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Leiomyosarcoma is a cancer of smooth muscle, most often in the womb, the abdomen behind the bowel or the wall of a large vein. Surgery is the only cure; for spread disease, doxorubicin-based chemotherapy, trabectedin and gemcitabine-docetaxel are the standards, with no targeted drug yet.
Leiomyosarcoma arises from smooth muscle and is genomically complex, with TP53, RB1 and ATRX loss and no recurrent fusion. Uterine leiomyosarcoma is usually found unexpectedly after surgery for presumed fibroids, which is why morcellation of unsuspected tumours is avoided; retroperitoneal and vascular tumours present late. Complete surgical resection, with radiotherapy for limb tumours, is the only curative treatment and adjuvant chemotherapy has not proven benefit. Metastatic disease is treated with doxorubicin, alone or with trabectedin (LMS-04) or dacarbazine, gemcitabine-docetaxel, trabectedin and pazopanib; responses are modest and median survival is about two years. Immunotherapy has little activity, and trials target the disease's DNA repair and cell-cycle vulnerabilities.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
One of the two commonest soft-tissue sarcomas in adults, arising in the uterus, the retroperitoneum, large veins and limbs; about half of patients with deep tumours eventually develop metastases, most often 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, 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), Myxofibrosarcoma, 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)
Wide surgical resection; radiotherapy for limb and trunk tumours; no morcellation of uterine masses that could be sarcoma; adjuvant chemotherapy not standard.
Doxorubicin plus trabectedin (LMS-04) or doxorubicin alone; gemcitabine-docetaxel as an alternative, especially in uterine disease.
Trabectedin, pazopanib, dacarbazine-based regimens, aromatase inhibitors in hormone receptor-positive low-grade uterine disease; metastasectomy for limited lung spread.
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Doxorubicin-trabectedin is a first-line standard for fit patients with metastatic leiomyosarcoma, one of the few histology-specific first-line regimens in sarcoma.
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.
Trabectedin is a standard later-line option for liposarcoma and leiomyosarcoma and is especially active in myxoid liposarcoma.
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.
Gemcitabine-docetaxel is a standard first- or second-line option for leiomyosarcoma, especially uterine, and remains in use alongside doxorubicin-based regimens.
Query for this cancer: (TITLE:"Leiomyosarcoma" OR ABSTRACT:"Leiomyosarcoma" OR TITLE:"LMS" OR ABSTRACT:"LMS" OR TITLE:"Uterine leiomyosarcoma" OR ABSTRACT:"Uterine leiomyosarcoma" OR TITLE:"Smooth muscle sarcoma" OR ABSTRACT:"Smooth muscle sarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Leiomyosarcoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
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.
A swollen painful calf, or sudden breathlessness with chest pain; the tamoxifen boxed warning covers pulmonary embolism and stroke.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Take on an empty stomach (1 hour before or 2 hours after food).
See all on the product pages:DocetaxelDoxorubicinGemcitabineLetrozole (and other aromatase inhibitors)PazopanibTrabectedin·Printable cards in the navigator
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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.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.