协和医学杂志

2016, (03)

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胸腺神经内分泌肿瘤手术治疗及预后因素
Neuroendocrine Tumors of the Thymus:Outcomes after Surgical Resection and Prognostic Factors

陈野野;刘洪生;李单青;黄诚;李力;秦应之

摘要(Abstract):

目的 探讨胸腺神经内分泌肿瘤(neuroendocrine tumors of the thymus,NETT)的临床特点、手术为主的综合治疗经验及相关预后因素。方法 2004年12月至2013年12月在北京协和医院诊治并经手术病理证实为NETT的患者26例,其中男性18例,女性8例,回顾性分析其临床表现、影像学资料、手术为主的综合治疗方案、围术期并发症及随访情况。结果 26例患者就诊时中位年龄为46岁(13~75岁),中位病程为3.5个月(1~84个月)。起病隐匿,临床症状不特异,7例合并库欣综合征,1例合并多发性内分泌腺瘤病1型。胸部增强CT可见胸腺区占位。所有患者均接受开胸手术切除治疗,22例肿瘤大体完整切除。无围术期死亡病例,出现并发症3例。16例患者术后行辅助治疗。术后病理类型为高分化的神经内分泌癌20例(典型类癌8例,不典型类癌12例),低分化的神经内分泌癌6例(小细胞神经内分泌癌5例,大细胞神经内分泌癌1例)。Masaoka-Koga分期为Ⅰ期4例,Ⅱ期3例,Ⅲ期12例,Ⅳ期7例。长期随访结果示中位生存期为51.0个月,3年、5年生存率为71.0%和44.6%。多因素分析结果示肿瘤分化程度(P=0.039)及分期(P=0.012)是影响预后的因素。结论 NETT是一种罕见的侵袭性极强的恶性病变,临床表现不特异,早期诊断及治疗存在很大难度,早期行增强CT有助于发现病变及评估手术可行性。肿瘤分化程度及分期可能是影响预后的主要因素。
Objective To discuss the clinical manifestations,surgery-based therapy of neuroendocrine tumors of the thymus (NETT) and the potential prognostic factors.Methods We selected 26 consecutive cases diagnosed, treated, and confirmed as NETT by postoperative pathology between December 2004 and December 2013 in Peking Union Medical College Hospital, including 18 males and 8 females. Their clinical manifestations, imaging findings, surgery-based therapy, perioperative complications, and follow-up were retrospectively analyzed. ResultsThe median age of the 26 cases was 46(13-75)years and the median duration of disease was 3.5(1-84)months. Early detection of NETT was difficult due to occult onset and nonspecific clinical manifestations. Seven cases were complicated with Cushings syndrome and 1 case with multiple endocrine neoplasia type 1. Thoracic contrast-enhanced computed tomography showed mass in the region of thymus. All the cases received thoracotomy and 22 cases got macroscopically radical resection, with 3 cases developing complications and no perioperative death. Sixteen cases received adjuvant therapy after the surgery. Pathologically, there were 20 well differentiated cases (8 typical carcinoids and 12 atypical carcinoids), and 6 poorly differentiated cases (5 small cell and 1 large cell neuroendocrine tumors). There were 4 Masaoka-Koga stage Ⅰ cases, 3 stage Ⅱ cases, 12 stage Ⅲ cases, and 7 stage Ⅳ cases. After long-term follow-up, the median survival was 51.0 months, and 3- and 5-year survival rates were 71.0% and 44.6%, respectively. Multivariate analysis showed that degree of tumor differentiation (P=0.039) and staging (P=0.012) had impact on prognosis. Conclusions NETT is a rare malignancy with tremendous aggressiveness. Early confirmed diagnosis and therapy is still a big challenge due to nonspecific clinical manifestations. Thoracic contrast-enhanced CT could help detect the tumor and evaluate the possibility of surgery. Stage and differentiation of the tumor might be major prognostic factors.

关键词(KeyWords): |神经内分泌肿瘤|胸腺|手术治疗|预后|
|Neuroendocrine Tumors|Thymus|Surgery|Prognosis|

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作者(Author): 陈野野;刘洪生;李单青;黄诚;李力;秦应之

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