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1、肺神经内分泌肿瘤肺神经内分泌肿瘤 Pulmonary neuroendocrine tumorsPulmonary neuroendocrine tumors 复旦大学附属肿瘤医院内科 王惠杰 Incidence of Neuroendocrine TumorsIncidence of Neuroendocrine Tumors Yao, JC, et al. J Clin Oncol 2008;26:3063-3072. WHO/IASLC/ATS/ERSWHO/IASLC/ATS/ERS Preinvasive lesionsPreinvasive lesions Diffuse idio
2、pathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) Carcinoid tumorCarcinoid tumor Typical carcinoid (TC)Typical carcinoid (TC) Atypical carcinoid (AC) Atypical carcinoid (AC) Small cell carcinomaSmall cell carcinoma Combi
3、ned small cell carcinomaCombined small cell carcinoma Large cell neuroendocrine carcinoma(LCNEC)Large cell neuroendocrine carcinoma(LCNEC) - Combined LCNEC- Combined LCNEC DIPNECHDIPNECH 女性，女性，50-7050-70岁岁 可无症状可无症状 慢性慢性干咳、呼吸困难干咳、呼吸困难 影像表现：磨玻璃影，双肺多发结节，细影像表现：磨玻璃影，双肺多发结节，细/ /支气管支气管 增增厚，闭塞性细支气管炎厚，闭塞性细支气
4、管炎 可发展为可发展为TC/ACTC/AC 皮质激素、皮质激素、a-IFNa-IFN、生长抑素，化疗、生长抑素，化疗 DIPNECHDIPNECH 100 cases reviewed100 cases reviewed Female 89%Female 89% 诊断平均年龄诊断平均年龄57.857.8 诊断前症状持续诊断前症状持续3-303-30年年 63%63%存在通气性障碍存在通气性障碍 5555例随访，例随访，66%66%病情稳定，病情稳定，27%27%缓慢进展，缓慢进展， 11%11%非疾病相关死亡非疾病相关死亡 SSA 6SSA 6例，例，SDSD TCACLNECSCLC NET
5、 GradeWell G1Well G2Poor G3Poor G3 Mitosis/10HP20% Clinical Young/male or female/non-smoker Young/male or female/non-smoker Older/male/smokerOlder/male/smoker Lung locationCentralPeripheralMidzone or peripheral Central Rate of regional metastasis 515%25%40%90% Small Cell Lung CellSmall Cell Lung Cel
6、l - a forgotten disease in targeted era- a forgotten disease in targeted era？！？！ 病理分类病理分类 WHO/IASLCWHO/IASLC肺上皮性肿瘤组织学分类肺上皮性肿瘤组织学分类 1.3.2. 1.3.2. 小细胞癌小细胞癌( (Small cell carcinoma)Small cell carcinoma) 变异变异( (Variant)Variant) 1.3.2.1. 1.3.2.1. 混合性小细胞癌混合性小细胞癌( (Combined small cell Combined small cell ca
7、rcinoma)carcinoma) SCLCSCLC混合任何一种混合任何一种NSCLCNSCLC成分，常见包括腺癌、鳞癌和大细胞癌（成分，常见包括腺癌、鳞癌和大细胞癌（ LCNECLCNEC），），较少见的梭形、巨细胞癌等较少见的梭形、巨细胞癌等 临床特点 男性约占80% 典型表现：肺门部原发肿瘤纵隔LN形成的大肿 块、远处转移 中央型占80% 空洞少见 胸腔积液发生率与其他类型相近 伴瘤综合征 受累器官组织 诊断%尸检% 纵隔淋巴结66-8073-87 肝21-2769 骨27-4154 肾上腺5-3135-65 骨髓15-30NA 脑10-1428-50 腹膜后淋巴结3-1229-52
8、锁骨上淋巴结1742 胸腔积液16-2030 对侧肺1-128-27 软组织519 SCLC-StagingSCLC-Staging History and physical examinationHistory and physical examination Routine hematologic and serum chemistry tests, LDHRoutine hematologic and serum chemistry tests, LDH Chest Chest + (upper) abdomen CT scan+ (upper) abdomen CT scan Bone
9、 scan Bone scan CT with contrast or MRI of the brainCT with contrast or MRI of the brain PET : PET : invasive procedures/better definition of invasive procedures/better definition of irradiation field, possible future role for better pt irradiation field, possible future role for better pt stagingst
10、aging VALGVALG：SCLC-StagingSCLC-Staging - - limited-stage disease : limited-stage disease : tumor confined to one hemithorax with tumor confined to one hemithorax with or without homolateral nodes/pleural or without homolateral nodes/pleural effusioneffusion that can be encompassed within a single t
11、hat can be encompassed within a single tolerable radiation therapy porttolerable radiation therapy port - - areas of controversyareas of controversy: contralateral hilar or : contralateral hilar or supraclavicular nodes or malignant pleural supraclavicular nodes or malignant pleural or pericardial e
12、ffusionsor pericardial effusions - - extensive diseaseextensive disease: : presence of metastatic diseasepresence of metastatic disease 20072007：SCLC Survival by TNM StageSCLC Survival by TNM Stage Shepherd FA J Thorac Oncol 2007; 2: 1067-1077 History of treatmentHistory of treatment 1969 1969 RT RT
13、 优于优于S S CTX CTX 优于优于BSCBSC 70 70年代年代 放疗更广泛应用放疗更广泛应用 联合化疗优于单药联合化疗优于单药 CAOCAO成为标准方案成为标准方案 1979 1979 EPEP方案出现方案出现 80 80年代年代 EPEP成为标准方案成为标准方案 90 90年代年代 化放联合治疗（化放联合治疗（LDLD） 新药研究（新药研究（CPT-11/ARMCPT-11/ARM等）等） 靶向治疗？靶向治疗？ 治疗原则治疗原则 Chemotherapy is the mainstay form of treatmentChemotherapy is the mainstay f
14、orm of treatment Radiotherapy also play a roleRadiotherapy also play a role，serving as “consolidation” therapy for serving as “consolidation” therapy for individual with LDindividual with LD For patients with EDFor patients with ED，chemotherapy alone is the standard of carechemotherapy alone is the standard of care American Cancer Society American Cancer Society Atlas of Clinical Oncology Atlas of Clinical Oncology：Lung Cancer 2002Lung Cancer
