Comaru-Schally Ana Maria, Schally Andrew V
Endocrine, Polypeptide and Cancer Institute, Veterans Affairs Medical Center, Department of Medicine, Tulane University School of Medicine, New Orleans, LA 70112, USA.
Int J Oncol. 2005 Feb;26(2):301-9.
Carcinoid tumors were first described more than a century ago, but the treatment of patients with advanced disease remains a challenge to physicians. The etiology of carcinoid tumors, the biologic determinants of the growth of these malignancies, as well as the high frequency of multiple carcinoid and/or non-carcinoid tumors in patients with this disease also remain to be elucidated. A 5-decade analysis of 13,715 carcinoid tumors in the USA showed that distant metastases were demonstrated at the time of diagnosis in 12.9% of patients with this neoplasia. The overall 5-year survival rate for all patients with carcinoids regardless of the site, was reported to be 67.2%. The prognosis of patients with early stage disease is good and surgical resection is the standard form of treatment. The resection of local or regional metastases can result in cure for some cases. However, patients with metastatic dissemination have poor outcomes since chemotherapy is generally ineffective. Surgical resection of isolated hepatic metastases, surgical hepatic artery ligation or embolization produce responses in selected patients. Radiation therapy may ease the pain of bone metastases. The administration of long acting analogs of somatostatin can control the symptoms of diarrhea and flushing in patients with the malignant carcinoid syndrome. However, a complete regression of metastatic carcinoid tumors following the administration of somatostatin analog octreotide has been reported so far in only 3 cases. Other modalities of treatment, including liver transplantation and the administration of radiolabeled somatostatin analogs have likewise been applied in patients with advanced disease. It is expected that advances in proteomics research will contribute to our understanding of the mechanisms of diseases and aid in designing new drugs.
类癌瘤在一个多世纪前就首次被描述，但晚期疾病患者的治疗对医生来说仍然是一项挑战。类癌瘤的病因、这些恶性肿瘤生长的生物学决定因素，以及该疾病患者中多发类癌和/或非类癌肿瘤的高发生率仍有待阐明。对美国13715例类癌瘤进行的一项长达50年的分析表明，在诊断时，12.9%的该肿瘤患者出现远处转移。据报道，所有类癌患者无论肿瘤部位如何，总体5年生存率为67.2%。早期疾病患者的预后良好，手术切除是标准的治疗方式。局部或区域转移灶的切除在某些情况下可实现治愈。然而，发生转移播散的患者预后较差，因为化疗通常无效。孤立性肝转移灶的手术切除、肝动脉结扎术或栓塞术在部分患者中有效。放射治疗可缓解骨转移引起的疼痛。长效生长抑素类似物的给药可控制恶性类癌综合征患者的腹泻和潮红症状。然而，迄今为止，仅报道了3例在使用生长抑素类似物奥曲肽后转移性类癌瘤完全消退的病例。其他治疗方式，包括肝移植和放射性标记生长抑素类似物的给药，也同样应用于晚期疾病患者。预计蛋白质组学研究的进展将有助于我们理解疾病机制并有助于设计新药。
