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      Intraperitoneal Liposarcoma: A Case Report and Literature Review of a Rare Entity

      case-report
      1 , , 2 , 1 , 1
      ,
      Cureus
      Cureus
      mdm2, intra-peritoneal, liposarcoma, recurrent sarcoma, dedifferentiated liposarcoma

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          Abstract

          Liposarcoma is a rare soft-tissue neoplasm originating from adipocytes. The exact cause of liposarcoma is unknown and symptoms vary depending on the tumor’s location. A 49-year-old man presented to the emergency room complaining of epigastric pain radiating to the back and right upper quadrant. Cross-sectional imaging revealed a large upper abdominal mass that was thought to be a gastrointestinal stromal tumor (GIST) arising from the duodenum at first. The patient underwent en-bloc resection of the mass and was planned for adjuvant chemotherapy. Subsequently, multiple tissue samples were examined, leading to the final diagnosis of de-differentiated liposarcoma. The patient eventually developed multiple recurrences and was subjected to re-resection surgeries and three different chemotherapy regimens. Given the rarity of the disease, no standardized therapy plan is available, highlighting the need for more case reports/series and trials to broaden our understanding of this disease.

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          Most cited references70

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          The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives

          Mesenchymal tumours represent one of the most challenging field of diagnostic pathology and refinement of classification schemes plays a key role in improving the quality of pathologic diagnosis and, as a consequence, of therapeutic options. The recent publication of the new WHO classification of Soft Tissue Tumours and Bone represents a major step toward improved standardization of diagnosis. Importantly, the 2020 WHO classification has been opened to expert clinicians that have further contributed to underline the key value of pathologic diagnosis as a rationale for proper treatment. Several relevant advances have been introduced. In the attempt to improve the prediction of clinical behaviour of solitary fibrous tumour, a risk assessment scheme has been implemented. NTRK-rearranged soft tissue tumours are now listed as an “emerging entity” also in consideration of the recent therapeutic developments in terms of NTRK inhibition. This decision has been source of a passionate debate regarding the definition of “tumour entity” as well as the consequences of a “pathology agnostic” approach to precision oncology. In consideration of their distinct clinicopathologic features, undifferentiated round cell sarcomas are now kept separate from Ewing sarcoma and subclassified, according to the underlying gene rearrangements, into three main subgroups (CIC, BCLR and not ETS fused sarcomas) Importantly, In order to avoid potential confusion, tumour entities such as gastrointestinal stroma tumours are addressed homogenously across the different WHO fascicles. Pathologic diagnosis represents the integration of morphologic, immunohistochemical and molecular characteristics and is a key element of clinical decision making. The WHO classification is as a key instrument to promote multidisciplinarity, stimulating pathologists, geneticists and clinicians to join efforts aimed to translate novel pathologic findings into more effective treatments.
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            Preoperative radiotherapy plus surgery versus surgery alone for patients with primary retroperitoneal sarcoma (EORTC-62092: STRASS): a multicentre, open-label, randomised, phase 3 trial

            Unlike for extremity sarcomas, the efficacy of radiotherapy for retroperitoneal sarcoma is not established. The aim of this study was to evaluate the impact of preoperative radiotherapy plus surgery versus surgery alone on abdominal recurrence-free survival.
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              Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation.

              We studied 155 cases of dedifferentiated liposarcoma to define its clinicopathologic features and behavior, in particular how the extent and grade of dedifferentiation affected outcome. Tumors occurred in late adult life (median, 61.5 years: range, 21-92 years), most commonly in the retroperitoneum (106 cases), extremities and trunk (32 cases), and scrotum/spermatic cord (13 cases). The majority of dedifferentiated liposarcomas presented as de novo lesions, whereas the remainder developed as a late complication of a preexisting well-differentiated liposarcoma after an average interval of 7.7 years. At the time of presentation, most of the dedifferentiated liposarcomas displayed extensive areas of high-grade dedifferentiation resembling malignant fibrous histiocytoma or high-grade fibrosarcoma, whereas a minority contained only areas of low-grade dedifferentiation resembling fibromatosis or well-differentiated fibrosarcoma. Divergent myosarcomatous or osteosarcomatous differentiation was observed focally in six cases. The behavior of dedifferentiated liposarcomas was that of a high-grade sarcoma with a local recurrence rate of 41%, a metastatic rate of 17%, and disease-related mortality of 28%. The most important prognostic factor was location in that retroperitoneal tumors had significantly worse survival than those in other sites. Tumors were divided into those having less than or those with more than 25% dedifferentiation, and dedifferentiated zones were classified into low grade or high grade. Neither low-grade dedifferentiation nor a low percentage of dedifferentiation was associated with an improved outcome for the tumors examined in this study; however, in no cases was the absolute size of the dedifferentiated focus <2 cm. Therefore, this study did not determine a minimum, or threshold, amount of dedifferentiation below which outcome was more favorable. The behavior of liposarcomas in which the dedifferentiated component was entirely low grade was more similar to that of traditional dedifferentiated liposarcoma than to that of well-dedifferentiated liposarcoma. Our study supports the expansion of the definition of dedifferentiated liposarcoma to include tumors with low-grade dedifferentiation and also suggests that low-grade dedifferentiation represents a precursor lesion of high-grade dedifferentiation.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                28 April 2024
                April 2024
                : 16
                : 4
                : e59244
                Affiliations
                [1 ] Radiology, King Fahad Medical City, Riyadh, SAU
                [2 ] Radiology, Altakassusi Alliance Medical, Riyadh, SAU
                Author notes
                Abdullah K. AlBathi aalbathi@ 123456kfmc.med.sa
                Article
                10.7759/cureus.59244
                11134115
                38813304
                6238775c-312d-4551-b60a-8586e95b7fde
                Copyright © 2024, AlBathi et al.

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

                History
                : 28 April 2024
                Categories
                Radiology
                General Surgery
                Oncology

                mdm2,intra-peritoneal,liposarcoma,recurrent sarcoma,dedifferentiated liposarcoma

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