PDF(Pubmed)
Abstract：
Patients with low-grade appendiceal mucinous carcinomas (LAMNs) treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) have a favorable prognosis. However, a subgroup of patients presents a clinically aggressive course with disease progression despite receiving treatment. The purpose of this study is to report the experience of clinically aggressive LAMN patients treated by the same team, and to present a review of the literature. The cases of four patients with clinically aggressive LAMNs were reviewed. Clinical and histopathological characteristics were re-examined. Recurrences and the time of recurrence, as well as the survival time, were recorded. These patients were four men with clinically aggressive LAMNs treated with CRS plus HIPEC. One of them underwent CC-0 surgery, two underwent CC-1 surgery, and one underwent CC-3 surgery. All patients received systemic chemotherapy after surgery. Recurrence was recorded in three of the patients within 4-23 months after the initial treatment. Two of the patients underwent secondary CRS. Three patients died of disease recurrence within 13-23 months, and one is alive with a disease relapse at 49 months after his initial surgery. LAMNs were identified in both the initial specimens and the specimens obtained during reoperation. The prognosis of LAMN patients treated with CRS plus HIPEC is favorable. A small number of patients present a clinically aggressive course that is unresponsive to any treatment. Molecular and genetic studies are required to identify this group of LAMN patients who have an unfavorable prognosis.
摘要：
接受细胞减灭术（CRS）和腹腔热化疗（HIPEC）治疗的低级别阑尾黏液癌（LAMN）患者预后良好。然而,一个亚组患者尽管接受了治疗,但仍表现出疾病进展的临床侵袭性病程.这项研究的目的是报告由同一团队治疗的临床侵袭性LAMN患者的经验，并对文献进行综述。回顾了4例临床侵袭性LAMN患者的病例。重新检查临床和组织病理学特征。复发和复发时间，以及生存时间，被记录下来。这些患者是4名接受CRS加HIPEC治疗的具有临床侵袭性LAMN的男性。其中一人接受了CC-0手术,两人接受了CC-1手术,其中一人接受了CC-3手术。所有患者术后均接受全身化疗。在初次治疗后4-23个月内，记录了其中三名患者的复发情况。其中两名患者接受了继发性CRS。3例患者在13-23个月内死于疾病复发，一个人在初次手术后49个月还活着，疾病复发。在初始标本和再次手术期间获得的标本中均鉴定了LAMN。接受CRS加HIPEC治疗的LAMN患者预后良好。少数患者表现出对任何治疗无反应的临床侵袭性病程。需要进行分子和遗传研究来鉴定这组预后不良的LAMN患者。
