肺朗格汉斯细胞组织细胞增生症误诊为肺肿瘤四例报告并文献复习_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

沈旦 ¹ ,  陈涛 ¹ , 祝清清 ¹ , 蒋军红 ¹ , 杜明占 ² , 黄建安 ¹

1. ;
2. ;

Corresponding author：

陈涛, Email: ct122@163.com

Keywords：

肺朗格汉斯细胞组织细胞增生症; 临床特征; 正电子发射计算机断层显像; 病理诊断

DOI：

10.7507/1671-6205.202106091

Video：

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目的探讨误诊为肺部肿瘤的肺朗格汉斯细胞组织细胞增生症（pulmonary Langerhans cell histiocytosis，PLCH）的临床特点，提高该疾病的诊治能力。方法收集本院4例初诊误诊为肺部肿瘤后经病理诊断的PLCH患者的临床资料，并回顾复习相关文献资料进行分析。结果 4例PLCH中，男1例，女3例，年龄45～78岁。吸烟2例，不吸烟2例。临床表现2例为体检发现，1例发热，1例浅表淋巴结肿大起病。胸部CT表现为2例多发的肺囊肿、肺大疱改变，4例均出现结节影，其中3例为单发，1例双肺多发圆形小结节影，部分见空洞。3例行正电子发射计算机断层显像（positron emission computed tomography/X-ray computed tomography，PET/CT）检查的患者均提示肺部及淋巴结等病灶标准摄取值增高。4例患者均被误诊为肺部肿瘤，经外科手术、肺穿刺及气管镜等检查，病理见朗格汉斯组织细胞增生，结合S100、CD1a、Langerin等特征性免疫组织化学标志物，最终确诊PLCH。结论 PLCH临床表现不典型，影像学表现为肺结节及多发囊气腔改变需考虑PLCH诊断可能，PET/CT易误诊，通过活检行常规及特征性免疫组织化学病理检查可明确诊断。

Citation： 沈旦, 陈涛, 祝清清, 蒋军红, 杜明占, 黄建安. 肺朗格汉斯细胞组织细胞增生症误诊为肺肿瘤四例报告并文献复习. Chinese Journal of Respiratory and Critical Care Medicine, 2022, 21(12): 885-889. doi: 10.7507/1671-6205.202106091 Copy

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2.	Girschikofsky M, Arico M, Castillo D, et al. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net. Orphanet J Rare Dis, 2013, 8: 72.
3.	Suri HS, Yi ES, Nowakowski GS, et al. Pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis, 2012, 7: 16.
4.	Ma J, Laird JH, Chau KW, et al. Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies. Cancer Med, 2019, 8(1): 58-66.
5.	Gupta N, Langenderfer D, McCormack FX, et al. Chest computed tomographic image screening for cystic lung diseases in patients with spontaneous pneumothorax is cost effective. Ann Am Thorac Soc, 2017, 14(1): 17-25.
6.	Radzikowska E, Błasińska-Przerwa K, Wiatr E, et al. Pneumothorax in patients with pulmonary Langerhans cell histiocytosis. Lung, 2018, 196(6): 715-720.
7.	Le Guen P, Chevret S, Bugnet E, et al. Management and outcomes of pneumothorax in adult patients with Langerhans cell Histiocytosis. Orphanet J Rare Dis, 2019, 14(1): 229.
8.	Agarwal KK, Seth R, Behra A, et al. 18F-Fluorodeoxyglucose PET/CT in Langerhans cell histiocytosis: spectrum of manifestations. Jpn J Radiol, 2016, 34(4): 267-276.
9.	Haroche J, Cohen-Aubart F, Rollins BJ, et al. Histiocytoses: emerging neoplasia behind inflammation. Lancet Oncol, 2017, 18(2): e113-e125.
10.	Roden AC, Hu XW, Kip S, et al. BRAF V600E expression in Langerhans cell histiocytosis: clinical and immunohistochemical study on 25 pulmonary and 54 extrapulmonary cases. Am J Surg Pathol, 2014, 38(4): 548-551.
11.	Mourah S, How-Kit A, Meignin V, et al. Recurrent NRAS mutations in pulmonary Langerhans cell histiocytosis. Eur Respir J, 2016, 47(6): 1785-1796.
12.	Phulware RH, Guleria P, Iyer VK, et al. Cytological diagnosis of Langerhans cell histiocytosis: a series of 47 cases. Cytopathology, 2019, 30(4): 413-418.
13.	Vassallo R, Ryu JH, Schroeder DR, et al. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med, 2002, 346(7): 484-490.
14.	Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood, 2010, 116(11): 1919-1923.
15.	Kolenová A, Schwentner R, Jug G, et al. Targeted inhibition of the MAPK pathway: emerging salvage option for progressive life-threatening multisystem LCH. Blood Adv, 2017, 1(6): 352-356.
16.	周婧, 李霞, 张稷, 等. 双肺移植后复发性朗格汉斯细胞组织细胞增生症1例并文献复习. 实用器官移植电子杂志, 2019, 7(5): 371-374.

