Umetsu Sarah E, Shafizadeh Nafis, Kakar Sanjay
Department of Pathology, University of California, San Francisco, San Francisco, CA, USA 94143.
Kaiser Permanente, Woodland Hills, CA 91367, USA.
Hum Pathol. 2017 Nov;69:81-89. doi: 10.1016/j.humpath.2017.09.005. Epub 2017 Sep 29.
The grading and staging of appendiceal mucinous neoplasms is challenging and fraught with terminology problems, but has critical prognostic and therapeutic implications. We utilized a small case series to examine the grading and staging systems of appendiceal mucinous neoplasms and outline the evidence for the new systems proposed in the upcoming 8th edition of the American Joint Committee on Cancer (AJCC) Staging Manual. We reviewed 33 cases of appendiceal mucinous neoplasms with available clinical follow-up data, 6 of which were widely disseminated in the peritoneum. An additional 4 cases with disseminated peritoneal involvement were also reviewed. A detailed review of the literature was performed with an emphasis on features associated with disease recurrence and correlation of grade with outcome. Recurrence was not seen in 64 low-grade appendiceal mucinous neoplasms (LAMNs) confined to the muscularis propria in our series (n=21) or in the literature (n=43). Of cases of LAMN with neoplastic epithelium present beyond the muscularis propria, 64% (57/89) had peritoneal disease at the time of diagnosis or follow-up. A majority of studies of disseminated appendiceal mucinous neoplasms showed significant five-year survival differences using a three-tier grading scheme. Thus, LAMNs confined to the muscularis propria are best considered as in situ tumors, as these are cured with complete excision. A three-tier system has prognostic significance and should be used for grading of disseminated appendiceal mucinous neoplasms. The conclusions of this case series and literature review provide evidence to support the changes proposed in the 8th edition of the AJCC Staging Manual.
阑尾黏液性肿瘤的分级和分期颇具挑战性且存在诸多术语问题，但对预后和治疗具有关键意义。我们利用一个小病例系列来研究阑尾黏液性肿瘤的分级和分期系统，并概述即将出版的第8版美国癌症联合委员会（AJCC）分期手册中提出的新系统的依据。我们回顾了33例有可用临床随访数据的阑尾黏液性肿瘤病例，其中6例在腹膜广泛播散。另外还回顾了4例有腹膜播散累及的病例。对文献进行了详细回顾，重点关注与疾病复发相关的特征以及分级与预后的相关性。在我们的系列研究（n = 21）或文献（n = 43）中，64例局限于固有肌层的低级别阑尾黏液性肿瘤（LAMN）均未出现复发。在肿瘤上皮超出固有肌层的LAMN病例中，64%（57/89）在诊断或随访时出现腹膜疾病。大多数关于播散性阑尾黏液性肿瘤的研究表明，使用三级分级方案时五年生存率存在显著差异。因此，局限于固有肌层的LAMN最好被视为原位肿瘤，因为完整切除可治愈这些肿瘤。三级系统具有预后意义，应被用于播散性阑尾黏液性肿瘤的分级。这个病例系列和文献回顾的结论为支持AJCC分期手册第8版中提出的变化提供了证据。
