New therapies in soft tissue sarcoma (original) (raw)

The Management of Adult Soft Tissue Sarcomas

American Journal of Clinical Oncology, 2009

Soft tissue sarcomas are a relatively rare, heterogeneous group of tumors arising from mesenchymal tissues and occurring almost anywhere in the body. The rate of progression and likelihood of hematogenous dissemination, usually to the lung, is determined primarily by tumor grade. The likelihood of regional spread is low. Pretreatment evaluation includes computed tomography of the primary site and chest and magnetic resonance imaging of the primary tumor. The mainstay of treatment is surgery; wide excision for low-grade lesions and wide or radical (compartmental) resection for high-grade tumors. Often, these procedures cannot be achieved either because of the location and extent of the sarcoma or anticipated functional deficit. Adjuvant preoperative and/or postoperative radiotherapy improves the likelihood of local control and preserves function when adequate margins cannot be achieved with surgery alone. The role of adjuvant chemotherapy is unclear; however, some data suggest the doxorubicin containing regimens may improve the likelihood of cure for high-grade lesions, particularly large tumors arising in the extremities. Prognosis is influenced by a variety of factors, including age, tumor size, histologic grade, depth (superficial or deep), histologic subtype, and site. Approximately 90% and 98% of recurrences are observed within 5 years and 10 years, respectively. Five-year overall survival rates range from approximately 60% to 80%.

SEOM Clinical Guideline of management of soft-tissue sarcoma (2016)

Clinical and Translational Oncology, 2016

Soft-tissue sarcomas are uncommon and heterogeneous tumors of mesenchymal origin. A soft-tissue mass that is increasing in size, greater than 5 cm, or located under deep fascia are criteria for suspicion of sarcoma. Diagnosis, treatment, and management should preferably be performed by a multidisciplinary team in reference centers. MRI and lung CT scan are mandatory for local and distant assessment. A biopsy indicating histological type and grade is needed previous to the treatment. Wide surgical resection with tumor-free tissue margin is the primary treatment for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not likely of being improved with reexcision. Neoadjuvant and adjuvant chemotherapy improve survival in selected cases, usually in high-grade sarcomas of the extremities. In the case of metastatic disease, patients with exclusive lung metastasis could be considered for surgery. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. New drugs have shown activity in second-line therapy and in specific histological subtypes.

A Review of Controversies in the Management of Soft Tissue Sarcomas

Soft tissue sarcomas (STS) constitute a rare and challenging group of solid tumor in the field of oncology. Unlike other malignancies STS can affect a wide variety of anatomical regions in the body with varied histopathological variants and clinical outcomes. There are controversies in the diagnosis and management of STS due to rarity and heterogeneity of the disease entity. Due to dedicated research and advances made in the field of imaging, pathology, surgery, radiotherapy and chemotherapy certain controversies were laid to rest and treatment approach to STS could be standardized to a large extent in the recent past. A review of controversies related to STS was performed in this article and an attempt was made to present a balanced view pertaining to these issues.

Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology

Journal of the National Comprehensive Cancer Network : JNCCN, 2016

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy.

NCCN Soft Tissue Sarcoma , Version 2 . 2016 Clinical Practice Guidelines in Oncology

2016

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/ superficial trunk/head and neck STS, as well as intra-abdominal/ retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy. J Natl Compr Canc Netw 2016;14(6):758–786 NCCN Categories of Evidence and Consensus Catego...

Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology

Journal of the National Comprehensive Cancer Network : JNCCN, 2018

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of STS of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations.

Soft Tissue Sarcomas with Special Reference to Molecular Aberration, Chemotherapy, and Recent Advances: A Review Article

Indian Journal of Medical and Paediatric Oncology, 2022

Soft tissue sarcomas (STS) are a diverse group of rare solid tumors of mesenchymal cell origin with distinct clinical and pathological features. They account for less than 1% of all adult malignancies and 15% of pediatric neoplasms. They include over hundreds of different histological subtypes. Many of these subtypes can occur at any age and are not confined to a specific site. Each subtype displays variable clinical behavior. Low incidence, variable presentation, behavior, and long-term outcomes further make it challenging to treat. There are multiple ongoing trials that focus on the anatomic site and histologic subtype to tailor the treatment. Further rarity of each histotype is a major barrier to recruit patients to randomized controlled trials. A multidisciplinary approach is mandatory in all cases of soft tissue sarcomas.The purpose of this review is to thoroughly understand the existing literature on history, incidence, epidemiology, etiology, histology, pathogenesis, diagnost...