Pôle des Maladies de l'Appareil Digestif, Service de Gastroentérologie-Pancréatologie, Hôpital Beaujon, AP-HP, Paris, Clichy, France.
Clin Gastroenterol Hepatol. 2010 Feb;8(2):206-12. doi: 10.1016/j.cgh.2009.09.009. Epub 2009 Sep 16.
BACKGROUND & AIMS: Hereditary pancreatitis (HP) is a risk factor for pancreatic adenocarcinoma. We performed a retrospective, multicenter study to characterize and evaluate the frequency of pancreatic intraepithelial neoplasia (PanIN) and to describe the characteristics of fibrosis in pancreatic surgical specimens from patients with HP.
Samples from partial pancreatectomies (n = 13) of patients with HP complications (n = 12; 7 males; mean age, 24 y; 1 patient underwent 2 surgeries over 16 years) were analyzed by histologic and immunohistologic analyses; patients with suspected or proven pancreatic adenocarcinoma were excluded. HP diagnosis was confirmed by analysis of PRSS1 mutations. Dysplastic lesions were described according to the PanIN classification.
Eleven patients were found to have the R122H mutation in PRSS1 and 1 patient was found to have the N29I mutation in PRSS1. Fifty-one PanIN lesions were observed in 10 specimens (77%): PanIN lesions 1a, 1b, 2, and 3 were observed in 8, 5, 8, and 5 specimens, respectively. The median number of PanIN lesions was 3.5 for each specimen. The density of the lesions was 2.6 per 10 cm(2). The size of lesions was greater than 0.5 mm in 55% of the samples. Two patients with PanIN-3 developed pancreatic cancer, 18 months and 44 years after surgery.
PanIN lesions are frequent, severe, and occur early in the course of HP. Among patients with PanINs, 50% had PanIN-3 lesions. Pancreatectomy could be considered as a prophylactic against pancreatic cancer in patients with high-grade dysplasia.
遗传性胰腺炎（HP）是胰腺腺癌的一个危险因素。我们进行了一项回顾性多中心研究，以描述和评估胰腺上皮内瘤变（PanIN）的发生率，并描述 HP 患者胰腺外科标本中纤维化的特征。
对 12 例 HP 并发症患者（7 名男性；平均年龄 24 岁；1 例患者在 16 年内接受了 2 次手术）的部分胰腺切除术（n=13）标本进行组织学和免疫组织化学分析；排除疑似或确诊为胰腺腺癌的患者。HP 诊断通过 PRSS1 突变分析得到确认。根据 PanIN 分类描述发育不良病变。
11 例患者发现 PRSS1 中的 R122H 突变，1 例患者发现 PRSS1 中的 N29I 突变。在 10 个标本中观察到 51 个 PanIN 病变（77%）：8 个标本观察到 PanIN 病变 1a、1b、2 和 3，5 个标本观察到 PanIN 病变 1a、1b、2 和 3，分别为 8、5、8 和 5 个。每个标本的 PanIN 病变中位数为 3.5 个。病变密度为每 10cm2 2.6 个。55%的样本中病变大小大于 0.5mm。2 例 PanIN-3 患者在手术后 18 个月和 44 年发生胰腺癌。
PanIN 病变在 HP 病程中频繁、严重且发生较早。在有 PanIN 的患者中，50%有 PanIN-3 病变。对于高级别发育不良的患者，胰切除术可作为预防胰腺癌的一种手段。
