{"entity":{"id":"liposarcoma","kind":"cancer","name":"Liposarcoma","aka":["Well-differentiated liposarcoma (atypical lipomatous tumour)","Dedifferentiated liposarcoma","Myxoid liposarcoma","Pleomorphic liposarcoma"],"tldr":"Liposarcoma is a cancer of fat cells that comes in four different forms: two driven by extra copies of the MDM2 gene, one by a fusion gene that makes it unusually sensitive to radiotherapy and trabectedin, and one that behaves like other aggressive sarcomas. Surgery is the mainstay and MDM2 inhibitors are the most promising drugs in trials.","summary":"Well-differentiated and dedifferentiated liposarcoma share amplification of MDM2 and CDK4 on chromosome 12 and arise mostly in the retroperitoneum and thigh; the well-differentiated form does not metastasise but recurs locally, and the dedifferentiated form is aggressive. Myxoid liposarcoma carries the FUS-DDIT3 fusion, occurs in younger adults, spreads to unusual soft-tissue and bone sites and is strikingly sensitive to radiotherapy and to trabectedin. Pleomorphic liposarcoma is genomically complex and treated like other high-grade sarcomas. Surgery is the mainstay, with radiotherapy for limb tumours and, for retroperitoneal disease, the STRASS trial failed to show a benefit of preoperative radiotherapy overall. Eribulin improved survival in advanced liposarcoma, trabectedin is effective in myxoid disease, and MDM2 inhibitors such as brigimadlin and CDK4/6 inhibitors are in late trials for the MDM2-amplified forms.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Liposarcoma","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Liposarcoma"}],"tags":["subtype-page"],"related":[],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"notes":[],"group":"sarcoma","burden":"The commonest soft-tissue sarcoma of adults, about a fifth of the total; outcomes range from near-benign well-differentiated tumours of the limbs to dedifferentiated retroperitoneal tumours that recur relentlessly and kill.","subtypes":["Well-differentiated liposarcoma (atypical lipomatous tumour; MDM2-amplified)","Dedifferentiated liposarcoma (MDM2-amplified, aggressive)","Myxoid and round cell liposarcoma (FUS-DDIT3)","Pleomorphic liposarcoma"],"biomarkers":["MDM2 and CDK4 amplification (FISH; separates from lipoma)","FUS-DDIT3 fusion (myxoid)","Round cell component (myxoid prognosis)","Grade and site (retroperitoneal versus limb)"],"standardOfCare":[{"setting":"Well-differentiated, limb","approach":"Marginal excision; no radiotherapy or systemic therapy; surveillance for local recurrence.","refs":["sarcoma"]},{"setting":"Retroperitoneal well- and dedifferentiated","approach":"Complete en bloc resection in a sarcoma centre; preoperative radiotherapy not routine after STRASS; repeat surgery for recurrence.","refs":["sarcoma","imrt-igrt"]},{"setting":"Myxoid liposarcoma","approach":"Surgery with preoperative radiotherapy (highly radiosensitive); trabectedin for advanced disease.","refs":["imrt-igrt","trabectedin"]},{"setting":"Advanced disease","approach":"Doxorubicin-based chemotherapy, eribulin (overall survival benefit in liposarcoma), trabectedin; MDM2 and CDK4 inhibitors in trials for amplified tumours.","refs":["doxorubicin","eribulin","trabectedin","mdm2"]}],"stateOfArt":["MDM2 amplification is both the diagnostic test and the therapeutic target, with brigimadlin in a phase 3 trial against doxorubicin.","Eribulin was the first drug to lengthen survival specifically in liposarcoma.","Myxoid liposarcoma's radiosensitivity allows reduced-dose preoperative radiotherapy."],"history":[{"year":1962,"title":"Enzinger and Winslow classify liposarcoma","refs":[]},{"year":1993,"title":"MDM2 amplification found in well-differentiated and dedifferentiated liposarcoma","refs":["mdm2"]},{"year":2016,"title":"Eribulin approved for liposarcoma after a survival benefit","refs":["eribulin"]},{"year":2020,"title":"STRASS: preoperative radiotherapy does not improve retroperitoneal sarcoma outcomes overall","refs":[]}],"pipeline":["mdm2-inhibitors","eribulin"],"openProblems":["Retroperitoneal dedifferentiated disease recurs in most patients despite complete surgery.","MDM2 inhibitors cause low platelets that limit dosing.","No systemic therapy is proven in the adjuvant setting."],"parent":"sarcoma"},"route":"/cancers/liposarcoma/","neighbours":{"target":[{"id":"mdm2","kind":"target","name":"MDM2","route":"/targets/mdm2/"}],"drug":[{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"eribulin","kind":"drug","name":"Eribulin","route":"/drugs/eribulin/"},{"id":"trabectedin","kind":"drug","name":"Trabectedin","route":"/drugs/trabectedin/"}],"technology":[{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"},{"id":"mdm2-inhibitors","kind":"technology","name":"MDM2 inhibitors","route":"/technologies/mdm2-inhibitors/"}],"cancer":[{"id":"sarcoma","kind":"cancer","name":"Sarcomas (soft tissue, bone, GIST)","route":"/cancers/sarcoma/"}]}}