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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.
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.
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.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
Wide resection planned on contrast-enhanced MRI to include tail-like extensions; preoperative or postoperative radiotherapy for intermediate- and high-grade and recurrent tumours.
Re-resection with radiotherapy if not previously given; amputation only for uncontrollable multiply recurrent disease.
Doxorubicin-based chemotherapy as for other high-grade soft tissue sarcomas; metastasectomy for limited lung disease.
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The general principles on the sarcoma subtype pages (reference centre surgery, radiotherapy for high-grade deep tumours, doxorubicin first line, histology-directed later lines) follow this guideline.
Single-agent doxorubicin is the standard first-line palliative treatment for most advanced sarcomas, with doxorubicin-ifosfamide reserved for fit patients in whom tumour shrinkage matters.
The recognition that myxofibrosarcoma infiltrates along tissue planes far beyond the visible mass underlies the wide, MRI-planned excision and adjuvant radiotherapy recommended today.
Query for this cancer: (TITLE:"Myxofibrosarcoma" OR ABSTRACT:"Myxofibrosarcoma" OR TITLE:"MFS" OR ABSTRACT:"MFS" OR TITLE:"Myxoid malignant fibrous histiocytoma historic" OR ABSTRACT:"Myxoid malignant fibrous histiocytoma historic") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Myxofibrosarcoma, not a curated reading list.
No targets or pathways are linked to this cancer yet. Browse the gene hub →
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.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Do not give if bilirubin above ULN, or AST/ALT above 1.5 x ULN with alkaline phosphatase above 2.5 x ULN (treatment-related deaths).
Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
Alkylating chemotherapy can damage a blood stem cell in a way that shows up years later as myelodysplastic syndrome or acute myeloid leukaemia. It is uncommon, it depends on the total dose, and the risk falls away after about ten years. Knowing the cumulative dose you were given is the single most useful thing on your treatment summary.
Heart damage from cancer treatment: anthracyclines weaken the heart muscle permanently in a dose-related way, trastuzumab does so reversibly, and some kinase inhibitors raise blood pressure or disturb rhythm. Heart function (LVEF) is monitored by ultrasound during treatment.
See all on the product pages:DocetaxelDoxorubicinGemcitabineIfosfamide·Printable cards in the navigator
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