弥漫性特发性肺神经内分泌细胞增生合并肺原发性滑膜肉瘤1例临床分析并文献复习

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia combined with primary pulmonary synovial sarcoma: A clinical analysis of 1 case and literature review

目的提高对弥漫性特发性肺神经内分泌细胞增生(diffuse idiopathic pulmonary neuroendocrine cell hyperplasia,DIPNECH)及肺原发性滑膜肉瘤(primary pulmonary synovial sarcoma,PPSS)的认识,并探讨两种疾病发生的关联性。方法对本院确诊的1例DIPNECH合并PPSS的临床资料、病理学及影像学表现进行回顾性分析,并复习相关文献。结果患者为20岁男性,临床表现为胸闷、咳嗽、咯血。CT增强扫描发现:1)双肺多发斑片影;2)左肺上叶空腔伴壁结节;3)左肺下叶血肿伴肿块。术后病理诊断为弥漫性特发性肺神经内分泌细胞增生合并肺原发性单相纤维型滑膜肉瘤。随访10个月,患者无任何不适,未见复发和转移。结论 DIPNECH和PPSS均是极其罕见的疾病,临床及影像学表现复杂多样且无特异性,确诊有赖于病理学检查。

Objective To improve the understanding of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia （DIPNECH） and primary pulmonary synovial sarcoma （PPSS）, and explore the possible correlation between them. Methods Clinical manifestations, pathological change and imaging findings of a patient with DIPNECH and PPSS admitted to our hospital were retrospectively analyzed with related literature. Results The 20 years old male patient＇s clinical manifestation were chest congestion, cough and hemoptysis. Contrast enhanced chest CT revealed that patchy shadows appeared in bilateral lungs, cyst with nodule appeared in superior lobe of left lung and a mass in hematoma showed in inferior lobe of left lung. The postoperative pathology examination proved to be diffuse idiopathic pulmonary neuroendocrine cell hyperplasia （DIPNECH） with primary pulmonary monophasic fibrous synovial sarcoma （PPSS）. The patient was followed up for 10 months during which he felt no discomfort and showed no recurrence or metastasis. Conclusion Both DIPNECH and PPSS are extremely rare diseases with complicated and nonspecific clinical manifestations and imaging findings. Pathology is essential for confirmation of the diagnosis.