Qiu S, Pellino G, Warren O J, Mills S, Goldin R, Kontovounisios C, Tekkis P P
a Department of Surgery and Cancer, Chelsea and Westminster Hospital , Imperial College London , London , UK.
b Department of Colorectal Surgery , Royal Marsden Hospital , London , UK.
Acta Chir Belg. 2018 Oct;118(5):273-277. doi: 10.1080/00015458.2018.1482697. Epub 2018 Jun 17.
Mixed adenoneuroendocrine carcinoma (MANEC) are rare cancers of the gastrointestinal (GI) and pancreatobiliary tract. They are characterized by the presence of a combination of epithelial and neuroendocrine elements, where each component represents at least 30% of the tumour. Review of literature and consolidation of clinicopathological data. Sixty-one cases of colorectal MANEC have been reported in literature and one seen in this centre. The median age of the patients affected was 61.9 ± 12.4 years (20-94 years). Male to female ratio is 1.0:1.2. Presentations were similar to other colorectal malignancies. 58.0% of colorectal MANECs were found in the right colon, 8.1% cases in the transverse, 16.1% in the left colon, 16.1% in the rectum. These tumours appeared invasiveness 79.1% were T3-T4. Over 90% of cases were presented with metastatic disease. The majority of patient underwent surgical resection of the primary cancer (96.6%). Of these, 10 operations (17.9%) were emergency operations due to obstruction, perforation, or bleeding. Three patients received first line palliative care. In eight cases (13.8%), patients underwent adjuvant chemotherapy. The median overall survival after diagnosis was 10 ± 2.4 months (95% CI: 5.37-14.64 months). MANECs are rare but aggressive colorectal cancers. Surgical resection of localized disease with adjuvant chemotherapy appears to significantly improve survival in small case series. Further understanding through the sharing of experiences is required.
混合性腺神经内分泌癌（MANEC）是胃肠道和胰胆管系统的罕见癌症。其特征是同时存在上皮和神经内分泌成分，且每种成分在肿瘤中所占比例至少为30%。文献回顾及临床病理数据汇总。文献报道了61例结直肠MANEC，本中心有1例。受影响患者的中位年龄为61.9±12.4岁（20 - 94岁）。男女比例为1.0:1.2。临床表现与其他结直肠恶性肿瘤相似。58.0%的结直肠MANEC位于右半结肠，8.1%位于横结肠，16.1%位于左半结肠，16.1%位于直肠。这些肿瘤具有侵袭性，79.1%为T3 - T4期。超过90%的病例出现转移性疾病。大多数患者接受了原发性癌症的手术切除（96.6%）。其中，10例手术（17.9%）是由于梗阻、穿孔或出血而进行的急诊手术。3例患者接受了一线姑息治疗。8例（13.8%）患者接受了辅助化疗。诊断后的中位总生存期为10±2.4个月（95%置信区间：5.37 - 14.64个月）。MANEC是罕见但侵袭性强的结直肠癌。在小样本系列中，对局限性疾病进行手术切除并辅以化疗似乎能显著提高生存率。需要通过经验分享来进一步了解。
