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.

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

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