直肠神经内分泌肿瘤的临床病理特征及预后

Clinical and pathological characteristics and prognosis of rectal neuroendocrine tumors

目的探讨84例直肠神经内分泌肿瘤(neuroendocrine neoplasms,NENs)的临床病理特征、预后分析,以提高对该疾病的认识水平,减少误诊及漏诊.方法收集2012-04/2015-04 3年间郑州大学第一附属医院内镜活检或手术病理检查确诊为NENs的84份患者的临床资料,对其临床表现、病理特征、诊断、治疗及预后进行分析.结果本组84例NENs患者,年龄范围21-70岁,平均48.8岁±13.4岁,其中男性52例(61.9%),主要症状为排便习惯改变、便血和腹痛,均无类癌综合征表现.病理分级G1级40例(47.6%),G2级40例(47.6%),G3级4例(4.8%);免疫组织化学指标突触素(Syn)阳性率者84例(100%),嗜铬颗粒蛋白(Cg A)阳性者58例(69.0%),神经元特异性烯醇化酶(NSE)阳性者36例(42.9%).其中,20例行内镜下治疗,56例行外科手术治疗,8例姑息治疗;发生转移者12例(14.3%),其中淋巴结转移8例,肝转移者4例.随访时间中位数为16 mo(3-32 mo).随访期内,共有4例(4.8%)复发,8例(9.5%)死亡.结论NENs是一种临床表现不典型、预后相对较好的低度恶性肿瘤,临床工作中应提高对其的认识,对内镜下难以确诊的可疑病变组织尽早行组织活检,减少误诊及漏诊.

AIM To analyze the clinical and pathological features and prognosis of rectal neuroendocrine neoplasms （NENs） to improve the diagnosis and treatment of this disease and reduce its misdiagnosis and missed diagnosis. METHODS We collected the clinical data of 84 patients with rectal NENs, who were diagnosed by endoscopic biopsy or surgical pathologic examination at the First Affiliated Hospital of Zhengzhou University from April 2012 to April 2015. The clinical manifestations, pathological manifestations, diagnosis, treatment and prognosis were analyzed. RESULTS The average age of the 84 patients was 48.8 ± 13.4 years （range 21-70, years）, and there were 52 （61.9%） males. Main symptoms were bowel habit change, gastrointestinal bleeding and abdominal pain, and there was no carcinoid syndrome. Pathological grading results were： G1 in 40 （47.6%） cases, G2 in 40 （47.6%）, and G3 in 4 （4.8%）. With regard to immunohistochemical markers, all cases were positive for Syn, 58 （69.0%） cases were positive for CgA, and 36 （42.9%） positive for NSE. Of all the patients, 20 were treated by endoscopic therapy, 56 treated by surgery, and 8 by palliative care. Twelve （14.3%） cases developed metastases, including lymph node metastasis in eight cases and liver metastasis in four cases. The median follow-up period was 16 mo （3-32 too）. During follow-up, four cases recurred and eight cases died. CONCLUSION Rectal NENs are a group of low grade malignant tumors with atypical clinical manifestations and relatively good prognosis. The awareness of rectal NENs should be improved. Suspicious lesions that cannot be diagnosed by endoscopic biopsy should be subject to pathological examination as early as possible to reduce the misdiagnosis and missed diagnosis.