关键词: cytoreductive surgery hyperthermic intraperitoneal chemotherapy low-grade appendiceal mucinous neoplasms oncology pseudomyxoma peritonei

来  源:   DOI:10.3389/fonc.2024.1396265   PDF(Pubmed)

Abstract:
Low-grade appendiceal mucinous neoplasms (LAMNs) are rare and heterogeneous diseases that, despite their increased incidence, are well differentiated, tend to be painless, and histologically lack distinctive invasive features without infiltrative growth, destructive infiltration, or associated pro-fibroproliferative responses. However, the biological behaviour of these tumours is difficult to determine preoperatively or intraoperatively, and the possibility of rupture puts patients at risk for peritoneal pseudomucinous neoplasms (PMPs).Patients with low-grade appendiceal mucinous tumours and peritoneal pseudomucinous tumours experience slow disease progression and are incurable and have a high risk of recurrence, morbidity, and ultimately death, despite the reported 5- and 10-year survival rates of 50-86% and 45-68%, respectively. In this article, we report the case of a 80-year-old male with a giant low-grade appendiceal mucinous tumour associated with a peritoneal pseudomucinous tumour, and discuss the diagnostic and management strategies for giant low-grade appendiceal mucinous tumours in the context of a literature review.
摘要:
低级别阑尾黏液性肿瘤(LAMNs)是罕见且异质性的疾病,尽管发病率增加,有很好的区别,往往是无痛的,组织学上缺乏独特的侵入性特征,没有浸润性生长,破坏性渗透,或相关的促纤维增生反应。然而,这些肿瘤的生物学行为在术前或术中难以确定,破裂的可能性使患者面临腹膜假黏液性肿瘤(PMPs)的风险。低级别阑尾黏液性肿瘤和腹膜假黏液性肿瘤患者病情进展缓慢,无法治愈,复发风险高,发病率,最终死亡,尽管报告的5年和10年生存率为50-86%和45-68%,分别。在这篇文章中,我们报告了一个80岁的男性,患有一个巨大的低级别阑尾粘液性肿瘤,与腹膜假粘液性肿瘤相关,并结合文献综述讨论巨大的低级别阑尾黏液性肿瘤的诊断和治疗策略。
公众号