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Abstract：
Anaplastic thyroid cancer (ATC) is the most aggressive solid tumour known and is a rare but highly lethal form of thyroid cancer that requires a multidisciplinary team approach. No Spanish consensus exists for management of patients with ATC. The Thyroid Cancer Group of the Spanish Society of Endocrinology and Nutrition and the GETHI (Grupo Español de Enfermedades Huérfanas e Infrecuentes) of the Spanish Society of Oncology, in agreement with the Boards of these Societies, commissioned an independent task force to develop a wide consensus on ATC. The relevant literature was reviewed, including serial PubMed searches supplemented with additional articles. The consensus includes the characteristics, diagnosis, initial evaluation, establishment of treatment goals, approaches to locoregional disease (surgery, radiotherapy, systemic therapy, supportive care during active treatment), approaches to advanced/metastatic disease, palliative care options, monitoring, and long-term follow-up of ATC. For operable disease, a combination of radical surgery with adjuvant radiotherapy or chemotherapy, using agents such as doxorubicin, cisplatin and paclitaxel, is the best treatment strategy. Cytotoxic drugs are poorly effective for advanced/metastatic ATC. On the other hand, targeted agents may represent a viable therapeutic option. Patients with stage IVA/IVB resectable disease have the best prognosis, particularly if a multimodal approach is used, and some stage IVB unresectable patients may respond to aggressive therapy. Patients with stage IVC disease should be considered for clinical trials or for hospice/palliative care depending on their preference. This is the first Spanish consensus for ATC, and provides recommendations for management of this extremely aggressive malignancy. Novel systemic therapies are being tested, and more effective combinations are needed to improve patient outcomes. Although more aggressive radiotherapy has reduced locoregional recurrence, mean overall survival has not improved in the past 50 years.
摘要：
间变性甲状腺癌（ATC）是已知最具侵袭性的实体瘤，是一种罕见但高度致命的甲状腺癌形式，需要多学科团队方法。西班牙对ATC患者的治疗尚无共识。西班牙内分泌与营养学会甲状腺癌小组和西班牙肿瘤学学会的GETHI（GrupoEspañoldeEnfermedadesHuérfanaseInfrecuentes），与这些协会的董事会达成一致，委托一个独立的工作队就ATC达成广泛共识。回顾了相关文献，包括补充了其他文章的串行PubMed搜索。共识包括特征，诊断,初步评估，建立治疗目标，局部疾病的方法(手术，放射治疗,全身治疗,积极治疗期间的支持性护理)，晚期/转移性疾病的方法，姑息治疗方案,监测,以及ATC的长期随访。对于可手术的疾病，根治性手术与辅助放疗或化疗相结合，使用阿霉素等药物，顺铂和紫杉醇，是最好的治疗策略。细胞毒性药物对晚期/转移性ATC效果不佳。另一方面,靶向药物可以代表可行的治疗选择。IVA/IVB期可切除疾病患者预后最好，特别是如果使用多模态方法，某些无法切除的IVB期患者可能对积极治疗有反应。IVC疾病阶段的患者应根据他们的喜好考虑进行临床试验或临终关怀/姑息治疗。这是西班牙对ATC的第一个共识，并为治疗这种极具侵袭性的恶性肿瘤提供了建议。新的系统疗法正在测试中，需要更有效的组合来改善患者的预后。虽然更积极的放疗减少了局部复发，在过去50年中,平均总生存期没有改善.
