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      A rare case of a giant retroperitoneal lipoma with multiple limb and trunk lipomata without familial multiple lipomatosis

      case-report

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          Abstract

          Retroperitoneal lipoma is exceedingly rare, and due to the difficulty in distinguishing between retroperitoneal lipoma and well-differentiated liposarcoma (WDLS), recommendation is en-bloc resection. A 58-year-old male was investigated for scrotal swelling, ultrasound and computed tomography showed a well-defined lipomatous mass occupying much of the left side of the lower abdomen. At laparotomy, a large left-sided retroperitoneal mass was found. Histology reported a 160 mm × 150 mm × 90 mm fatty tumour weighing 1540 g. MDM2 gene amplification was not present on fluorescence in situ hybridization. No significant somatic signatures were identified on whole exome sequencing. Retroperitoneal fatty tumours represent a diagnostic dilemma. Sampling via core biopsy has been recorded at 85% accuracy for WDLS. Positive amplification of the MDM2 gene supports a diagnosis of WDLS; however, a negative biopsy does not exclude the diagnosis due to varied amplification among different cells in the same tumour.

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          Most cited references11

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          Management of primary retroperitoneal sarcoma (RPS) in the adult: a consensus approach from the Trans-Atlantic RPS Working Group.

          (2014)
          Retroperitoneal soft tissue sarcomas (RPS) are rare tumors that include several well-defined histologic subtypes. Although surgery is the mainstay of curative therapy, no universally accepted recommendations concerning the best management have been developed to date. Optimization of the initial approach is critical for maximizing patient outcomes. An RPS Trans-Atlantic Working Group was established in 2013. The primary aim was to evaluate the current evidence critically and to develop a consensus document on the approach to this difficult disease. The outcome applies to primary RPS that is nonvisceral in origin. The evaluation included sarcomas of major veins (inferior vena cava, renal vein, ovarian/testicular vein), undifferentiated pleomorphic sarcoma of the psoas, and ureteric leiomyosarcoma (LMS). It excluded desmoid, lipoma and angiomyolipoma, gastrointestinal stromal tumors, visceral sarcomas such as those arising from the gut or its mesentery, uterine LMS, prostatic sarcoma, paratesticular/spermatic cord sarcoma, Ewing's sarcoma, alveolar/embryonal rhabdomyosarcoma, primitive peripheral neuro-ectodermal tumor, sarcoma arising from teratoma, carcinosarcoma, sarcomatoid carcinoma, clear cell sarcoma, radiation-induced sarcoma, paraganglioma, and malignant pheochromocytoma. Management of RPS was evaluated from diagnosis to follow-up, and a level of evidence was attributed to each statement. This rare and complex malignancy is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, and an individualized management plan should be made based on the statements included in this article. International collaboration is critical for adding to the current knowledge. A prospective registry will be set up.
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            Lipomas, lipoma variants, and well-differentiated liposarcomas (atypical lipomas): results of MRI evaluations of 126 consecutive fatty masses.

            Our objectives were to evaluate the reliability of MRI in distinguishing simple lipomas, lipoma variants, and well-differentiated liposarcomas (atypical lipomas) and to identify various imaging mimics of well-differentiated liposarcoma. One hundred twenty-six consecutively imaged grossly fatty masses were retrospectively reviewed. MRI examinations, their prospective interpretations, and their corresponding pathology reports were compared to determine the reliability of MRI in distinguishing simple lipomas, lipoma variants, and well-differentiated liposarcomas. The success of MRI in identifying well-differentiated liposarcomas among other fatty masses was as follows: sensitivity, 100%; specificity, 83%; accuracy, 84%; positive predictive value, 38%; and negative predictive value, 100%. MRI was 100% specific in the diagnosis of simple lipoma. Sixty-three percent of lesions considered suspicious for well-differentiated liposarcoma were actually simple lipomas (13%) and benign lipoma variants (50%), including chondroid lipoma (13%), osteolipoma (6%), hibernoma (6%), lipoleiomyoma (6%), angiolipoma (6%), and infarcted lipoma (13%). Because of differences in treatment, prognosis, and long-term follow-up, it is important to preoperatively distinguish simple lipomas from well-differentiated liposarcomas. MRI is highly sensitive in the detection of well-differentiated liposarcomas and highly specific in the diagnosis of simple lipomas. However, when an extremity or body wall lesion is considered suspicious for well-differentiated liposarcoma, it is more likely (64%) to represent one of many benign lipoma variants.
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              Accuracy of Preoperative Percutaneous Biopsy for the Diagnosis of Retroperitoneal Liposarcoma Subtypes

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                Author and article information

                Contributors
                Journal
                J Surg Case Rep
                J Surg Case Rep
                jscr
                Journal of Surgical Case Reports
                Oxford University Press
                2042-8812
                March 2022
                26 March 2022
                26 March 2022
                : 2022
                : 3
                : rjac121
                Affiliations
                Upper Gastro-intestinal and Soft Tissue Unit , Princess Alexandra Hospital , Woolloongabba, QLD, Australia
                C/O Department of Surgical Specialties , Princess Alexandra Hospital , Woolloongabba, QLD, Australia
                Upper Gastro-intestinal and Soft Tissue Unit , Princess Alexandra Hospital , Woolloongabba, QLD, Australia
                Upper Gastro-intestinal and Soft Tissue Unit , Princess Alexandra Hospital , Woolloongabba, QLD, Australia
                Mayne Professor and Head , Discipline of Surgery, The University of Queensland , Woolloongabba, QLD, Australia
                Department of Anatomical Pathology , Princess Alexandra Hospital , Woolloongabba, QLD, Australia
                Author notes
                Correspondence address: C/O Department of Surgical Specialties, Princess Alexandra Hospital, Woolloongabba, QLD 4102, Australia. Tel: 0414745913; E-mail: Jasonlaurens78@ 123456gmail.com
                Author information
                https://orcid.org/0000-0003-2110-5306
                Article
                rjac121
                10.1093/jscr/rjac121
                8963145
                35355572
                18914d61-5a9b-40bd-aa5c-e3a16023cbad
                Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2022.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 1 October 2021
                : 2 March 2022
                : 4 March 2022
                Page count
                Pages: 3
                Categories
                Case Report
                AcademicSubjects/MED00910
                jscrep/0160

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