Atwal Dinesh, Joshi Krishna Prasad, Jeffus Susanne, Ntambi James, Mahmoud Fade
Internal Medicine Resident at the University of Arkansas for Medical Sciences in Little Rock.
Assistant Professor of Pathology at the University of Arkansas for Medical Sciences in Little Rock.
Perm J. 2017;21:16-193. doi: 10.7812/TPP/16-193.
Incidence of well-differentiated neuroendocrine tumors (NETs) of the colon and rectum is increasing and is now approximately 1 per 100,000 in the US. NETs are either well-differentiated (indolent) or poorly differentiated (aggressive). The majority of these tumors are found incidentally during screening colonoscopies and rarely are associated with symptoms of hormonal syndrome, even during the advanced stage. Metastatic well-differentiated NETs of the colon and rectum are incurable, hard to treat, and associated with a poor prognosis and survival rates similar to colorectal adenocarcinoma survival.
A 56-year-old man presented to our clinic with right-sided weakness and a 40-pound weight loss during the previous 2 months. A neurologic examination was remarkable for atrophy of the right trapezius muscle and decreased strength in the right upper extremity. Imaging revealed extensive blastic and lytic lesions involving the axial skeleton, a large rectal mass, a large necrotic nodal mass extending from the left iliac region to the level of the left renal veins, and multiple necrotic liver metastasis. Liver lesion fine-needle aspiration findings were consistent with metastatic well-differentiated neuroendocrine carcinoma.
This case illustrates how a low-grade tumor can have an aggressive course with poor outcomes. Metastatic well-differentiated NETs of the colon and rectum remain difficult to treat because evidence is scarce. More research is needed on this topic.
结肠和直肠高分化神经内分泌肿瘤（NETs）的发病率正在上升，目前在美国约为十万分之一。NETs分为高分化（惰性）或低分化（侵袭性）。这些肿瘤大多在结肠镜筛查时偶然发现，即使在晚期也很少伴有激素综合征症状。结肠和直肠转移性高分化NETs无法治愈，难以治疗，预后不良，生存率与结直肠癌相似。
一名56岁男性因右侧肢体无力和在过去2个月内体重减轻40磅前来我院就诊。神经系统检查显示右侧斜方肌萎缩，右上肢肌力下降。影像学检查发现广泛的成骨和溶骨性病变累及中轴骨骼、一个大的直肠肿块、一个从左髂区延伸至左肾静脉水平的大的坏死性淋巴结肿块以及多个坏死性肝转移灶。肝脏病变细针穿刺结果与转移性高分化神经内分泌癌一致。
本病例说明了低级别肿瘤如何具有侵袭性病程和不良预后。结肠和直肠转移性高分化NETs仍然难以治疗，因为证据稀少。关于这个主题需要更多的研究。
