House Michael G, Yeo Charles J, Schulick Richard D
Department of Surgery, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.
Ann Surg Oncol. 2002 Nov;9(9):869-74. doi: 10.1007/BF02557523.
Somatostatinomas involving the gastrointestinal tract are extremely rare neoplasms that typically present with indolent, nonspecific symptoms in the absence of systemic neuroendocrine manifestations that characterize the somatostatinoma syndrome. Because of a relatively large size at the time of presentation (average diameter of 5 cm) and common location within the head of the pancreas, the Whipple procedure (pancreaticoduodenectomy) serves as the predominant modality for curative and palliative surgical approaches.
Two cases of somatostatinoma involving the minor duodenal papilla with concomitant pancreatic divisum were reviewed, with a general overview of this unique islet cell tumor.
Unlike typical somatostatinomas, these two tumors were subcentimeter in size but were associated with synchronous regional metastasis.
Somatostatinomas are often associated with regional and/or portal metastases at the time of diagnosis, and only 60% to 70% of surgical cases result in complete tumor resection. Predictors of an unfavorable prognosis include size >3 cm, poor cytological differentiation, regional and/or portal metastasis, and incomplete surgical resection. Even in the presence of synchronous metastases, the 5-year overall survival for patients with somatostatinoma is approximately 40%. Currently, there are no clinical trials demonstrating significant improvement in survival with the use of adjuvant therapy.
累及胃肠道的生长抑素瘤是极其罕见的肿瘤，通常表现为隐匿性、非特异性症状，且无生长抑素瘤综合征所特有的全身神经内分泌表现。由于其在就诊时相对较大（平均直径5厘米）且常见于胰头内，惠普尔手术（胰十二指肠切除术）是根治性和姑息性手术治疗的主要方式。
回顾了2例累及十二指肠小乳头并伴有胰腺分裂症的生长抑素瘤病例，并对这种独特的胰岛细胞瘤进行了概述。
与典型的生长抑素瘤不同，这两个肿瘤大小小于1厘米，但伴有同步区域转移。
生长抑素瘤在诊断时常伴有区域和/或门静脉转移，只有60%至70%的手术病例能实现肿瘤完全切除。预后不良的预测因素包括肿瘤大小>3厘米、细胞分化差、区域和/或门静脉转移以及手术切除不完全。即使存在同步转移，生长抑素瘤患者的5年总生存率约为40%。目前，尚无临床试验表明辅助治疗能显著提高生存率。
