# Myxofibrosarcoma

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

## TL;DR

Myxofibrosarcoma is a sarcoma of older people that grows just beneath the skin of the limbs and sends invisible tails along the tissue planes, so it comes back after surgery more often than any other sarcoma. Wide surgery guided by MRI, usually with radiotherapy, is the treatment; chemotherapy is reserved for spread disease.

## Summary

Myxofibrosarcoma is a fibroblastic sarcoma of late adult life, usually presenting as a painless superficial or subcutaneous mass in the limbs, and is now the commonest sarcoma diagnosis in the elderly. Its defining behaviour is infiltrative growth along fascial planes and septa well beyond the palpable mass, seen on MRI as curvilinear tail-like enhancement, and this explains a local recurrence rate that exceeds every other soft tissue sarcoma. Grade rises with each recurrence, and metastasis, mainly to the lungs, follows grade: rare in low-grade tumours, common in high-grade ones.

Treatment is wide surgical resection planned on contrast-enhanced MRI to encompass the tail sign, with radiotherapy for most intermediate- and high-grade tumours and for recurrent disease, given preoperatively or postoperatively as for other extremity sarcomas. Positive margins are common even in expert hands and re-excision is frequently needed. Systemic therapy for high-grade or metastatic disease follows the general soft tissue sarcoma approach with doxorubicin-based chemotherapy, and the tumour has no recurrent driver alteration to target.

Research has focused on the genomics of progression, with recurrent copy number gains and losses and TP53, CDKN2A and RB1 alterations shared with undifferentiated pleomorphic sarcoma, and on defining surgical margins by imaging so that the first operation is the last.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: MFS; Myxoid malignant fibrous histiocytoma (historic)
- Tags: subtype-page
- Group: sarcoma
- Burden: A common sarcoma of the elderly, typically a slow-growing lump just under the skin of the leg or arm; it recurs locally in a large minority of patients because of finger-like spread along tissue planes, and higher-grade tumours metastasise to the lungs.
- Subtypes: Low-grade myxofibrosarcoma (mostly local recurrence); Intermediate- and high-grade myxofibrosarcoma (metastatic risk); Epithelioid myxofibrosarcoma (aggressive variant); Superficial (subcutaneous) versus deep tumours
- Biomarkers: FNCLCC grade and grade progression at recurrence; MRI tail sign (infiltrative margins); TP53, CDKN2A and RB1 alterations; Absence of specific fusion or MDM2 amplification (excludes liposarcoma)

## 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/myxofibrosarcoma/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/myxofibrosarcoma/#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/myxofibrosarcoma/#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/myxofibrosarcoma/#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/myxofibrosarcoma/#treating-it [3 settings, 3 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/myxofibrosarcoma/#evidence [3 key papers, 3 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/myxofibrosarcoma/#science
- 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/myxofibrosarcoma/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/myxofibrosarcoma/#living-with-it [12 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/myxofibrosarcoma/coming/ [4 medicines, 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/myxofibrosarcoma/data/ [17 connected records]

## Standard of care

- Localised: Wide resection planned on contrast-enhanced MRI to include tail-like extensions; preoperative or postoperative radiotherapy for intermediate- and high-grade and recurrent tumours. ([Limb-salvage surgery and endoprosthetic reconstruction](https://onco.cc/technologies/limb-salvage-surgery/), [MRI](https://onco.cc/technologies/mri/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [FNCLCC grade (soft-tissue sarcoma)](https://onco.cc/terms/fnclcc-grade/))
- Recurrent local disease: Re-resection with radiotherapy if not previously given; amputation only for uncontrollable multiply recurrent disease. ([Limb-salvage surgery and endoprosthetic reconstruction](https://onco.cc/technologies/limb-salvage-surgery/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Metastatic: Doxorubicin-based chemotherapy as for other high-grade soft tissue sarcomas; metastasectomy for limited lung disease. ([Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Gemcitabine](https://onco.cc/drugs/gemcitabine/), [Docetaxel](https://onco.cc/drugs/docetaxel/))

## State of the art

- MRI-defined surgical margins that encompass the tail sign lower local recurrence.
- Radiotherapy is used for most tumours because margins are so often positive.
- Genomic studies place myxofibrosarcoma on a spectrum with undifferentiated pleomorphic sarcoma.

## Open problems

- Local recurrence remains the highest of any sarcoma.
- No targetable driver has been found.
- Elderly patients often cannot tolerate full-dose chemotherapy for metastatic disease.

## Sources

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

## Connected records

- cancers: [Leiomyosarcoma](https://onco.cc/cancers/leiomyosarcoma/), [Liposarcoma](https://onco.cc/cancers/liposarcoma/), [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/), [Undifferentiated pleomorphic sarcoma (UPS)](https://onco.cc/cancers/undifferentiated-pleomorphic-sarcoma/)
- key papers: [EORTC 62012: doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced soft tissue sarcoma](https://onco.cc/key-papers/paper-eortc-62012-doxorubicin-ifosfamide-judson-lancet-oncol-2014/), [Myxofibrosarcoma: clinicopathological analysis of 75 cases with emphasis on the low-grade variant](https://onco.cc/key-papers/paper-myxofibrosarcoma-mentzel-ajsp-1996/), [Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS clinical practice guideline](https://onco.cc/key-papers/paper-esmo-sts-guideline-gronchi-ann-oncol-2021/)
- technologies: [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Limb-salvage surgery and endoprosthetic reconstruction](https://onco.cc/technologies/limb-salvage-surgery/), [MRI](https://onco.cc/technologies/mri/)
- terms: [CDKN2A/B homozygous deletion](https://onco.cc/terms/cdkn2a-homozygous-deletion/), [FNCLCC grade (soft-tissue sarcoma)](https://onco.cc/terms/fnclcc-grade/)
- drugs: [Docetaxel](https://onco.cc/drugs/docetaxel/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Gemcitabine](https://onco.cc/drugs/gemcitabine/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/)

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