Lim Li Xian, De Robles Marie Shella, Winn Robert Duncan, Hart Kimberly Anne
Department of General Surgery, Level 2, Block B, Wollongong Hospital, Loftus Street, Wollongong, NSW 2521, Australia.
Department of Pathology, Level 7, Block C, Wollongong Hospital, 348-352 Crown Street, NSW 2500, Australia.
Int J Surg Case Rep. 2020;71:240-243. doi: 10.1016/j.ijscr.2020.04.065. Epub 2020 May 11.
Mixed adeno-neuroendocrine carcinoma (MANEC) is a rare disease, and much of the available literature to date has consisted of case reports. A recent systematic review revealed heterogeneity in the data as not all reports documented treatment regimens and course of disease. The recent 2019 WHO update on neuroendocrine carcinoma nomenclature adds to the pre-existing classification system based on biologic activity, to better represent the spectrum of neuroendocrine non-neuroendocrine tumours (Frizziero et al., 2020).
We present a case of a patient who presented with anal pain, had a wide local excision which on histopathology revealed poorly differentiated MANEC. Despite adjuvant chemotherapy with cisplatin and etoposide as well as pelvic radiotherapy, the patient developed bi-lobar liver metastases within 9 months of initial presentation. The patient succumbed to colonic perforation 10 months after initial presentation.
Most patients present with advanced disease with site-specific symptoms, and despite treatment of localised disease, many recur with distant metastasis.
Although rare, this disease is highly aggressive, thus it is hoped that more clinicians can be made aware about its various clinical manifestations and disease course.
混合性腺神经内分泌癌（MANEC）是一种罕见疾病，迄今为止，现有文献大多为病例报告。最近一项系统评价显示数据存在异质性，因为并非所有报告都记录了治疗方案和疾病进程。世界卫生组织（WHO）2019年关于神经内分泌癌命名法的最新更新在基于生物学活性的现有分类系统基础上进行了补充，以更好地体现神经内分泌-非神经内分泌肿瘤的谱系（弗里齐耶罗等人，2020年）。
我们报告一例患者，该患者以肛门疼痛就诊，接受了广泛局部切除，组织病理学检查显示为低分化MANEC。尽管接受了顺铂和依托泊苷辅助化疗以及盆腔放疗，但患者在初次就诊后9个月内出现双叶肝转移。患者在初次就诊后10个月死于结肠穿孔。
大多数患者以晚期疾病及部位特异性症状就诊，尽管对局限性疾病进行了治疗，但许多患者仍会出现远处转移复发。
尽管这种疾病罕见，但具有高度侵袭性，因此希望能让更多临床医生了解其各种临床表现和疾病进程。
