Lang Brian Hung-Hin, Lo Chung-Yau
Division of Endocrine Surgery, Department of Surgery, University of Hong Kong Medical Centre, Queen Mary Hospital, Hong Kong, SAR, China.
World J Surg. 2007 May;31(5):969-77. doi: 10.1007/s00268-007-0776-7.
Undifferentiated or anaplastic carcinoma is an uncommon histologic type of thyroid cancer. It is one of the most aggressive malignancies associated with a poor prognosis. Most patients are elderly presenting as locally advanced disease with nodal and distant metastases. Complete surgical resection is frequently not possible and there is no effective systemic therapy. Aggressive multimodal therapy including surgery, radiation, and chemotherapy is recommended for management. However, because of the rarity of the disease, its aggressiveness and the lack of prospective treatment protocols, nearly all evidence in the literature comes from retrospective case series or cohort studies for selected patients' subgroups treated over a relatively long study period. Evidence of therapeutic benefit of one treatment option over another is lacking. Prognosis remains dismal with a median survival of 2-12 months Surgery remains an important component of the multimodal therapy and is commonly adopted as primary treatment. Although radical resection should be discouraged, resection without inducing significant morbidity can be considered for good risk patients with resectable tumors. It provides an effective form of palliation with potential cure when combined with postoperative radiotherapy and/or chemotherapy. Patients selected for surgical resection was frequently identified to have improved survival. A regime of preoperative hyperfractionated radiotherapy and doxorubicin followed by surgery when feasible has been documented to achieve local control and avoid tracheostomy for ATC patients. Because of the ineffectiveness of all conventional treatment modalities, novel molecular targeted therapies are being developed to tackling this uniformly fatal disease with promising results.
未分化癌或间变性癌是一种罕见的甲状腺癌组织学类型。它是最具侵袭性的恶性肿瘤之一,预后较差。大多数患者为老年人,表现为局部晚期疾病并伴有淋巴结和远处转移。通常无法进行完整的手术切除,且没有有效的全身治疗方法。建议采用包括手术、放疗和化疗在内的积极多模式治疗。然而,由于该疾病罕见、具有侵袭性且缺乏前瞻性治疗方案,文献中的几乎所有证据都来自回顾性病例系列或对相对较长研究期间选定患者亚组的队列研究。缺乏一种治疗方案优于另一种治疗方案的治疗益处证据。预后仍然很差,中位生存期为2至12个月。手术仍然是多模式治疗的重要组成部分,通常被用作主要治疗方法。虽然不鼓励进行根治性切除,但对于肿瘤可切除且风险较低的患者,可以考虑进行不会导致严重并发症的切除。当与术后放疗和/或化疗联合使用时,它提供了一种有效的姑息治疗形式,具有潜在的治愈可能。被选择进行手术切除的患者生存期常常得到改善。术前超分割放疗和阿霉素治疗,然后在可行时进行手术,已被证明可实现局部控制并避免对间变性甲状腺癌患者进行气管造口术。由于所有传统治疗方式均无效,正在开发新的分子靶向疗法来攻克这种普遍致命的疾病,并取得了有希望的结果。