Soft Tissue Sarcoma Knowledge Centre
Understanding
Types by Site
STS are divided into a number of subtypes according to their histology. Some of the main subtypes are summarised in Table 1. The most frequently occurring STS subtypes include liposarcoma, leiomyosarcoma, synovial sarcoma and malignant fibrous histiocytoma1,2. The different subtypes differ in their biological behaviour, their response to treatment, and their prognosis. The majority of STS subtypes, including fibrosarcoma, liposarcoma, leiomyosarcoma, pleomorphic and synovial sarcomas, show limited sensitivity to current systemic therapies and are generally incurable when diagnosed as advanced ormetastatic disease3.
| Site | Histological subtype (% of patients) |
|---|---|
| Fat | Liposarcoma (15%) |
| Muscle | Leiomyosarcoma (12%), rhabdomyosarcoma (5%), gastrointestinal stromal tumours (GIST) |
| Perivascular | Haemangiopericytoma (<1%) |
| Joints | Synovial sarcoma (10%) |
| Fibrous | Fibrosarcoma (3%),malignant fibrous histiocytoma (28%) |
| Nervous System | Malignant schwannoma, neurofibrosarcoma, neurogenic sarcoma, primitive neuroectodermal tumours (PNET) - neuroblastoma (6%), Ewing’s sarcoma (2%) |
| Blood or Lymph vessels | Angiosarcoma (2%), Kaposi’s haemangioendothelioma (1%) |
In contrast, other histologic types like small round cell tumours (SRCTs), including Ewing’s sarcoma, are sensitive to current chemotherapy and potentially curable.4,5.
References:
1. Clark MA, Fisher C et al. (2005) “Soft-tissue sarcomas in adults.” N Engl JMed 353(7): 701–11.
2. Coindre J-M, Terrier P et al. (2001) “Predictive value of grade formetastasis development in themain histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group.” Cancer 91(10): 1914–26.
3. Hartmann JT, Patel S. (2005) “Recent developments in salvage chemotherapy for patients with metastatic soft tissue sarcoma.” Drugs 65(2): 167–78.
4. Bertuzzi A, Castagna L et al. (2002) “High-dose chemotherapy in poor-prognosis adult small round-cell tumors: clinical and molecular results from a prospective study.” J Clin Oncol 20(8): 2181–88.
5. Kushner BH, LaQuaglia MP et al. (1996) “Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy.” J Clin Oncol 14(5): 1526–31.
6. Cormier JN, Pollock RE (2004). “Soft tissue sarcomas”. CA Cancer J Clin 54(2): 94-109.