Johan Gagnière, Aurélien Dupré, Denis Pezet, Emmanuel Buc, Department of Digestive and Hepatobiliary Surgery, Centre Hospitalier Universitaire Estaing, 63003 Clermont-Ferrand, France.
World J Gastrointest Surg. 2014 Mar 27;6(3):42-6. doi: 10.4240/wjgs.v6.i3.42.
Mucinous cystic adenoma (MCA) of the pancreas is a rare benign cystic tumor with ovarian-like stroma and lack of communication with the pancreatic ductal system. The ovarian tissue is incorporated from the left gonad within the dorsal pancreas during embryogenesis. Consequently, congenital dorsal agenesis of the pancreas (DAP) cannot be associated with MCA. We report the case of a giant MCA associated with atrophy of the dorsal pancreas mimicking complete DAP. Pancreato-magnetic resonance imaging failed to identify the dorsal pancreas but the absence of diabetes mellitus and compression of the splenic vein with major tributaries rectified the diagnosis of secondary atrophy of the distal pancreas. Unusual proximal location of the cyst in the pancreas may have induced chronic obstruction of both the dorsal pancreatic duct and the splenic vein, with secondary atrophy of the distal pancreas.
胰腺黏液性囊腺瘤（MCA）是一种罕见的良性囊性肿瘤，具有卵巢样基质，与胰管系统无相通。卵巢组织在胚胎发生过程中从背侧胰腺的左侧性腺中融合形成。因此，先天性背侧胰腺发育不全（DAP）不能与 MCA 相关。我们报告了一例与背侧胰腺萎缩相关的巨大 MCA 病例，其表现类似于完全性 DAP。胰腺磁共振成像未能识别背侧胰腺，但无糖尿病和脾静脉及其主要属支受压纠正了远端胰腺继发性萎缩的诊断。囊肿在胰腺中异常的近端位置可能导致背侧胰管和脾静脉慢性阻塞，从而导致远端胰腺继发性萎缩。
