Sada Alaa, Habermann Elizabeth B, Szabo Yamashita Thomas, Thompson Geoffrey B, Lyden Melanie L, Foster Trenton R, Dy Benzon M, Halfdanarson Thorvardur R, Vella Adrian, McKenzie Travis J
From the Department of Surgery (Sada, Szabo Yamashita, Thompson, Lyden, Foster, Dy, McKenzie), Mayo Clinic, Rochester, MN.
Division of Health Care Delivery Research (Habermann), Mayo Clinic, Rochester, MN.
J Am Coll Surg. 2022 Nov 1;235(5):756-763. doi: 10.1097/XCS.0000000000000307. Epub 2022 Oct 17.
The differences between sporadic and multiple endocrine neoplasia type 1 (MEN-1)-associated insulinoma are not well described. Herein, we compared demographics, neoplasm characteristics, presentation, and survival in patients with sporadic vs MEN-1 insulinomas including benign and malignant disease.
A retrospective study identified insulinoma patients. MEN-1 was defined based on genetic testing or clinically in patients with 2 or more primary MEN-1 tumor types.
A total of 311 patients were identified: 84% benign and 16% malignant. The incidence of malignancy was similar (18% vs 16%, MEN-1 vs sporadic, p = 0.76). Within malignant patients, the median (interquartile range) age was 33 (25 ,44) years in MEN-1 vs 54 (41, 70) years in sporadic insulinoma (p = 0.04). There was no difference in sex or tumor size between MEN-1 and sporadic malignant insulinoma (p > 0.05). Of the 260 patients with benign insulinoma, 7% had MEN-1 syndrome. MEN-1 patients presented with insulinoma at a younger age: median (interquartile range) age was 38 (24, 49) years vs 52 (43, 65) years (p < 0.01). Resection of benign insulinoma was performed in 78% of the MEN-1 and 94% of the sporadic group (p = 0.03). Resected benign tumors were larger in the MEN-1 group: 2.0 (1.65, 2.45) cm vs 1.5 (1.2, 2.0) cm, respectively (p = 0.03). Concurrent insulinomas were more common in MEN-1 (17% vs 2%; p < 0.01).
MEN-1 patients present with insulinoma at younger age and have larger benign pancreatic lesions at the time of resection compared with sporadic neoplasms. Younger patients and those with multifocal pancreatic neuroendocrine tumor in the setting of endogenous hyperinsulinism should be evaluated for MEN-1.
散发性胰岛素瘤与1型多发性内分泌肿瘤（MEN - 1）相关的胰岛素瘤之间的差异尚未得到充分描述。在此，我们比较了散发性与MEN - 1胰岛素瘤患者（包括良性和恶性疾病）的人口统计学特征、肿瘤特征、临床表现和生存率。
一项回顾性研究确定了胰岛素瘤患者。MEN - 1基于基因检测或根据患有两种或更多种原发性MEN - 1肿瘤类型的患者的临床情况来定义。
共确定了311例患者：84%为良性，16%为恶性。恶性肿瘤的发生率相似（MEN - 1组与散发性组分别为18%和16%，p = 0.76）。在恶性患者中，MEN - 1组的中位（四分位间距）年龄为33（25，44）岁，而散发性胰岛素瘤组为54（41，70）岁（p = 0.04）。MEN - 1和散发性恶性胰岛素瘤在性别或肿瘤大小方面无差异（p > 0.05）。在260例良性胰岛素瘤患者中，7%患有MEN - 1综合征。MEN - 1患者出现胰岛素瘤的年龄较小：中位（四分位间距）年龄为38（24，49）岁，而散发性组为52（43，65）岁（p < 0.01）。MEN - 1组中78%的患者和散发性组中94%的患者进行了良性胰岛素瘤切除术（p = 0.03）。MEN - 1组切除的良性肿瘤更大：分别为2.0（1.65，2.45）cm和1.5（1.2，2.0）cm（p = 0.03）。同时存在胰岛素瘤在MEN - 1中更常见（17%对2%；p < 0.01）。
与散发性肿瘤相比，MEN - 1患者出现胰岛素瘤的年龄更小，且在切除时良性胰腺病变更大。对于内源性高胰岛素血症情况下的年轻患者和多灶性胰腺神经内分泌肿瘤患者，应评估是否患有MEN - 1。
