# Liposarcoma

Source: https://onco.cc/cancers/liposarcoma/  
OnCo record `liposarcoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Well-differentiated liposarcoma (atypical lipomatous tumour); Dedifferentiated liposarcoma; Myxoid liposarcoma; Pleomorphic liposarcoma
- Tags: subtype-page
- 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 repeatedly and are often fatal.
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/liposarcoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/liposarcoma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/liposarcoma/#what-it-is [4 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/liposarcoma/#finding-it [4 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/liposarcoma/#treating-it [4 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/liposarcoma/#evidence [5 trials, 4 key papers, 4 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/liposarcoma/#science [5 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/liposarcoma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/liposarcoma/#living-with-it [15 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/liposarcoma/coming/ [4 medicines, 5 trials, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/liposarcoma/data/ [29 connected records]

## Standard of care

- Well-differentiated, limb: Marginal excision; no radiotherapy or systemic therapy; surveillance for local recurrence. ([Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/))
- Retroperitoneal well- and dedifferentiated: Complete en bloc resection in a sarcoma centre; preoperative radiotherapy not routine after STRASS; repeat surgery for recurrence. ([Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Myxoid liposarcoma: Surgery with preoperative radiotherapy (highly radiosensitive); trabectedin for advanced disease. ([IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Trabectedin](https://onco.cc/drugs/trabectedin/))
- Advanced disease: Doxorubicin-based chemotherapy, eribulin (overall survival benefit in liposarcoma), trabectedin; MDM2 and CDK4 inhibitors in trials for amplified tumours. ([Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Eribulin](https://onco.cc/drugs/eribulin/), [Trabectedin](https://onco.cc/drugs/trabectedin/), [MDM2](https://onco.cc/targets/mdm2/))

## State of the art

- 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Liposarcoma
- Wikipedia: https://en.wikipedia.org/wiki/Liposarcoma

## Connected records

- cancers: [Leiomyosarcoma](https://onco.cc/cancers/leiomyosarcoma/), [Myxofibrosarcoma](https://onco.cc/cancers/myxofibrosarcoma/), [Retroperitoneal sarcoma](https://onco.cc/cancers/retroperitoneal-sarcoma/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/), [Soft tissue sarcoma of the extremity (localised and advanced)](https://onco.cc/cancers/extremity-soft-tissue-sarcoma/), [Synovial sarcoma](https://onco.cc/cancers/synovial-sarcoma/), [Undifferentiated pleomorphic sarcoma (UPS)](https://onco.cc/cancers/undifferentiated-pleomorphic-sarcoma/), [Uterine sarcoma](https://onco.cc/cancers/uterine-sarcoma/)
- drugs: [Brigimadlin](https://onco.cc/drugs/brigimadlin/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Eribulin](https://onco.cc/drugs/eribulin/), [Trabectedin](https://onco.cc/drugs/trabectedin/)
- trials: [APG-115 in Combination With PD-1 Inhibitor in Patients With Advanced Liposarcoma or Advanced Solid Tumors](https://onco.cc/trials/nct04785196/), [Efficacy and Safety of Plitidepsin in Patients With Advanced Unresectable or Metastatic, Relapsed/Refractory, Dedifferentiated Liposarcoma (DLPS): an Exploratory Phase II Multicenter Trial](https://onco.cc/trials/nct01876043/), [SARC028](https://onco.cc/trials/sarc028/), [Spearhead 1 Study in Subjects With Advanced Synovial Sarcoma or Myxoid/Round Cell Liposarcoma](https://onco.cc/trials/nct04044768/), [STRASS (EORTC 62092)](https://onco.cc/trials/strass/)
- key papers: [Eribulin versus dacarbazine in previously treated advanced liposarcoma or leiomyosarcoma](https://onco.cc/key-papers/paper-eribulin-liposarcoma-schoffski-lancet-2016/), [Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS clinical practice guideline](https://onco.cc/key-papers/paper-esmo-sts-guideline-gronchi-ann-oncol-2021/), [STRASS (EORTC 62092): preoperative radiotherapy plus surgery versus surgery alone for primary retroperitoneal sarcoma](https://onco.cc/key-papers/paper-strass-lancet-oncol-2020/), [Trabectedin versus dacarbazine for metastatic liposarcoma or leiomyosarcoma after anthracycline failure](https://onco.cc/key-papers/paper-trabectedin-vs-dacarbazine-demetri-jco-2016/)
- targets: [ACSL6](https://onco.cc/targets/acsl6/), [DDIT3](https://onco.cc/targets/ddit3/), [FUS](https://onco.cc/targets/fus/), [MB21D2](https://onco.cc/targets/mb21d2/), [MDM2](https://onco.cc/targets/mdm2/)
- technologies: [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [MDM2 inhibitors](https://onco.cc/technologies/mdm2-inhibitors/), [Methylation classifier for sarcomas](https://onco.cc/technologies/sarcoma-methylation-classifier/)
- people: [Patrick Schöffski](https://onco.cc/people/patrick-schoffski/), [Sylvie Bonvalot](https://onco.cc/people/sylvie-bonvalot/)
- companies: [SARC (Sarcoma Alliance for Research through Collaboration)](https://onco.cc/companies/sarc/)

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JSON: https://onco.cc/api/v1/entities/liposarcoma.json