Rare mesenchymal tumors that are categorized as soft tissue or bone sarcomas.
More common in pediatric cancers and make up 15-30% of lesions.
Only 1% of adult cancers are sarcomas.
More than 70 different subtypes exist.
Approximately 15% of pediatric cancers are sarcomas.
Soft tissue sarcomas makeup approximately 80% of all sacomas, and the remaining 20% from bone.
Approximately 13,000 new cases of only and soft tissue sarcoma were diagnosed in the United States in 2008.
Approximately 3 and 100,000 people develop soft tissue sarcomas annually and one in 100,000 people develop bone sarcomas annually.
High incidence of locally aggressive behavior and predilection for metastases.
Approximately 50% of patients will either present or ultimately develop metastatic disease.
Alveolar rhabdomyosarcomas, Ewing’s sarcoma and synovial sarcoma occur in younger patients and have tumor specific chromosomal translocations that are incorporated into diagnostic criteria.
Leiomyosarcomas and malignant fibrous histiocytomas occur more frequently in older adults and are characterized by frequent chromosome copy number changes.
Most lesions have abnormalities in retinoblastoma, p53. and growth factor signaling pathways.
Insulin-like growth factor 1 receptor pathway commonly activated pathway in many sarcomas.
Abnormalities lead to the activation of the mammalian target of rapamycin (m-TOR) pathway.
X-rays have a limited role in the detection and evaluation of soft tissue and bone sarcomas because greater than 40% of bone destruction is necessary before it is evident on conventional radiographs.
Radiographs may be helpful in the evaluation of new bone formation in osteosarcoma and chondroid calcifications in chondroid lesions.
Radiographs may be helpful in patients with soft tissue calcifications such as scene with synovial sarcoma.
CT scans have limited ability to differentiate subtle soft tissue differences and therefore plays a complementary role to MRI in the evaluation of the extent of sarcomas.
MRI with superior soft tissue contrast resolution is the dominant imaging technique in evaluating extremity sarcomas.
Gemcitabine and docetaxel associated with an improved progression free survival of an overall survival compared to Gemcitabine alone.
Patients randomized to eribulin compared to dacarbazine: the median survival of 13months versus 11.5 months.
Combination of nivolumab and pembrolizumab have a response rate of 16% in heavily pretreated patients in metastatic sarcoma (D’angelo S).