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Leiomyosarcoma

Malignant Tumor of Smooth Muscle Differentiation

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Onkoloji department. Book Appointment →

What is Leiomyosarcoma?

Leiomyosarcoma (LMS) is a malignant mesenchymal neoplasm with smooth muscle differentiation, accounting for 10-20% of soft tissue sarcomas.

Anatomic subtypes include uterine LMS (most common, distinct biology), retroperitoneal LMS (often arising from inferior vena cava), gastrointestinal LMS (rare, distinct from GIST), cutaneous LMS, and vascular LMS.

Molecular landscape involves complex karyotypes, frequent TP53 and RB1 alterations, and loss of MED12 in uterine forms.

Symptoms

Uterine LMS: postmenopausal bleeding, rapid uterine enlargement, pelvic pain
Retroperitoneal LMS: vague abdominal/back pain, lower extremity edema, weight loss
Vascular LMS (IVC): leg swelling, Budd-Chiari syndrome, abdominal pain
Gastrointestinal LMS: bleeding, obstruction, abdominal mass
Cutaneous LMS: dermal nodule, often misdiagnosed as benign lesion
Pulmonary metastasis common at diagnosis or follow-up: dyspnea, cough
Systemic symptoms in advanced disease: fatigue, weight loss

Risk Factors

Adult age 40-70 years
Female predominance for uterine and retroperitoneal forms
Prior tamoxifen therapy (uterine LMS — modest risk increase)
Hereditary leiomyomatosis and renal cell cancer syndrome (FH gene mutations)
Lynch syndrome (uterine LMS rarely)
Prior pelvic radiation
Immunosuppression (rare association)
Black ethnicity (uterine LMS slightly higher)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Postmenopausal bleeding or rapidly growing uterine fibroid
  • Persistent abdominal/pelvic pain with palpable mass
  • Unexplained leg swelling, especially unilateral
  • Cutaneous nodule rapidly enlarging or bleeding
  • GI bleeding or obstruction with imaging-detected mass
  • Pulmonary nodules in patient with prior sarcoma history

Treatment Methods

01
Wide surgical resection with negative margins — primary curative option
02
Hysterectomy with bilateral salpingo-oophorectomy for uterine LMS
03
Adjuvant chemotherapy controversial; considered for high-risk uterine LMS
04
First-line systemic for advanced disease: doxorubicin or doxorubicin + dacarbazine, or gemcitabine + docetaxel
05
Subsequent lines: trabectedin, eribulin, pazopanib, dacarbazine
06
Radiation therapy for local control in extremity or retroperitoneal cases
07
Metastasectomy for solitary or oligometastatic pulmonary disease
08
Long-term surveillance with chest CT and abdominal/pelvic imaging — high recurrence risk
09
Clinical trials with immunotherapy combinations and targeted agents

Which Department to Visit?

You can visit our Onkoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.