The German guideline for low-grade appendiceal mucinous neoplasms (LAMN) and pseudomyxoma peritonei (PMP) offers comprehensive recommendations for diagnosis, treatment, and surveillance of these rare tumours. Developed by the German Society of General and Visceral Surgery (DGAV) alongside 14 other medical societies or task groups, this S2k-guideline addresses the need for standardised care in the absence of high-quality randomized controlled trials due to the rarity of LAMN and PMP. The guideline covers classification and staging of LAMN according to WHO and TNM systems, emphasising histological analysis and surgical protocols aimed at preventing intra-abdominal perforation. Diagnostic recommendations include imaging (MRI or CT) and preoperative tumour marker assessment, along with screening colonoscopies to rule out synchronous colorectal malignancies in specific age groups. Therapeutic guidelines focus on the importance of treatment in specialised centres with expertise in cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). CRS combined with HIPEC is recommended for patients with PMP, with an emphasis on multidisciplinary team involvement and psycho-oncological support. The guideline outlines post-treatment surveillance, recommending six-monthly imaging and tumour marker evaluations for five years. It highlights the importance of considering fertility preservation in patients undergoing cytoreductive surgery and HIPEC. This consensus-based guideline aims to enhance the quality and consistency of care for patients with LAMN and PMP, offering a structured approach despite limited clinical trial data.

德国低级别阑尾粘液瘤（LAMN）和腹膜假性粘液瘤（PMP）指南为这些罕见肿瘤的诊断、治疗和监测提供了全面的建议。该S2k指南由德国普通与内脏外科学会（DGAV）与其他14个医学会或工作组共同制定，由于LAMN和PMP的罕见性，在缺乏高质量随机对照试验的情况下，该指南满足了对标准化治疗的需求。该指南根据世界卫生组织和TNM系统对LAMN进行分类和分期，强调组织学分析和旨在预防腹腔穿孔的手术方案。诊断建议包括成像（MRI或CT）和术前肿瘤标记物评估，以及筛查结肠镜检查，以排除特定年龄组的同步结直肠恶性肿瘤。治疗指南重点强调了在具有细胞减毒手术（CRS）和腹腔内热化疗（HIPEC）专长的专科中心进行治疗的重要性。建议对PMP患者进行CRS联合HIPEC治疗，并强调多学科团队的参与和肿瘤心理支持。该指南概述了治疗后的监测，建议在五年内每六个月进行一次成像和肿瘤标志物评估。它强调了对接受细胞减灭术和HIPEC的患者考虑保留生育能力的重要性。尽管临床试验数据有限，但这一基于共识的指南旨在提高LAMN和PMP患者的治疗质量和一致性，并提供了一种结构化方法。