Norton Jeffrey A, Fang Tony D, Jensen Robert T
Department of Surgery, Stanford University Medical Center, Room H-3591, 300 Pasteur Drive, Stanford, CA 94305-5641, USA.
J Natl Compr Canc Netw. 2006 Feb;4(2):148-53. doi: 10.6004/jnccn.2006.0015.
The surgical management of pancreatic endocrine tumors in patients with multiple endocrine neoplasia type 1 remains controversial. Gastrinoma and insulinoma are the 2 most common functional pancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1. Gastrinomas cause gastric acid hypersecretion and peptic ulcer disease that are best managed using proton pump inhibitors. Surgery to remove the gastrinoma in patients with multiple endocrine neoplasia type 1 is seldom curative unless a more extensive Whipple pancreaticoduodenectomy is performed. Because the prognosis is excellent, aggressive resections such as a Whipple procedure are only indicated for large, locally metastatic, advanced tumors. Furthermore, surgery to remove imageable tumors that are 2 cm in diameter is associated with excellent outcomes and decreased probability of liver metastases. Because gastrinomas are commonly multiple and most originate in the duodenum and develop lymph node metastases, the duodenum should be opened and all tumors and lymph nodes excised. Insulinomas cause hypoglycemia that results in neuroglycopenic symptoms. Medical management of the hypoglycemia is less effective than that of the gastric acid hypersecretion. Fortunately, the insulinoma is usually clearly identified using routine pancreatic imaging studies. There is a high likelihood of cure when the insulinoma is excised surgically. However, recurrent hypoglycemia may occur, and careful follow-up is indicated.
1型多发性内分泌腺瘤患者胰腺内分泌肿瘤的手术治疗仍存在争议。胃泌素瘤和胰岛素瘤是1型多发性内分泌腺瘤患者中最常见的两种功能性胰腺神经内分泌肿瘤。胃泌素瘤导致胃酸分泌过多和消化性溃疡疾病，使用质子泵抑制剂治疗效果最佳。1型多发性内分泌腺瘤患者切除胃泌素瘤的手术很少能治愈，除非进行更广泛的惠普尔胰十二指肠切除术。由于预后良好，只有对于大的、局部转移的晚期肿瘤才考虑进行如惠普尔手术这样的积极切除。此外，切除直径2 cm的可成像肿瘤的手术效果良好，肝转移概率降低。由于胃泌素瘤通常为多发，且大多数起源于十二指肠并发生淋巴结转移，应打开十二指肠并切除所有肿瘤和淋巴结。胰岛素瘤导致低血糖，进而产生神经低血糖症状。低血糖的药物治疗不如胃酸分泌过多的治疗有效。幸运的是，通过常规胰腺影像学检查通常能清楚地识别胰岛素瘤。手术切除胰岛素瘤治愈的可能性很高。然而，可能会出现复发性低血糖，需要仔细随访。
