Department of General, Visceral Transplantation, Vascular and Pediatric Surgery, University Hospital, University of Wuerzburg, Oberduerrbacherstr. 6, 97080, Würzburg, Germany.
Department of Biochemistry and Molecular Biology, University of Wuerzburg, Am Hubland, 97074, Würzburg, Germany.
Langenbecks Arch Surg. 2022 Dec;407(8):3615-3622. doi: 10.1007/s00423-022-02639-w. Epub 2022 Aug 12.
INTRODUCTION: Low-grade appendiceal mucinous neoplasms (LAMN) are semi-malignant tumors of the appendix which are incidentally found in up to 1% of appendectomy specimen. To this day, no valid descriptive analysis on LAMN is available for the German population. METHODS: Data of LAMN (ICD-10: D37.3) were collected from the population-based cancer registries in Germany, provided by the German Center for Cancer Registry Data (Zentrum für Krebsregisterdaten-ZfKD). Data was anonymized and included gender, age at diagnosis, tumor staging according to the TNM-classification, state of residence, information on the performed therapy, and survival data. RESULTS: A total of 612 cases were reported to the ZfKD between 2011 and 2018. A total of 63.07% were female and 36.93% were male. Great inhomogeneity in reporting cases was seen in the federal states of Germany including the fact that some federal states did not report any cases at all. Age distribution showed a mean age of 62.03 years (SD 16.15) at diagnosis. However, data on tumor stage was only available in 24.86% of cases (n = 152). A total of 49.34% of these patients presented with a T4-stage. Likewise, information regarding performed therapy was available in the minority of patients: 269 patients received surgery, 22 did not and for 312 cases no information was available. Twenty-four patients received chemotherapy, 188 did not, and for 400 cases, no information was available. Overall 5-year survival was estimated at 79.52%. Patients below the age of 55 years at time of diagnosis had a significantly higher 5-year survival rate compared to patients above the age of 55 years (85.77% vs. 73.27%). DISCUSSION: In this study, we observed an incidence of LAMN in 0.13% of all appendectomy specimen in 2018. It seems likely that not all cases were reported to the ZfKD; therefore, case numbers may be considered underestimated. Age and gender distribution goes in line with international studies with females being predominantly affected. Especially regarding tumor stage and therapy in depth information cannot be provided through the ZfKD-database. This data analysis emphasizes the need for further studies and the need for setting up a specialized registry for this unique tumor entity to develop guidelines for the appropriate treatment and follow-up.
简介：低级别阑尾黏液性肿瘤（LAMN）是阑尾的半恶性肿瘤，在高达 1%的阑尾切除术标本中偶然发现。迄今为止，德国还没有关于 LAMN 的有效描述性分析。
方法：从德国癌症登记中心（Zentrum für Krebsregisterdaten-ZfKD）提供的基于人群的癌症登记处收集了 LAMN（ICD-10：D37.3）的数据。数据经过匿名处理，包括性别、诊断时的年龄、根据 TNM 分类的肿瘤分期、居住地、治疗信息和生存数据。
结果：2011 年至 2018 年期间，ZfKD 共报告了 612 例病例。女性占 63.07%，男性占 36.93%。德国各联邦州的病例报告存在很大差异，包括一些联邦州根本没有报告任何病例。年龄分布显示诊断时的平均年龄为 62.03 岁（标准差 16.15）。然而，只有 24.86%的病例（n=152）有肿瘤分期数据。这些患者中，49.34%为 T4 期。同样，关于所行治疗的信息在少数患者中可用：269 例患者接受了手术，22 例未接受，312 例患者无信息。24 例患者接受了化疗，188 例未接受，400 例患者无信息。总体 5 年生存率估计为 79.52%。诊断时年龄低于 55 岁的患者 5 年生存率明显高于年龄大于 55 岁的患者（85.77% vs. 73.27%）。
讨论：在这项研究中，我们观察到 2018 年所有阑尾切除术标本中 LAMN 的发病率为 0.13%。似乎并非所有病例都向 ZfKD 报告；因此，病例数可能被低估。年龄和性别分布与国际研究一致，女性受影响更为普遍。特别是关于肿瘤分期和治疗的深入信息，ZfKD 数据库无法提供。这项数据分析强调了进一步研究的必要性，以及为这个独特的肿瘤实体建立专门登记处的必要性，以制定适当治疗和随访的指南。
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