Department of Medicine, University of Verona, Italy. mariavittoria.davi @ ospedaleuniverona.it
Neuroendocrinology. 2011;94(1):58-65. doi: 10.1159/000326164. Epub 2011 Apr 5.
To assess presentation and outcome of pancreaticoduodenal endocrine tumors (PDETs) in a single center series of multiple endocrine neoplasia type 1 (MEN1) patients.
Retrospective analysis of prospectively collected data of MEN1 patients observed at the University of Verona.
Thirty-one MEN1 patients had PDETs, including 16 nonfunctioning (NF), 6 insulinomas and 9 Zollinger-Ellison syndrome (ZES). In 16 of these patients (52%), PDET was the manifestation which led to the diagnosis of MEN1; among this group, 15 patients (94%) previously had unidentified primary hyperparathyroidism (PHPT), which was asymptomatic in 9 cases (60%). Of the 31 patients, 19 (61%) underwent curativesurgery and 13 (68%, 7 NF-PDETs, 4 insulinomas and 2 ZES) were disease-free after a median follow-up of 3 years (range: 0.5-15). One patient had debulking surgery with stable disease after 2 years of follow-up. Eight patients with NF-PDETs ≤20 mm and 2 with ZES, treated with a conservative approach, showed stable disease. One patient with insulinoma was lost to follow-up.
PDET may be the manifestation that leads to MEN1 diagnosis since the almost constant presence of PHPT is very often unrecognized or considered sporadic. Conversely, the presence of PDETs should be looked for in all patients presenting PHPT, even if asymptomatic, particularly before age 50. Surgery may be curative in the majority of insulinomas and can prolong disease-free survival in NF-PDET, but is not proven to be effective in ZES. A conservative approach can be safely reserved for patients with NF-PDETs ≤20 mm.
评估单一中心系列多发性内分泌肿瘤 1 型（MEN1）患者胰腺十二指肠内分泌肿瘤（PDETs）的表现和结局。
回顾性分析在维罗纳大学观察到的 MEN1 患者前瞻性收集的数据。
31 例 MEN1 患者存在 PDETs，包括 16 例无功能（NF）、6 例胰岛素瘤和 9 例 Zollinger-Ellison 综合征（ZES）。在这些患者中的 16 例（52%），PDET 是导致 MEN1 诊断的表现；在这一组中，15 例（94%）之前存在未识别的原发性甲状旁腺功能亢进症（PHPT），其中 9 例（60%）无症状。在 31 例患者中，19 例（61%）接受了根治性手术，13 例（68%，7 例 NF-PDETs、4 例胰岛素瘤和 2 例 ZES）在中位随访 3 年后无疾病（范围：0.5-15）。1 例患者在 2 年随访后行肿瘤减灭术，病情稳定。8 例 NF-PDETs ≤20mm 和 2 例 ZES 患者采用保守治疗，病情稳定。1 例胰岛素瘤患者失访。
由于 PHPT 的存在几乎是恒定的，且常常未被识别或被认为是散发性的，因此 PDET 可能是导致 MEN1 诊断的表现。相反，在所有出现 PHPT 的患者中都应寻找 PDETs 的存在，即使无症状，特别是在 50 岁之前。手术在大多数胰岛素瘤中可能是治愈性的，并能延长 NF-PDET 的无病生存，但在 ZES 中尚未证明有效。对于 NF-PDETs ≤20mm 的患者，可以安全地保留保守治疗方法。
