• In the United States, there are 11,280 new cases annually; fewer than 1% of cancer diagnoses.
  • No specific etiologic agent is identifiable in the majority of cases.
  • Occasional cases are related to previous radiation, chemical exposure, alkylating chemotherapeutic agents, or chronic lymphedema.
  • Genetic conditions related to soft-tissue sarcoma include neurofibromatosis, tuberous sclerosis, basal cell nevus syndrome, Gardner syndrome, and Li-Fraumeni syndrome.
  • Core-needle biopsy (large lesions) or excisional biopsy (small lesions).
  • Pathological review of histologic subtype, grade, and assessment of margins (excisional biopsies).
  • Magnetic resonance imaging or computed tomography (CT) of primary site.
  • Chest x-ray for low-grade tumours and high-grade T1 lesions, chest CT for high-grade T2 tumours.
  • High-grade histology, deep location, and T2 tumour size are independent adverse prognostic factors for distant metastasis and survival.
  • Presentation with recurrent disease and positive surgical margin (gross or microscopic) are independent adverse prognostic factors for local recurrence.
  • Individual patient prognosis may be estimated by using a nomogram or newer techniques.
  • The American Joint Committee on Cancer (International Union Against Cancer) system uses criteria that include grade, size, and location relative to the investing muscular fascia, nodal status, and distant metastases. Other means to characterize risk of recurrence of soft-tissue sarcomas include nomograms and Bayesian belief networks.
  • Surgical resection with an adequate margin of normal tissue; for extremity lesions, a limb-sparing approach is possible in more than 90% of patients and offers survival comparable to amputation without the associated morbidity.
  • For most patients, local control is improved with preoperative or postoperative radiotherapy, with size and primary site also impacting its use.
  • The role of chemotherapy for high-risk patients remains controversial, but chemotherapy is used at several major centres for high-risk patients, especially for extremity tumours with known chemotherapy sensitivity, preoperatively when possible.
  • In addition to surgery, 3 years of adjuvant imatinib is the standard of care for high-risk gastrointestinal stromal tumour (GIST).
  • Local recurrence rates vary depending on the anatomic primary site and the adequacy of local therapy; for extremity lesions, approximately 20% of patients experience locally recurrent disease.
  • Systemic cytotoxic chemotherapy with selective use of surgery is the mainstay of therapy for patients with metastatic disease.
  • Kinase-directed therapy is clinically beneficial for patients with GIST; pazopanib was shown active in patients with other types of sarcoma who failed to respond to other systemic therapies, but has comparatively limited benefit.
  • For the small subset of patients who experience isolated (solitary) lung metastases, 20% to 50% 3-year survival rates have been reported with metastasis resection.

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About Genomic Medicine UK

Genomic Medicine UK is the home of comprehensive genomic testing in London. Our consultant medical doctors work tirelessly to provide the highest standards of medical laboratory testing for personalised medical treatments, genomic risk assessments for common diseases and genomic risk assessment for cancers at an affordable cost for everybody. We use state-of-the-art modern technologies of next-generation sequencing and DNA chip microarray to provide all of our patients and partner doctors with a reliable, evidence-based, thorough and valuable medical service.

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