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[弥漫性特发性肺神经内分泌细胞增生症:一种罕见的肿瘤前状态]

[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a rare preneoplastic condition].

作者信息

Lebras L, Arpin D, Collardeau-Frachon S, Isaac S, Guerin J-C, Perol M

机构信息

Service de Pneumologie, Hôpital de la Croix Rousse, CHU de Lyon, France.

出版信息

Rev Mal Respir. 2008 Nov;25(9):1131-5. doi: 10.1016/s0761-8425(08)74985-4.

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an uncommon preneoplastic condition, often associated with typical carcinoid tumours. The observations reported below concern two women, both suffering from chronic pulmonary symptoms. These patients underwent computed tomography that showed a solitary nodule in the first patient and multiple sub centimetre nodules in the second. In both cases histological studies of the pulmonary biopsies revealed: a proliferation of neuroendocrine cells dispersed in the bronchial and bronchiolar epithelium, more specifically superficial to the basement membrane; some tumourlets; a typical carcinoid tumour was also found in the first patient's biopsy. The choice of treatment remains difficult, mainly because the existing studies are restricted to small numbers of patients or isolated cases, a consequence of the low prevalence of this disease. Considering its slow evolution, management by long-term clinical, endoscopic and radiologic surveillance may be considered. If a carcinoid tumour is present or appears during the surveillance, the standard treatment is still surgical resection.

摘要

弥漫性特发性肺神经内分泌细胞增生症(DIPNECH)是一种罕见的癌前病变,常与典型类癌肿瘤相关。以下报告的观察结果涉及两名患有慢性肺部症状的女性。这两名患者均接受了计算机断层扫描,结果显示第一名患者有一个孤立结节,第二名患者有多个小于1厘米的结节。在这两个病例中,肺活检的组织学研究显示:神经内分泌细胞在支气管和细支气管上皮中呈弥漫性增生,更具体地说是在基底膜表面;存在一些微瘤;在第一名患者的活检中还发现了一个典型类癌肿瘤。治疗方案的选择仍然很困难,主要是因为现有研究仅限于少数患者或个别病例,这是该疾病发病率低的结果。考虑到其进展缓慢,可以考虑通过长期临床、内镜和放射学监测进行管理。如果在监测期间出现类癌肿瘤,标准治疗方法仍然是手术切除。

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