Köhler Franziska, Matthes Niels, Lock Johan F, Germer Christoph-Thomas, Wiegering Armin
Klinik und Poliklinik für Allgemein‑, Viszeral‑, Transplantations‑, Gefäß- und Kinderchirurgie, Universitätsklinikum Würzburg, Oberdürrbacherstr. 6, 97080, Würzburg, Deutschland.
Comprehensive Cancer Center Mainfranken, Universitätsklinikum Würzburg, Würzburg, Deutschland.
Chirurgie (Heidelb). 2023 Oct;94(10):832-839. doi: 10.1007/s00104-023-01910-0. Epub 2023 Jun 28.
Low-grade appendiceal mucinous neoplasms (LAMN) are rare and are diagnosed in at least 0.13% of appendectomies in Germany, although significant underreporting is likely. Perforation of the tumors can result in abdominal mucinous collections, so-called pseudomyxoma peritonei (PMP). The challenge in the treatment of LAMN is the adequate approach to the incidental finding of these tumors. If a mucinous neoplasm is preoperatively suspected in cases of an acute condition, usually appendicitis, it must be weighed up whether a conservative approach is justifiable or whether immediate appendectomy is necessary. If this is the case, an intraoperative perforation of the appendix must be avoided and the complete abdominal cavity must be inspected for mucin deposits. If conservative treatment is possible, further treatment should take place at a specialized center. If the neoplasm is first found incidentally during surgery, perforation of the appendix should also be avoided and the entire abdominal cavity should be inspected for a PMP. If a PMP is present cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) should be performed in a specialized center. If LAMN are found during the postoperative histological work-up, it should be evaluated whether a perforation was present and mucin collections are noted in the surgical report. In the case of LAMN without evidence of a PMP, appendectomy is the adequate treatment. In cases of intra-abdominal mucinous collections, samples should be taken and further treatment should be performed at a center with sufficient expertise. An ileocecal resection or oncological hemicolectomy is not indicated. After adequate treatment, all patients should receive a follow-up using cross-sectional imaging (preferably magnetic resonance imaging, MRI) and determination of the tumor markers CEA, CA 19-9 and CA 125.
低级别阑尾黏液性肿瘤(LAMN)较为罕见,在德国,至少0.13%的阑尾切除术中会诊断出此类肿瘤,不过很可能存在大量漏报情况。肿瘤穿孔可导致腹腔黏液聚集,即所谓的腹膜假黏液瘤(PMP)。LAMN治疗中的挑战在于如何妥善处理这些肿瘤的偶然发现。如果在急性病症(通常为阑尾炎)病例中术前怀疑存在黏液性肿瘤,就必须权衡保守治疗是否合理,或者是否有必要立即进行阑尾切除术。若是这种情况,必须避免术中阑尾穿孔,且必须对整个腹腔进行检查,查看是否有黏液沉积。如果可行保守治疗,后续治疗应在专业中心进行。如果肿瘤是在手术过程中首次偶然发现,同样应避免阑尾穿孔,并对整个腹腔进行检查,查看是否存在PMP。如果存在PMP,应在专业中心进行细胞减灭术和热灌注腹腔内化疗(HIPEC)。如果在术后组织学检查中发现LAMN,应评估手术报告中是否存在穿孔及黏液聚集情况。对于无PMP证据的LAMN病例,阑尾切除术是恰当的治疗方法。对于腹腔内有黏液聚集的病例,应采集样本,并在具备足够专业知识的中心进行进一步治疗。不建议进行回盲部切除术或肿瘤性半结肠切除术。经过充分治疗后,所有患者都应接受采用断层成像(最好是磁共振成像,MRI)以及测定肿瘤标志物癌胚抗原(CEA)、糖类抗原19-9(CA 19-9)和糖类抗原125(CA 125)的随访。