title: Emerging therapies for adult soft tissue sarcoma.
abstract: Soft tissue sarcoma (STS) are a broad group of rare tumors. Cornerstone of treatment is surgery. Complementary radiotherapy is recommended in high-risk STS arising from extremities. Doxorubicine ± ifosfamide based cytotoxic chemotherapy, explored in few randomized trials, showed a certain degree of activity, playing an established role only in unresectable disease. Since peculiar chemosensitivity towards alternative drugs was described for different metastatic subtypes in second or further lines, the modern concept of 'histology-driven chemotherapy' has been accepted and employed: gemicitabine ± dacarbazine, trabectedin and taxanes used respectively in patients with leiomyosarcoma, solitary fibrous tumor, myxoid/round cell liposarcoma, angiosarcoma. Recent discoveries about molecular pathways involved in STS tumorogenesis led to develop molecular targeted agents such as imatinib used in advanced dermatofibrosarcoma protuberans (DFSP) or metastatic DFSP-related fibrosarcoma, pazopanib, approved as second line regimen in advanced non-adipocitic STS and recently sunitinib in solitary fibrous tumors, alveolar soft part sarcoma and extraskeletal myxoid chondrosarcoma.
authors.affiliation: Department of Surgery, Sarcoma service, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133, Milano, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Radaelli
authors.forename: Sivia
authors.initials: S
authors.lastname: Stacchiotti
authors.forename: Paolo G
authors.initials: PG
authors.lastname: Casali
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gronchi
mesh_terms: D000328:Adult; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D056572:Histone Deacetylase Inhibitors / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D058990:Molecular Targeted Therapy; D020360:Neoadjuvant Therapy; D009363:Neoplasm Proteins / Q000037:antagonists & inhibitors; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D018714:Radiotherapy, Adjuvant; D012509:Sarcoma / Q000556:secondary / Q000628:therapy*; D015398:Signal Transduction / Q000187:drug effects; D012983:Soft Tissue Neoplasms / Q000473:pathology / Q000628:therapy*; D058570:TOR Serine-Threonine Kinases / Q000037:antagonists & inhibitors; D035703:Therapies, Investigational
chemical_list: D020533:Angiogenesis Inhibitors; D018931:Antineoplastic Agents, Hormonal; D056572:Histone Deacetylase Inhibitors; D007155:Immunologic Factors; D009363:Neoplasm Proteins; D047428:Protein Kinase Inhibitors; C546842:MTOR protein, human; D058570:TOR Serine-Threonine Kinases
keywords: adjuvant; chemotherapy; doxorubicin; ifosfamide; imatinib; neoadjuvant; palliation; sarcoma; trabectedin; trgeted therapy