ObjectiveTo understand the pathogenesis and the research progress of comprehensive treatment of primary retroperitoneal liposarcoma (PRLPS) and to provide evidence for clinical diagnosis and treatment.MethodThe recent literatures on the pathological classification, pathogenesis of PRLPS, and comprehensive treatment including the surgery, radiotherapy, chemotherapy, and molecular targeted therapy were reviewed.ResultsThe pathological types of PRLPS were highly differentiated, dedifferentiated, mucoid/round cell, polymorphic, and mixed. The main molecular pathogenesis was the synergistic effect of MDM2 with related genes, abnormal expressions of c-myc gene and microRNAs, Prune-nm23-H1 mechanism, and abnormal protein products of FUS-CHOP fusion gene which regulated the growth of tumor. The treatment of PRLRS included the radical resection, extended resection, and palliative resection combined with radiotherapy, chemotherapy, and molecular targeted therapy.ConclusionsPRLPS is a rare malignant tumor with high recurrence rate, but early diagnosis and treatment are difficult. With the further study of the molecular mechanism of PRLPS, the treatment of PRLPS has been transformed into a comprehensive treatment based on surgery, adjuvant radiotherapy and chemotherapy, and molecular targeted therapy.
ObjectiveTo analyze the ultrasonic manifestations of myxoid liposarcoma (MLS) in soft tissues.MethodsWe retrospectively analyzed the ultrasonic characteristics of 10 cases of soft tissue MLS which were pathologically confirmed between January and June 2006. Results Ultrasonic manifestations: 8 cases occurred in muscles and 2 in subcutaneous tissues and muscles; the maximum mass diameter was (71.1±39.4) mm; 10 cases had clear border, 8 had regular shape, and 9 had posterior echo enhancement; 10 cases were all solid masses with mucous liquid zone (9 cases had mainly weak echo, among which 5 had strong echo areas; 1 case had mainly strong echo); bloodstream grade: 3 cases belonged to level 0, 3 level Ⅰ, 2 level Ⅱ, and 2 level Ⅲ. Pathologic findings: 2 cases had low differentiation; 1 case was a mixture of MLS and atypical lipoma with mainly strong ultrasonic echo; the rest 7 cases had middle or high differentiation.ConclusionsUltrasonic manifestations of soft tissue MLS are mostly large masses in the muscular layer with clear border, regular shape, posterior echo enhancement and mucous liquid zone. Various degrees of strong echo zone exist in the masses. Bloodstream and mucous liquid zone are correlated with differentiation degree, and ultrasonic manifestations can be influenced if other factors exist.
ObjectiveTo present the diagnosis and treatment of a giant retroperitoneal liposarcoma (RPLS) involving the abdominal aorta, left renal hilum, pancreas, spleen, and left hemicolon under a multidisciplinary team (MDT) model. Methods A retrospective study of the clinical data of a 34-year-old female patient with RPLS who admitted to the Department of Vascular Surgery, West China Hospital of Sichuan University in July 2024. ResultsAbdomina contrast-enhanced CT revealed a large heterogeneous mass in the retroperitoneum and abdominal cavity, with the largest cross-section measuring approximately 31.4 cm×28.4 cm. The mass demonstrated poorly defined border, pressing and encasing the pancreas, stomach, duodenum, spleen, left kidney, left adrenal gland, pancreatic body and tail. Tumor showed indistinct demarcation to adjacent intestinal canal and closely adjacent to the abdominal aorta, inferior vena cava and its major branches. After the initial MDT discussion, the patient underwent extended retroperitoneal tumor resection, combined pancreaticosplenectomy, left hemicolectomy, and repair of the abdominal aorta and left renal vessels. The tumor was completely resected, and the left kidney was successfully preserved. The procedure lasted approximately 6 h, with an estimated blood loss of 4 000 mL. The patient received 8 U suspended red blood cells and 600 mL fresh frozen plasma. One week postoperatively, the patient developed grade B pancreatic fistula, abdominal infection, jaundice, and post-splenectomy thrombocytosis (peak platelet count 1 285×109/L), all of which were effectively managed after the second MDT consultation and coordinated treatment. The patient was discharged on postoperative day 20. Pathology confirmed dedifferentiated liposarcoma, graded as Fédération Nationale des Centres de Lutte Contre le Cancer grade 3. No tumor recurrence was observed during the 3-month follow-up. ConclusionIn the management of complex giant RPLS involving critical major vessels and organs, a comprehensive MDT-guided approach, including meticulous preoperative planning, precise intraoperative manipulation and protection of organ function, systematic perioperative care, are essential to achieving successful outcomes and patient recovery.