Michalinos Adamantios, Alexandrou Parakevi, Papalambros Alexandros, Oikonomou Dimitrios, Sakellariou Stratigoula, Baliou Evangelia, Alexandrou Andreas, Schizas Dimitrios, Felekouras Evangelos
First Department of Surgery, National and Kapodistrian University of Athens, Ag. Thoma 17 Str., Goudi, Athens, Greece.
First Department of Pathology, National and Kapodistrian University of Athens, Athens, Greece.
World J Surg Oncol. 2016 Aug 2;14(1):202. doi: 10.1186/s12957-016-0962-x.
Intracholecystic papillary-tubular neoplasms are rare precursor lesions of gallbladder cancer. They were proposed as a separate pathologic entity in 2012 by Adsay et al. for the unification of a variety of mass-forming precursor lesions including papillary adenomas, tubulopapillary adenomas, intestinal adenomas, and others. They are considered homologous to intrapapillary mucinous neoplasms of the pancreas and intrabiliary papillary neoplasms of the common bile duct. In contrast with the commoner flat-type precursor gallbladder cancer lesions, they follow a more indolent clinical course and probably different genetic pathways to carcinogenesis. They are largely uninvestigated with only a handful of studies providing biological and clinical information. Choledochal cysts are dilation of the common bile duct. Diagnosis is usually established during childhood, and only a minority of patients are diagnosed at adulthood. They are of major clinical importance as they are known predisposing factors for biliary carcinogenesis.
The current report describes a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. The patient underwent excision of the extrahepatic biliary tree for a Todani I choledochal cyst, and histological examination of the specimen revealed an intracholecystic papillary-tubular neoplasm of the gallbladder. Authors describe diagnostic and clinical course of the patient alongside clinical and biological characteristics of these rare lesions.
To the best of our knowledge, this is the first report of a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. Those rare lesions shed light on different forms of gallbladder cancer carcinogenesis and its relationship with choledochal cysts and cholestasis.
胆囊内乳头管状肿瘤是胆囊癌罕见的前驱病变。2012年，阿德赛等人将其作为一种单独的病理实体提出，以统一包括乳头状腺瘤、微乳头管状腺瘤、肠腺瘤等多种形成肿块的前驱病变。它们被认为与胰腺内乳头状黏液性肿瘤及胆总管内胆管乳头状肿瘤同源。与更常见的扁平型前驱胆囊癌病变不同，它们的临床病程更为惰性，可能遵循不同的致癌遗传途径。目前对它们的研究较少，仅有少数研究提供了生物学和临床信息。胆总管囊肿是胆总管的扩张。诊断通常在儿童期确立，仅有少数患者在成年期被诊断。它们具有重要的临床意义，因为已知是胆管癌发生的易感因素。
本报告描述了一名同时诊断为胆总管囊肿和胆囊内乳头管状肿瘤的患者。该患者因托达尼I型胆总管囊肿接受了肝外胆管树切除术，标本的组织学检查显示为胆囊内乳头管状肿瘤。作者描述了该患者的诊断和临床病程以及这些罕见病变的临床和生物学特征。
据我们所知，这是首例同时诊断为胆总管囊肿和胆囊内乳头管状肿瘤的患者报告。这些罕见病变为胆囊癌致癌的不同形式及其与胆总管囊肿和胆汁淤积的关系提供了线索。
