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胆囊低分化大细胞神经内分泌癌的罕见病例:一例报告并文献复习

A rare occurrence of a poorly differentiated large cell neuroendocrine carcinoma of the gallbladder: A case report and review of the literature.

作者信息

Shaikh Saamia, Abouzead Logine, Leone Celia, Talishinskiy Toghrul

机构信息

Department of Surgery, St. Joseph's University Medical Center, Paterson, NJ, United States of America.

Department of Surgery, St. Joseph's University Medical Center, Paterson, NJ, United States of America.

出版信息

Int J Surg Case Rep. 2023 Aug;109:108476. doi: 10.1016/j.ijscr.2023.108476. Epub 2023 Jul 7.

Abstract

INTRODUCTION

Neuroendocrine carcinoma (NEC) of the gallbladder is a rare entity with much of the surgical strategy and management mirroring that of adenocarcinoma of the gallbladder. In contrast to gallbladder adenocarcinoma, however, it tends to be a more aggressive and lethal malignancy associated with a short survival after the time of diagnosis. Furthermore, due to its rarity, there are no consensus guidelines for its management.

PRESENTATION OF CASE

We present the case of a 73-year-old female who presented with acute cholecystitis and was found to have a poorly differentiated large cell NEC of the gallbladder after laparoscopic cholecystectomy. She was treated with adjuvant chemotherapy and has shown no evidence of cancer recurrence on three-year follow up.

DISCUSSION

Surgical resection is the cornerstone of curative treatment for gallbladder NEC. Management may include simple cholecystectomy, extended cholecystectomy, radical cholecystectomy, or palliative cholecystectomy. It is unclear whether lymphadenectomy confers a survival benefit. Adjuvant chemotherapy with platinum-based regimens, on the other hand, has been associated with increased survival rates. Radiation, immunotherapy, somatostatin analogs and targeted therapy have also been used for treatment.

CONCLUSION

NEC of the gallbladder is extremely rare, aggressive, and carries a dismal prognosis. Further studies are needed to develop the optimal treatment approach to increase survival rates and establish best practices to manage these patients.

摘要

引言

胆囊神经内分泌癌(NEC)是一种罕见的疾病,其手术策略和管理在很大程度上与胆囊腺癌相似。然而,与胆囊腺癌不同的是,它往往是一种更具侵袭性和致命性的恶性肿瘤,诊断后生存期较短。此外,由于其罕见性,目前尚无关于其管理的共识指南。

病例介绍

我们报告一例73岁女性患者,该患者因急性胆囊炎就诊,在腹腔镜胆囊切除术后被发现患有低分化大细胞型胆囊神经内分泌癌。她接受了辅助化疗,三年随访期间未发现癌症复发迹象。

讨论

手术切除是胆囊神经内分泌癌根治性治疗的基石。治疗方式可能包括单纯胆囊切除术、扩大胆囊切除术、根治性胆囊切除术或姑息性胆囊切除术。目前尚不清楚淋巴结清扫术是否能带来生存获益。另一方面,基于铂类方案的辅助化疗与生存率提高相关。放射治疗、免疫治疗、生长抑素类似物和靶向治疗也已用于治疗。

结论

胆囊神经内分泌癌极为罕见,具有侵袭性,预后不佳。需要进一步研究以制定最佳治疗方法,提高生存率,并确立管理这些患者的最佳实践。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b4d/10382665/17e036519095/gr1.jpg

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