1. Emile JF, Abla O, Fraitag S, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood, 2016, 127(22): 2672-2681.
2. Girschikofsky M, Arico M, Castillo D, et al. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net. Orphanet J Rare Dis, 2013, 8: 72.
3. Suri HS, Yi ES, Nowakowski GS, et al. Pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis, 2012, 7: 16.
4. Ma J, Laird JH, Chau KW, et al. Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies. Cancer Med, 2019, 8(1): 58-66.
5. Gupta N, Langenderfer D, McCormack FX, et al. Chest computed tomographic image screening for cystic lung diseases in patients with spontaneous pneumothorax is cost effective. Ann Am Thorac Soc, 2017, 14(1): 17-25.
6. Radzikowska E, Błasińska-Przerwa K, Wiatr E, et al. Pneumothorax in patients with pulmonary Langerhans cell histiocytosis. Lung, 2018, 196(6): 715-720.
7. Le Guen P, Chevret S, Bugnet E, et al. Management and outcomes of pneumothorax in adult patients with Langerhans cell Histiocytosis. Orphanet J Rare Dis, 2019, 14(1): 229.
8. Agarwal KK, Seth R, Behra A, et al. 18F-Fluorodeoxyglucose PET/CT in Langerhans cell histiocytosis: spectrum of manifestations. Jpn J Radiol, 2016, 34(4): 267-276.
9. Haroche J, Cohen-Aubart F, Rollins BJ, et al. Histiocytoses: emerging neoplasia behind inflammation. Lancet Oncol, 2017, 18(2): e113-e125.
10. Roden AC, Hu XW, Kip S, et al. BRAF V600E expression in Langerhans cell histiocytosis: clinical and immunohistochemical study on 25 pulmonary and 54 extrapulmonary cases. Am J Surg Pathol, 2014, 38(4): 548-551.
11. Mourah S, How-Kit A, Meignin V, et al. Recurrent NRAS mutations in pulmonary Langerhans cell histiocytosis. Eur Respir J, 2016, 47(6): 1785-1796.
12. Phulware RH, Guleria P, Iyer VK, et al. Cytological diagnosis of Langerhans cell histiocytosis: a series of 47 cases. Cytopathology, 2019, 30(4): 413-418.
13. Vassallo R, Ryu JH, Schroeder DR, et al. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med, 2002, 346(7): 484-490.
14. Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood, 2010, 116(11): 1919-1923.
15. Kolenová A, Schwentner R, Jug G, et al. Targeted inhibition of the MAPK pathway: emerging salvage option for progressive life-threatening multisystem LCH. Blood Adv, 2017, 1(6): 352-356.
16. 周婧, 李霞, 张稷, 等. 双肺移植后复发性朗格汉斯细胞组织细胞增生症1例并文献复习. 实用器官移植电子杂志, 2019, 7(5): 371-374.

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肺朗格汉斯细胞组织细胞增生症误诊为肺肿瘤四例报告并文献复习

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