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巨大胆囊肿瘤,罕见癌症——病例报告及文献简短综述

Giant Gallbladder Tumor, Unusual Cancer-Case Report and Short Review of Literature.

作者信息

Constantin Adrian, Achim Florin, Turcu Tudor, Birceanu Adelina, Evsei Anca, Socea Bogdan, Predescu Dragos

机构信息

Department of Esophageal and General Surgery, "Saint Mary" Clinical Hospital Bucharest, 011192 Bucharest, Romania.

Faculty of Medicine, Carol Davila University of Medicine and Pharmacy Bucharest, 050474 Bucharest, Romania.

出版信息

Diagnostics (Basel). 2023 Jan 5;13(2):194. doi: 10.3390/diagnostics13020194.

Abstract

BACKGROUND

Giant gallbladder is an uncommon condition that can result from a benign pathology and rarely presents with malignancy. Intracholecystic papillary-tubular neoplasm (ICPN) is a relatively new entity first described by V. Adsay in 2012 and included in the World Health Classification of Digestive System Tumours in 2019. Intracholecystic papillary-tubular neoplasm is a preinvasive lesion with an incidence of around 1% that may present as four histologic subtypes-biliary, gastric, intestinal, or oncocytic-of which the biliary subtype has the highest risk of associated invasive cancer. Although invasive carcinoma is present in about 50% of cases of ICPN, these patients have a significantly better prognosis than those with usual gallbladder cancer, suggesting that the entities may have distinct biological signatures.

CASE REPORT

A 77-year-old female presented to the hospital with progressive swelling in the right hemiabdomen, a loss of appetite, and weight loss. MRI highlighted a giant abdominal tumor located in the right hypochondrium and right abdominal flank with liver invasion (segment V). Preoperatively, a gallbladder 25 × 17 cm in size was noted, and the patient underwent radical cholecystectomy. It was surprising to find such a giant malignant gallbladder tumor, diagnosed as invasive poorly cohesive carcinoma associated with ICPN.

DISCUSSION

A megacholecyst is a rare discovery. Although most often found in benign pathologies, giant gallbladder cancer can be considered. The neoplastic features and the loco-regional extension of the tumor must be evaluated by imaging scans. Few cases of giant benign gallbladder have been reported in the literature; however, this appeared to be the largest resectable gallbladder carcinoma reported to date according to the literature.

CONCLUSION

The stage of gallbladder neoplasia is not correlated with the size of the gallbladder. Regardless of tumor size, the prognosis seems to be directly related to the stage, morphology, and resectability.

摘要

背景

巨大胆囊是一种罕见的情况,可由良性病变引起,很少伴有恶性肿瘤。胆囊内乳头状管状肿瘤(ICPN)是一种相对较新的实体,于2012年由V. Adsay首次描述,并于2019年被纳入《世界卫生组织消化系统肿瘤分类》。胆囊内乳头状管状肿瘤是一种浸润前病变,发病率约为1%,可表现为四种组织学亚型——胆管型、胃型、肠型或嗜酸性细胞型,其中胆管型与浸润性癌相关的风险最高。尽管约50%的ICPN病例存在浸润性癌,但这些患者的预后明显优于普通胆囊癌患者,这表明这些实体可能具有不同的生物学特征。

病例报告

一名77岁女性因右半腹进行性肿胀、食欲不振和体重减轻入院。磁共振成像(MRI)显示一个巨大的腹部肿瘤位于右季肋部和右腹外侧,侵犯肝脏(Ⅴ段)。术前发现胆囊大小为25×17cm,患者接受了根治性胆囊切除术。令人惊讶的是,发现了这样一个巨大的恶性胆囊肿瘤,诊断为与ICPN相关的浸润性低黏附性癌。

讨论

巨大胆囊是一种罕见的发现。尽管最常见于良性病变,但也应考虑巨大胆囊癌。肿瘤的特征和局部扩展必须通过影像学检查进行评估。文献中报道的巨大良性胆囊病例很少;然而,根据文献,这似乎是迄今为止报道的最大的可切除胆囊癌。

结论

胆囊肿瘤的分期与胆囊大小无关。无论肿瘤大小如何,预后似乎直接与分期、形态和可切除性相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2762/9857600/8efd104e9068/diagnostics-13-00194-g001.jpg

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