{"entity":{"id":"myxofibrosarcoma","kind":"cancer","name":"Myxofibrosarcoma","aka":["MFS","Myxoid malignant fibrous histiocytoma (historic)"],"tldr":"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.\n\nTreatment 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.\n\nResearch 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.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Myxofibrosarcoma","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Myxofibrosarcoma"}],"tags":["subtype-page"],"related":["undifferentiated-pleomorphic-sarcoma","extremity-soft-tissue-sarcoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"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)"],"standardOfCare":[{"setting":"Localised","approach":"Wide resection planned on contrast-enhanced MRI to include tail-like extensions; preoperative or postoperative radiotherapy for intermediate- and high-grade and recurrent tumours.","refs":["limb-salvage-surgery","mri","imrt-igrt","fnclcc-grade"]},{"setting":"Recurrent local disease","approach":"Re-resection with radiotherapy if not previously given; amputation only for uncontrollable multiply recurrent disease.","refs":["limb-salvage-surgery","imrt-igrt"]},{"setting":"Metastatic","approach":"Doxorubicin-based chemotherapy as for other high-grade soft tissue sarcomas; metastasectomy for limited lung disease.","refs":["doxorubicin","ifosfamide","gemcitabine","docetaxel"]}],"stateOfArt":["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."],"history":[{"year":1977,"title":"Angervall, Kindblom and Merck describe myxofibrosarcoma","refs":[]},{"year":2002,"title":"WHO recognises myxofibrosarcoma as a distinct entity","refs":[]},{"year":2014,"title":"MRI tail sign linked to local recurrence and used to plan margins","refs":["mri"]}],"pipeline":[],"openProblems":["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."],"parent":"sarcoma"},"route":"/cancers/myxofibrosarcoma/","neighbours":{"cancer":[{"id":"sarcoma","kind":"cancer","name":"Sarcomas (soft tissue, bone, GIST)","route":"/cancers/sarcoma/"},{"id":"extremity-soft-tissue-sarcoma","kind":"cancer","name":"Soft tissue sarcoma of the extremity (localised and advanced)","route":"/cancers/extremity-soft-tissue-sarcoma/"},{"id":"undifferentiated-pleomorphic-sarcoma","kind":"cancer","name":"Undifferentiated pleomorphic sarcoma (UPS)","route":"/cancers/undifferentiated-pleomorphic-sarcoma/"}],"technology":[{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"},{"id":"limb-salvage-surgery","kind":"technology","name":"Limb-salvage surgery and endoprosthetic reconstruction","route":"/technologies/limb-salvage-surgery/"},{"id":"mri","kind":"technology","name":"MRI","route":"/technologies/mri/"}],"term":[{"id":"fnclcc-grade","kind":"term","name":"FNCLCC grade (soft-tissue sarcoma)","route":"/terms/fnclcc-grade/"}],"drug":[{"id":"docetaxel","kind":"drug","name":"Docetaxel","route":"/drugs/docetaxel/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"gemcitabine","kind":"drug","name":"Gemcitabine","route":"/drugs/gemcitabine/"},{"id":"ifosfamide","kind":"drug","name":"Ifosfamide","route":"/drugs/ifosfamide/"}]}}