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阑尾黏液性肿瘤起源的腹膜假黏液瘤:一例报告并文献复习

Pseudomyxoma peritonei of appendiceal mucinous neoplasm origin: A case report and review of literature.

作者信息

Mousa Ahmed Hafez, Nukaly Houriah Yasir, Samman Rayyan Rafat, Fuadah Samratul, Saddiq Bushra Wadi Bin, Alshowaikhat Shahad Jamal, Khalid Islam

机构信息

Department of Neurosurgery, Graduate Medical Education Department (GME), Mohammed Bin Rashid University of Medicine and Health Sciences (MBRU), Dubai Health, Dubai, United Arab Emirates.

College of Medicine and Surgery, Batterjee Medical College, Jeddah, Saudi Arabia.

出版信息

Radiol Case Rep. 2024 Sep 28;19(12):6565-6573. doi: 10.1016/j.radcr.2024.08.158. eCollection 2024 Dec.

DOI:10.1016/j.radcr.2024.08.158
PMID:39391033
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11465061/
Abstract

Appendiceal mucinous neoplasms, a rarity comprising less than 1% of all cancers, present intricate challenges in clinical management, and their incidence is on the rise. Notably, these neoplasms tend to metastasize intraperitoneally, leading to peritoneal carcinomatosis and concurrent accumulation of mucinous material, resulting in pseudomyxoma peritonei. Due to its spectrum of presentation, the classification of the appendiceal mucinous neoplasms remains a controversial subject with a range of management from a simple appendicectomy to a complex hyperthermic intraperitoneal chemotherapy (HIPEC). A 42-year-old Chadian male presented to the hospital with a sudden onset of right lower abdominal pain radiating to the inguinal region for 24 hours, associated with nausea and vomiting. The abdomen was distended and ascitic. Laboratory investigations revealed anemia, leukocytosis, hypernatremia, hypokalemia, elevated ESR, high CEA marker, and normal CA19-9. An abdominopelvic CT with contrast demonstrated extensive ascites and cystic masses in the liver, and pancreas with soft tissue thickening of the cecum; however, the appendix is not well-delineated. Patient was managed with chemotherapy and HIPEC followed by removal of all the affected parts. Nodules of the peritoneum and liver were submitted for histopathological analysis and a final diagnosis of pseudomyxoma peritonei of primary appendicular origin was established. This case highlights a case of extensive pseudomyxoma peritonei of appendicular origin managed aggressively by HIPEC and multiple resections of the involved organs. Prognosis of such a case is determined by the grade of the appendiceal tumor and the extent of invasion.

摘要

阑尾黏液性肿瘤是一种罕见病,在所有癌症中占比不到1%,给临床管理带来了复杂的挑战,且其发病率呈上升趋势。值得注意的是,这些肿瘤倾向于腹膜内转移,导致腹膜癌病并伴有黏液物质的蓄积,从而引发腹膜假黏液瘤。由于其临床表现多样,阑尾黏液性肿瘤的分类仍然是一个有争议的话题,其治疗范围从简单的阑尾切除术到复杂的腹腔内热灌注化疗(HIPEC)。一名42岁的乍得男性因突发右下腹痛并向腹股沟区放射24小时入院,伴有恶心和呕吐。腹部膨隆且有腹水。实验室检查显示贫血、白细胞增多、高钠血症、低钾血症、血沉升高、癌胚抗原(CEA)标志物升高,而糖类抗原19-9(CA19-9)正常。腹部盆腔增强CT显示大量腹水以及肝脏、胰腺的囊性肿块,盲肠软组织增厚;然而,阑尾显示不清。患者接受了化疗和HIPEC治疗,随后切除了所有受累部位。将腹膜和肝脏的结节送去做组织病理学分析,最终确诊为原发性阑尾来源的腹膜假黏液瘤。本病例突出了一例阑尾来源的广泛性腹膜假黏液瘤,通过HIPEC和对受累器官的多次切除进行积极治疗。此类病例的预后取决于阑尾肿瘤的分级和浸润程度。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be14/11465061/58606721326f/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be14/11465061/58606721326f/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be14/11465061/58606721326f/gr1.jpg

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本文引用的文献

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Pseudomyxoma peritonei arising from low-grade appendiceal mucinous neoplasm: A rare case report.源于低级别阑尾黏液性肿瘤的腹膜假黏液瘤:一例罕见病例报告。
Asian J Surg. 2024 Apr;47(4):1789-1790. doi: 10.1016/j.asjsur.2023.12.114. Epub 2024 Jan 5.
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High-grade appendiceal mucinous neoplasm presenting as renal colic; a case report and review of literature.表现为肾绞痛的高级别阑尾黏液性肿瘤:一例病例报告并文献复习
J Surg Case Rep. 2023 Oct 17;2023(10):rjad567. doi: 10.1093/jscr/rjad567. eCollection 2023 Oct.
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Contrast-enhanced ultrasonography findings of LAMNs with peritoneal and splenic metastases: a case report and literature review.
淋巴管肌瘤病伴腹膜和脾转移的超声造影表现:1例报告并文献复习
Front Oncol. 2023 Sep 25;13:1238042. doi: 10.3389/fonc.2023.1238042. eCollection 2023.
4
Low-grade appendiceal mucinous neoplasm encountered during risk-reducing salpingo-oophorectomy: A case of laparoscopic surgery.在降低风险的输卵管卵巢切除术期间遇到的低级别阑尾黏液性肿瘤:腹腔镜手术一例。
J Obstet Gynaecol Res. 2023 Dec;49(12):2975-2978. doi: 10.1111/jog.15802. Epub 2023 Sep 28.
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APPENDICEAL MUCINOUS NEOPLASMS: A CASE REPORT AND REVIEW OF LITERATURE.阑尾黏液性肿瘤:一例病例报告及文献综述
Gastroenterol Nurs. 2024;47(2):152-155. doi: 10.1097/SGA.0000000000000778. Epub 2023 Sep 20.
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A Case of Low-Grade Appendiceal Mucinous Neoplasm That Led to Surgery After 12 Years of No Treatment.一例低度恶性阑尾黏液性肿瘤未经治疗12年后接受手术治疗的病例。
Cureus. 2023 Aug 6;15(8):e43024. doi: 10.7759/cureus.43024. eCollection 2023 Aug.
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Case report: A rare case of synchronous mucinous neoplasms of the renal pelvis and the appendix.病例报告:一例罕见的肾盂和阑尾同时发生黏液性肿瘤的病例。
Front Oncol. 2023 Jun 26;13:1213631. doi: 10.3389/fonc.2023.1213631. eCollection 2023.
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