Introduction

Anorectal neuroendocrine carcinomas (NECs) are uncommon malignancies with poor prognosis. Consensus guidelines exist for treating extrapulmonary NEC. However, limited data is available to guide treatment for anorectal NEC. In this study, we sought to review the clinical characteristics and outcomes of patients with NEC of the rectum and/or anus at Mayo Clinic.

Patients and Methods

This is a retrospective study of all patients with the diagnosis of NEC of the anus and/or rectum treated across Mayo Clinic sites since 2000. Baseline patient characteristics, tumor pathology, imaging profiles, treatment strategies utilized, and survival outcomes were analyzed. Kaplan-Meier analysis was used with a significance level of P < .05.

Results

The study included a total of 38 patients with primary NEC of the anus and/or rectum. The median age at diagnosis was 55.5 years. The median follow-up was 18.8 months. Fifteen patients had locoregional disease (LRD) at diagnosis. The remaining 23 had metastatic disease. Overall survival was significantly shorter in patients with LRD compared with those with metastatic disease at diagnosis (18.1 vs. 13.8 months; P = .039). The majority (n = 11) of patients with LRD were treated with concurrent chemoradiation therapy, and 10 underwent surgical resection of the primary tumor. The majority (13/15) of patients with LRD progressed, with the majority (11/15) of progressions being distant. The median progression-free survival for patients with LRD was 5.7 months (1-year progression-free survival, 26.7%).

Conclusion

Anorectal NEC is an aggressive malignancy with poor prognosis requiring multidisciplinary discussion. In addition, the systemic nature of anorectal NEC with distant recurrences in LRD and poor outcomes in metastatic disease emphasizes the need to further develop better systemic treatment options that can potentially improve outcomes in NEC.

中文翻译：

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肛门和直肠神经内分泌癌：患者特征和治疗选择

介绍

肛门直肠神经内分泌癌 (NEC) 是一种罕见的恶性肿瘤，预后较差。存在治疗肺外 NEC 的共识指南。然而，可用于指导肛门直肠 NEC 治疗的数据有限。在这项研究中，我们试图回顾梅奥诊所直肠和/或肛门 NEC 患者的临床特征和结果。

患者和方法

这是对自 2000 年以来在 Mayo Clinic 站点接受治疗的所有诊断为肛门和/或直肠 NEC 的患者进行的回顾性研究。分析了基线患者特征、肿瘤病理、影像学特征、使用的治疗策略和生存结果。使用 Kaplan-Meier 分析，显着性水平为P  < .05。

结果

该研究共包括 38 名肛门和/或直肠原发性 NEC 患者。诊断时的中位年龄为 55.5 岁。中位随访时间为 18.8 个月。15 名患者在诊断时患有局部区域疾病 (LRD)。其余 23 人患有转移性疾病。与诊断时转移性疾病患者相比，LRD 患者的总生存期显着缩短（18.1 个月与 13.8 个月；P  = .039）。大多数（n = 11）LRD 患者接受了同步放化疗，10 名接受了原发肿瘤的手术切除。大多数 (13/15) LRD 患者出现进展，大多数 (11/15) 进展为远处。LRD 患者的中位无进展生存期为 5.7 个月（1 年无进展生存期，26.7%）。

结论

肛门直肠 NEC 是一种侵袭性恶性肿瘤，预后不良，需要多学科讨论。此外，肛门直肠 NEC 的全身性质与 LRD 远处复发和转移性疾病的不良预后强调需要进一步开发更好的全身治疗方案，以潜在改善 NEC 的预后。