1, or the adenomatous polyposis coli gene, APC. Proof of a CTNNB1 mutation may be useful when the pathological differential diagnosis is difficult and location might be predictive for disease recurrence. Many issues regarding the optimal treatment of patients with desmoids remain controversial; however, surgery is the therapeutic mainstay, except if mutilating and associated with considerable function loss. Postoperative radiotherapy reduces the local recurrence rate, in cases of involved surgical margins. Because of the heterogeneity of the biological behavior of desmoids, including long periods of stable disease or even spontaneous regression, treatment needs to be individualized to optimize local tumor control and preserve patients' quality of life. Therefore, the application of a multidisciplinary assessment with multimodality treatment forms the basis of care for these patients. Watchful waiting may be the most appropriate management in selected asymptomatic patients. Patients with desmoids located at the mesentery or in the head and neck region could present with life-threatening complications and often need more aggressive treatment. This review describes treatment options and management strategies for patients with desmoid tumors with a focus on advanced disease.
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            High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors.

            Andreas Unger, C Adolph, Gabriela Moeslein … (2004)
            Desmoid tumors are mesenchymal nonmetastasizing neoplasms. Although rare in the general population, they are a common extracolonic manifestation of familial adenomatous polyposis (FAP). Because of high tumor recurrence rates, surgery has been less than satisfactory in the treatment of desmoid tumors. In the current study, high doses of tamoxifen in combination with sulindac were used to treat severe desmoid tumors to avoid surgery. Since 1992, 25 patients at Heinrich Heine University (Dusseldorf, Germany) were treated with a combination of tamoxifen and sulindac. In the current study, 17 patients with FAP-associated and 8 patients with sporadic desmoid tumors received 120 mg of tamoxifen and 300 mg of sulindac daily. Every 6 months, the protracted course of desmoid growth was measured by computed tomography and/or magnetic resonance imaging scans. Tumor responses were characterized as progressive disease, stable disease (SD), partial regression (PR), and complete regression (CR). Of the group of patients who received tamoxifen and sulindac as a primary treatment, all three patients with sporadic desmoid tumors demonstrated cessation of growth, and 10 of the 13 patients with FAP-associated tumors achieved either a PR or CR. In the sporadic desmoid tumor group, eight of nine patients developed tumor recurrences after undergoing surgery at other institutions. Of these, two patients had SD and two patients had a PR to CR. The patients with desmoid tumors who were managed conservatively with high-dose tamoxifen and sulindac had the best outcome. Desmoid tumor recurrence after surgery was high and in the FAP-associated tumor group, therapy with tamoxifen and sulindac was found to be less successful. Based on this experience, the authors recommended high-dose tamoxifen and sulindac as the primary treatment for patients with FAP-associated desmoid tumors. However, to our knowledge, the best approach after surgical intervention for patients with sporadic desmoid tumors remains to be determined. Copyright 2003 American Cancer Society.
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              Abdominal desmoid tumors.

              G H Sakorafas, George Peros, Christos Nissotakis (2007)
              Desmoid tumors are rare, benign, fibromatous lesions that are the result of abnormal proliferation of myofibroblasts. Desmoid tumors can be classified as extra-abdominal and abdominal. Abdominal desmoid tumors are either superficial or intraabdominal. These tumors are associated with a high recurrence rates, even if their microscopic characters indicate a benign disease; their biologic behavior often indicates rather a "malignant" disease, which can cause even the death. Intraabdominal desmoid tumors can engulf surrounding viscera and vessels, thereby greatly complicating their surgical treatment. Management is multidisciplinary. Simple observation is a reasonable management option for asymptomatic patients; spontaneous regression of these tumors may be observed. Complete excision is the treatment of choice for tumors causing symptoms or complications. Surgery should be minimized as much as feasible, while at the same time achieving free margins. Adjuvant therapy should be considered in selected cases; the role of other management options (including gene transfer therapy) is currently under intensive investigation.
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                Author and article information

                Contributors
                Beatrice Sacconi
                Renato Argirò
                Angelo Iannarelli
                Alessandro Di Gaeta
                Mario Bezzi
                Journal
                Journal ID (nlm-ta): BJR Case Rep
                Journal ID (iso-abbrev): British Institute of Radiology
                Journal ID (publisher-id): bjrcr
                Title: BJR | case reports
                Publisher: The British Institute of Radiology
                ISSN (Electronic): 2055-7159
                Publication date Collection: 2016
                Publication date (Electronic): 16 December 2015
                Volume: 2
                Issue: 2
                Electronic Location Identifier: 20150099
                Affiliations
                [1] Department of Radiological, Oncological and Anatomopathological Sciences , Sapienza University of Rome, Rome, Italy
                Author notes
                Address correspondence to: Dr Renato Argirò E-mail: renato.argiro@ 123456gmail.com
                Article
                Publisher-manuscript ID: bjrcr.20150099
                DOI: 10.1259/bjrcr.20150099
                PMC ID: 6180855
                SO-VID: fa4eac97-162c-4338-af95-646cfaf00d06
                Copyright © © 2015 The Authors. Published by the British Institute of Radiology
                License:

                This is an open access article under the terms of the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 09 March 2015
                Date revision received : 09 November 2015
                Date revision received : 02 September 2015
                Date accepted : 16 November 2015
                Categories
                Subject: Case Report
                Compound subject: bjrcr, BJR|case reports
                Compound subject: gas-abd, Gastrointestinal and abdominal
                Compound subject: gen-trct, Genitourinary tract
                Compound subject: ct, CT

                Data availability:

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