Koliakos Evangelos, Thomopoulos Theodoros, Abbassi Ziad, Duc Christophe, Christodoulou Michel
Department of General Surgery, Hospital of Valais (CHVR), Sion, Switzerland.
Department of Histopathology, Hospital of Valais (CHVR), Sion, Switzerland.
Am J Case Rep. 2017 Sep 11;18:975-979. doi: 10.12659/ajcr.904468.
BACKGROUND Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder that is characterized by diffuse hyperplasia of bronchiolar and bronchial pulmonary neuroendocrine cells. In this condition, when no other pathological pulmonary condition is detected, DIPNECH is considered to be an idiopathic lung disease. DIPNECH is a rare condition that can be difficult to distinguish from other forms of reactive pulmonary neuroendocrine cell hyperplasia (NECH). We present a case of DIPNECH and describe the approach to diagnosis of this rare condition. CASE REPORT A 69-year-old woman with a past medical history of successfully treated lobular carcinoma of the breast, presented to our department with a respiratory tract infection. High-resolution computed tomography (HRCT) of the chest showed a suspicious pulmonary nodule, measuring 13 mm, in the right middle pulmonary lobe. Combined positron emission tomography (PET) and computed tomography (CT), showed a solid and metabolically active nodule. A transbronchial biopsy and histopathology confirmed a diagnosis of DIPNECH. CONCLUSIONS It is possible that DIPNECH is an under-diagnosed pulmonary condition because it is rarely associated with symptoms. At this time, there are no evidence-based management guidelines. While the majority of cases have stable clinical course, some cases can progress to cause airway obstruction. This case report highlights this rare, but potentially progressive condition, and the need for evidence-based management guidelines for DIPNECH.
背景 弥漫性特发性肺神经内分泌细胞增生（DIPNECH）是一种罕见的肺部疾病，其特征为细支气管和支气管肺神经内分泌细胞的弥漫性增生。在这种情况下，当未检测到其他病理性肺部疾病时，DIPNECH被认为是一种特发性肺部疾病。DIPNECH是一种罕见疾病，可能难以与其他形式的反应性肺神经内分泌细胞增生（NECH）相区分。我们报告一例DIPNECH病例，并描述诊断这种罕见疾病的方法。病例报告 一名69岁女性，既往有成功治疗的乳腺小叶癌病史，因呼吸道感染就诊于我科。胸部高分辨率计算机断层扫描（HRCT）显示右肺中叶有一个可疑的肺结节，大小为13毫米。正电子发射断层扫描（PET）与计算机断层扫描（CT）联合检查显示为实性且代谢活跃的结节。经支气管活检及组织病理学检查确诊为DIPNECH。结论 DIPNECH可能是一种诊断不足的肺部疾病，因为它很少伴有症状。目前，尚无基于证据的管理指南。虽然大多数病例临床过程稳定，但有些病例可能进展导致气道阻塞。本病例报告突出了这种罕见但可能进展的疾病，以及对DIPNECH基于证据的管理指南的需求。
