Department of Thoracic Surgery, Hôpital Cochin, APHP.CUP, Université de Paris, France.
Department of Chest Radiology, Hôpital Cochin, APHP.CUP, Université de Paris, France.
Lung Cancer. 2021 Jun;156:117-121. doi: 10.1016/j.lungcan.2021.04.024. Epub 2021 Apr 30.
Diffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) is a rare disease often associated with carcinoid tumors. We aimed at evaluating the impact of DIPNECH on characteristics and prognosis of patients who underwent radical treatment of pulmonary carcinoid tumors.
We reviewed all patients operated on for curative-intent resection of carcinoid tumor in our department from 2001 to 2020. Cases exhibiting both pathological and radiological features of DIPNECH, as assessed by respective thoracic expert physicians, were analyzed separately.
172 cases of resected carcinoid tumors were identified, including 25 (14.5 %) harboring pathological criteria of DIPNECH and radiologic features like mosaic attenuation (92.0 %), multiple nodules < 5 mm (76.0 %), and mucoid impactions (32 %). In DIPNECH patients, major pulmonary resections were usually performed (92.0 %) and resected tumors were mostly classified as pT1 (92 %). Mean Ki67 staining was 3.7 ± 5.2 %. The early postoperative period was mostly uneventful (96.0 %) and 5-year survival was 92.9 ± 6.9 %. Compared to non-DIPNECH cases, we found that patients were older (mean 65.6 ± 9.3 versus 54.1 ± 17.9, p = 0.002), more frequently female (84.0 % versus 56.5 %, p = 0.009), and exhibiting diabetes mellitus (45.8 % versus 18.5 %, p < 0.001) or hypertension (45.8 % versus 24.1 %, p = 0.039). The rate of atypical carcinoid tumors was significantly higher in DIPNECH patients (40.0 % versus 19.9 %, p = 0.027), as well as rate of mediastinal lymph-nodes involvement (pN2+) (36.0 % versus 4.1 %, p < 0.001). At multivariate analysis, only DIPNECH pattern and atypical histology were independent factors of pN2 invasion which was the only predictor of poorer prognosis on Log-Rank test.
Carcinoid tumors with proven DIPNECH are associated with negative pathological features and may deserve a dedicated perioperative management.
弥漫性特发性肺神经内分泌细胞增生症（DIPNECH）是一种罕见疾病，常与类癌肿瘤相关。我们旨在评估 DIPNECH 对接受肺类癌肿瘤根治性治疗患者的特征和预后的影响。
我们回顾了 2001 年至 2020 年期间在我科接受根治性切除术治疗类癌肿瘤的所有患者。由各自的胸部专家医生评估，具有 DIPNECH 的病理和影像学特征的病例分别进行分析。
共确定了 172 例切除的类癌肿瘤病例，其中 25 例（14.5%）具有 DIPNECH 的病理标准和马赛克衰减（92.0%）、<5mm 的多个结节（76.0%）和粘蛋白嵌塞（32.0%）等影像学特征。在 DIPNECH 患者中，通常进行主要的肺切除术（92.0%），切除的肿瘤大多分类为 pT1（92.0%）。平均 Ki67 染色为 3.7±5.2%。术后早期大多无并发症（96.0%），5 年生存率为 92.9±6.9%。与非 DIPNECH 病例相比，我们发现患者年龄较大（平均 65.6±9.3 岁比 54.1±17.9 岁，p=0.002），女性更常见（84.0%比 56.5%，p=0.009），并患有糖尿病（45.8%比 18.5%，p<0.001）或高血压（45.8%比 24.1%，p=0.039）。DIPNECH 患者中不典型类癌肿瘤的发生率明显更高（40.0%比 19.9%，p=0.027），纵隔淋巴结受累（pN2+）的发生率也更高（36.0%比 4.1%，p<0.001）。多因素分析显示，只有 DIPNECH 模式和非典型组织学是 pN2 侵犯的独立因素，是 Log-Rank 检验中预后较差的唯一预测因素。
证实患有 DIPNECH 的类癌肿瘤与阴性病理特征相关，可能需要专门的围手术期管理。
