Reichardt P
Sarkomzentrum Berlin-Brandenburg, Klinik für Interdisziplinäre Onkologie, HELIOS Klinikum Berlin-Buch, Schwanebecker Chaussee 50, 13125, Berlin, Deutschland.
Internist (Berl). 2016 Mar;57(3):245-56. doi: 10.1007/s00108-016-0021-2.
Soft tissue sarcomas are rare tumors that represent a major challenge due to varying clinical presentations and often interdisciplinary treatment concepts. Gold standard for the treatment of localized resectable soft tissue sarcomas is complete surgical removal. In metastatic soft tissue sarcoma, systemic therapy is the treatment of choice. The most active drugs are anthracyclines and ifosfamide. Combination chemotherapy has improved both response rate and progression-free survival at the cost of increased toxicity. Imatinib at a dose of 400 mg/day is the gold standard for patients with advanced or metastatic gastrointestinal stromal tumors (GIST). In patients with a mutation in KIT exon 9, 800 mg/day is the recommended dose. In imatinib refractory or intolerant patients, sunitinib is recommended. Regorafenib has been approved for third-line therapy.
软组织肉瘤是罕见肿瘤，因其临床表现各异且治疗理念常涉及多学科，故而构成重大挑战。局限性可切除软组织肉瘤的治疗金标准是完整手术切除。在转移性软组织肉瘤中，全身治疗是首选治疗方法。最有效的药物是蒽环类药物和异环磷酰胺。联合化疗虽提高了缓解率和无进展生存期，但代价是毒性增加。对于晚期或转移性胃肠道间质瘤（GIST）患者，400毫克/天的伊马替尼是金标准治疗剂量。对于KIT外显子9发生突变的患者，推荐剂量为800毫克/天。对于伊马替尼难治或不耐受的患者，推荐使用舒尼替尼。瑞戈非尼已被批准用于三线治疗。
