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      En-bloc resection of a giant retroperitoneal lipoma: a case report and review of the literature

      case-report

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          Abstract

          Background

          Retroperitoneal lipomas are an extremely rare condition with only 17 cases described in the literature since 1980. They can reach enormous size and cause significant abdominal symptoms. The most important differential diagnosis is the well-differentiated liposarcoma, which preoperatively often may not definitely be ruled out.

          Case presentation

          We present the case of a 73 year-old Caucasian patient with a giant retroperitoneal lipoma of 9 kg measuring 55 cm in diameter. The patient presented with abdominal pain and swelling that had been slowly progressive for the last 15 years. On computerized tomography an immense retroperitoneal tumor was revealed. Intraoperatively, the tumor did not show any signs of infiltrative growth, therefore sole tumor extirpation was performed.

          Conclusion

          Retroperitoneal lipomas are not clearly distinguishable from well-differentiated liposarcomas on imaging and even biopsies may be misleading. Moreover, abdominal symptoms, i.e. pain, obstipation and dysphagia may occur due to mechanical displacement. Therefore, surgical exploration with complete oncological resection is the therapy of choice if malignity cannot be ruled out.

          Electronic supplementary material

          The online version of this article (doi:10.1186/s13104-015-1038-7) contains supplementary material, which is available to authorized users.

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          Most cited references27

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          Lipomatous tumors.

          Lipomatous tumors are a common group of mesenchymal lesions. Over the years the major changes in the classification of lipomatous tumors have included the addition of several new variants of lipoma, the use of the term atypical lipoma for well differentiated liposarcoma of the subcutaneous tissue, and recognition of the entity dedifferentiated liposarcoma. Lipomas, the most common lipomatous tumor, account for nearly one-half of all benign lesions. In their typical form they seldom present diagnostic problems for the pathologist. However, lipomas occurring in deep locations (e.g., intramuscular lipoma, perineural lipoma) or those having unusual features (e.g., chondroid lipoma, lipoma with hibernoma, cellular angiolipoma, spindle cell/pleomorphic lipoma) may be confused with liposarcoma. Recent cytogenetic studies have reaffirmed the separate nature of many of the variants of lipoma. Solitary lipomas commonly have rearrangements of chromosome 12, a finding not encountered in multiple lipomas or in spindle cell/ pleomorphic lipoma. Liposarcoma is the most common adult soft tissue sarcoma. It seldom arises from subcutaneous tissues or from preexisting lipomas, and is seldom found in children. The hallmark of liposarcoma is the immature fat cell or lipoblast. Diagnostic lipoblasts have an eccentric, hyperchromatic nucleus, which is indented or scalloped by the presence of one or more fat vacuoles. It is important that these cells occur in the appropriate histologic background because similar cells can be seen in a variety of nonlipomatous lesions (e.g., silicone reaction). Failure to apply strict criteria in identifying such cells and in noting the milieu in which they occur can lead to overdiagnosis of liposarcoma. Liposarcomas are divided into several subtypes: well differentiated, myxoid, round cell, pleomorphic, and dedifferentiated. Liposarcomas can be conceptualized as occurring in two broad groups, myxoid/round cell liposarcoma and well differentiated/dedifferentiated liposarcoma. Myxoid/round cell liposarcomas occur in middle-aged adults primarily as an extremity lesion. Tumors range from pure myxoid (low grade) to pure round cell (high-grade lesions) with some cases having transitional features. Behavior can be related to the amount of round cell areas. A consistent chromosome abnormality t(12;16) characterizes this spectrum of lesions. Well differentiated/dedifferentiated liposarcomas, in contrast, occur in late adult life as extremity or retroperitoneal lesions. They consist of mature fat interlaced with atypical hyperchromatic cells and rare lipoblasts. These lesions recur commonly, but they do not metastasize. Their behavior is strongly influenced by location, with retroperitoneal lesions having the worse prognosis. As a long-term complication of the disease, these lesions may progress histologically (dedifferentiate), a phenomenon that confers upon them metastatic potential. Dedifferentiation is largely a time-dependent phenomenon that occurs in sites in which there is a high likelihood for clinical persistence of disease (e.g., the retroperitoneum). Dedifferentiated liposarcomas occur in an age group similar to well differentiated liposarcoma, but are found far more commonly in the retroperitoneum. Most occur as de novo lesions, with only a small percentage occurring as a late complication of well differentiated liposarcoma, as described above. They consist of well differentiated areas associated with nonlipogenic sarcoma usually resembling high-grade malignant fibrous histiocytoma or fibrosarcoma. However, the range of histologic features occurring in the dedifferentiated areas is more varied than previously appreciated. Low-grade areas resembling fibromatosis or low-grade fibrosarcoma may be seen as the sole expression of dedifferentiation or may co-exist with high-grade sarcoma. (ABSTRACT TRUNCATED)
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            Soft tissue sarcomas: ESMO clinical recommendations for diagnosis, treatment and follow-up.

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              Cellular and molecular features of lipoma tissue: comparison with normal adipose tissue.

              Involvement of adipose-derived stem/progenitor/stromal cells (ASCs) in the development of lipomas has been suggested, but the pathogenesis and pathophysiology of this tumour remain unclear.
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                Author and article information

                Contributors
                Maximilian.Weniger@med.uni-muenchen.de
                Jan.DHaese@med.uni-muenchen.de
                Wolfgang.Kunz@med.uni-muenchen.de
                Sebastian.Pratschke@med.uni-muenchen.de
                Markus.Guba@med.uni-muenchen.de
                Jens.Werner@med.uni-muenchen.de
                Martin.Angele@med.uni-muenchen.de
                Journal
                BMC Res Notes
                BMC Res Notes
                BMC Research Notes
                BioMed Central (London )
                1756-0500
                10 March 2015
                10 March 2015
                2015
                : 8
                : 75
                Affiliations
                [ ]Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Campus Grosshadern, Ludwig Maximilians-University, Marchioninistraße 15, D-81377 Munich, Germany
                [ ]Institute for Clinical Radiology, Campus Grosshadern, Ludwig Maximilians-University, Munich, Germany
                Article
                1038
                10.1186/s13104-015-1038-7
                4365774
                25777239
                8e166bf9-a38d-497d-ac73-fc8414cce018
                © Weniger et al.; licensee BioMed Central. 2015

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 14 October 2014
                : 27 February 2015
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2015

                Medicine
                well-differentiated liposarcoma,en-bloc resection,tumor debulking
                Medicine
                well-differentiated liposarcoma, en-bloc resection, tumor debulking

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