Management challenges of a giant retroperitoneal liposarcoma: A case report and review of the literature.

Abstract

Introduction and importance: Atypical lipomatous tumor/well differentiated liposarcoma (ATL/WDL) is an intermediate, locally aggressive malignant mesenchymal neoplasm composed either entirely or in part of a mature adipocytic proliferation showing significant variation in cell size and at least focal nuclear atypia in both adipocytes and stromal cells. Symptoms related to these tumors depend on the anatomic site.

Case presentation: A 61-year-old female presented with a long-standing worsening abdominal distension. Her vital signs and blood workup tests were within normal ranges. CT scans revealed a gigantic soft tissue mass occupying the entire abdominal cavity, displacing visceral organs. An en block surgical resection was attempted. Histopathology report confirmed the diagnosis of ALT/WDL, incompletely excised. Postoperative period was uneventful. To date, a year of close follow-up has passed; she remains disease-free.

Clinical discussion: The most important prognostic factor includes anatomic location and tumor size. These tumors do not metastasize unless they dedifferentiate, which is associated with significantly shorter overall survival. Subcutaneous or intramuscular tumors may recur but typically do not dedifferentiate or metastasize. The risk of dedifferentiation is directly related to location and duration of growth. Wide local excision with negative margins is curative. The efficacy and safety of further therapeutic choices, such as chemotherapy and radiotherapy, are still controversial.

Conclusion: As clinicians, it is important that we have a thorough understanding of the clinico-pathology, diagnosis, treatment, and prognosis of this tumor. Regular follow-up is important after treatment due to the risk of recurrence.