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Updated: Jun 22 2021


Images xray.jpg t1 lms.jpg 1.jpg mri lms.jpg 4.jpg
  • Summary
    • Leiomyosarcoma is an aggressive sarcoma thought to arise from the smooth muscle cells lining small blood vessels.
    • The condition usually presents in patients in the 5th and 6th decades of life, with pain and a palpable mass.
    • Diagnosis is made with biopsy showing a spindle cell neoplasm with similar characteristics shared between the osseous and soft tissue forms of this disease. Immunostains are positive for actin and vimentin.
    • Treatment is generally wide surgical resection with radiation. 
  • Epidemiology
    • Incidence
      • fewer than 100 case reports of extra-facial leiomyosarcoma of bone
    • Demographics
      • the mean age of presentation is in 5th and 6th decades of life
    • Anatomic location
      • most frequently occurs in the metaphysis of long bones, but can occur in diaphyseal locations.
      • the most frequent sites of boney presentation are the femur, tibia, ilium, and humerus
  • Presentation
    • Symptoms
      • bone pain
      • palpable mass
      • typical duration of symptoms prior to diagnosis is 6 months
    • Physical exam
      • pelvic masses can be difficult to appreciate on inspection exam
      • can be tender or nontender to palpation
      • masses will be firm
  • Imaging
    • Radiographs
      • involved extremity
        • purely osteolytic lesions with ill-distinct margins, moth-eaten, or permeative pattern of bone destruction.
        • primarily intra-medullary but may extend into the soft tissues.
      • chest
        • indicated for all staging of tumors to evaluate for lung metastasis
    • CT
      • indications
        • help evaluate bone loss of involved extremity or pelvis
        • chest CT indicated in all patients as part of staging workup to evaluate for metastasis
    • MRI
      • indications
        • differentiate from benign soft tissue tumors
        • evaluate size, depth, and surrounding anatomy
      • views
        • appears dark on T1, similar to muscle tissue
        • appears heterogeneous on T2 with areas of increased signal intensity
        • contrast causes diffuse enhancement of signal within the lesion
  • Studies
    • Histology
      • a spindle cell neoplasm with similar characteristics shared between the osseous and soft tissue forms of this disease
      • cigar-shaped nuclei
      • cells arranged into fascicles along with myofibrils running parallel
      • presence of actin and vimentin immunoreactivity
    • Staging
      • all tumors should be staged appropriately prior to treatment initiation
  • Treatment
    • Nonoperative
      • chemotherapy
        • indications
          • diffuse metastatic disease to the lungs
          • large pelvic masses with neurovascular involvement that preclude safe resection
        • outcomes
          • variable response to chemotherapy
          • better survival when chemotherapy combined with surgery than surgery alone
      • radiation
        • indications
          • controversial
          • contaminated resection bed
        • outcomes
          • variable response, difficult to predict
    • Operative
      • early wide resection of the primary lesion and secondary reconstruction
        • indications
          • standard of care in localized disease
        • technique
          • clean margin is an important goal of surgical resection
        • chemotherapy
          • Neo-adjuvant or adjuvant chemo/radiotherapy in the treatment of leiomyosarcoma of bone is controversial
        • radiation
          • if unable to obtain wide margin consider adjuvant radiation
  • Prognosis
    • LMS of bone in adults
      • 25% recurrence rate and 25% metastasis rate
      • 75% survival at 3 years with treatment
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