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Introduction to the management of soft tissue sarcomas
KEY POINTS
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Sarcomas are an extremely heterogeneous group of mesenchymal cancers accounting for approximately 1% of all adult malignancies.
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Pathologic diagnosis should rely on specialized expertise of highly skilled pathologists in referral centers.
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Molecular pathology, including individual genetic profiling, is becoming an integral part of diagnosis and is a platform for future research to find new therapeutic options.
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A prevalent nihilistic approach of treating advanced/metastatic soft tissue sarcomas (STS) cannot be accepted; median survival after the development of distant metastases is up to 14.3 to 19 months, not any worse than figures seen in some of the more frequent solid tumor types.
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Auxiliary local interventions, such as metastasectomy, radiofrequency ablation/microwave ablation should always be considered in cases with oligometastatic disease.
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One of the most important factors to consider in therapeutic decision-making is the ascertained chemosensitivity of the specific sarcoma subtype to be treated.
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Doxorubicin-based therapy is still the “gold standard” first-line choice in the palliation of all STS.
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The introduction of novel antineoplastic agents, such as pazopanib, trabectedin, and eribulin have further exposed the differential sensitivity of various subtypes of sarcomas to specific agents, and challenges a rigid “one therapeutic sequence fits all” approach.
Introduction
Owing to the extreme histologic heterogeneity of soft tissue sarcomas (STS), their management is an art of its own. More recently the treatment of STS has been slowly shifting toward a more individualized, histology driven approach. The introduction of novel antineoplastic agents, such as pazopanib, trabectedin, and eribulin has further exposed the differential sensitivity of various sarcoma subtypes to specific agents.
Epidemiology
Sarcomas are an extremely heterogeneous group of mesenchymal cancers accounting for approximately 1% of all adult malignancies and 12% of pediatric cancers. With an incidence of approximately 45 million population per year, approximately every fifth sarcoma originates from the bone, whereas the remaining 80% to 85% of cases arise from soft tissues. Although most STS arise from the extremities, they can originate from any part of the body, necessitating complex multidisciplinary management.
The latest World Health Organization classification lists around 100 sarcoma subtypes; thus, the pathologic diagnostic process often relies (and should rely) on specialized expertise of highly skilled pathologists in referral centers. Among adults, gastrointestinal stromal tumors (GIST) are the most common STS (~18%), followed by pleomorphic/unclassified STS (~16%), liposarcomas (~15%), leiomyosarcomas (~11%), and dermatofibrosarcoma (~5%).
Risk factors and screening
In the majority of cases there is no known risk factor. Radiation-associated sarcomas are a well-known complication of previous therapy for lymphoma, breast, cervical, and testicular cancer. Radiation-associated sarcomas occur in less than 1% of treated patients. Pleomorphic and angiosarcoma are common radiation-associated sarcoma subtypes.
A number of genetic conditions predispose to the development of sarcomas and soft tissue tumors, including neurofibromatosis 1, retinoblastoma, Li-Fraumeni, and Gardener’s syndrome.
Chronic lymphoedema can result in angiosarcoma, which was first described by Stewart and Treves after mastectomy in patients with breast cancer.
Immunodeficiency and human herpes virus 8 infection cause Kaposi’s sarcoma. Furthermore, Epstein-Barr virus infection has been associated with smooth muscle tumors, including leiomyosarcoma.
Currently, there is no screening for sarcomas. However, patients with any of the hereditary syndromes described above should undergo appropriate surveillance and follow up in experienced clinics.
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