Clinical analysis of Langerhans cell histiocytosis originating in the base of nasal skull
-
摘要: 目的:总结首发于鼻颅底的朗格汉斯细胞增生症(LCH)的临床特点,探讨其诊断及治疗方案。方法:回顾性分析以鼻颅底症状为首发表现的10例LCH患儿的资料,总结其鼻部及颅底症状的特点,并分析累及其他系统的相关性,总结手术治疗的特点及手术联合化疗的转归。结果:10例患儿年龄1岁5个月~8岁,平均3岁。影像学表现主要为溶骨性改变及软组织侵犯,7例患儿为单灶性,3例患儿为多系统多灶性。对于局限性的病变患儿,采取彻底切除病变加后续化疗的方法;对于累及多系统并有明显全身症状的患儿,采取保守治疗。随访4~96个月,8例存活,2例死亡。结论:LCH好发于儿童,具有一定的临床特征,单系统单病灶的手术治疗效果较好,配合后续的化疗可获得较大的生存率。Abstract: Objective: To summarize the clinical features, diagnosis and treatment of Langerhans histiocytosis(LCH) which first appeared in the nasal skull base. Method: Ten cases of LCH with nasal and skull base symptoms were analyzed retrospectively. The clinical characteristics of LCH with nasal and skull base symptoms were summarized. The correlation of other systems involved in LCH was analyzed. Result: Among the 10 patients, the youngest was 1 year and 5 months, and the oldest was 8 years, the average age was 3 years. The main imaging manifestations were osteolytic changes and soft tissue invasion. Seven patients were monofocal and three patients were multifocal. For localized lesions, radical resection and follow-up chemotherapy were performed, and conservative treatment was performed for patients with multiple system involvement and obvious systemic symptoms. Eight patients survived, 2 died. Conclusion: LCH occurs frequently in children and has certain clinical characteristics. Single system and single lesion surgery have a better therapeutic effect, and can achieve a greater survival rate with follow-up chemotherapy.
-
Key words:
- Langerhans histiocytosis /
- nasal skull base /
- child /
- surgical procedures, operative
-
-
[1] LIANG C,LIANG Q,DU C,et al.Langerhans' cell histiocytosis of the temporal fossa:A case report[J].Oncol Lett,2016,11:2625-2628.
[2] JEZIERSKA M,STEFANOWICZ J,ROMANOWICZ G,et al.Langerhans cell histiocytosis in children-a disease with many faces.Recent advances in pathogenesis,diagnostic examinations and treatment[J].Postepy Dermatol Alergol,2018,35:6-17.
[3] EMILE J F,ABLA O,FRAITAG S,et al.Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages[J].Blood,2016,127:2672-2681.
[4] BATES S V,LAKSHMANAN A,GREEN A L,et al.BRAF V600E-positive multisiteLangerhans cell histiocytosis in a preterm neonate[J].AJP Rep,2013,3:63-69.
[5] RIZZO F M,CIVES M,SIMONE V,et al.New insights into the molecular pathogenesis of langerhans cell histiocytosis[J].Oncologist,2014,19:151-163.
[6] BANASAL M,SRIVASTAVA V K,BANASAL R,et al.Severe periodontal disease manifested in chronic disseminated type of langerhans cell histiocytosis in a 3-year old child[J].Int J Clin Pediatr Dent,2014,7:217-219.
[7] IMASHUKU S,ARCECI R J.Strategies for the Prevention of Central Nervous System Complications in Patients with Langerhans Cell Histiocytosis:The Problem of Neurodegenerative Syndrome[J].Hematol Oncol Clin North Am,2015,29:875-893.
[8] 韩晓丹,周健,喻凤宽,等.朗格汉斯细胞增多症111例预后分析[J].中国实用内科学杂志,2015,35(12):1022-1026.
[9] KAZAHAYA K.Lateral Skull Base Approaches in Pediatric Skull Base Surgery[J].J Neurol Surg B Skull Base,2018,79:47-57.
[10] HAUPT R,MINKOV M,ASTIGARRAGA I,et al.Langerhans cell histiocytosis(LCH):guidelines for diagnosis,clinical work-up,and treatment for patients till the age of 18 years[J].Pediatr Blood Cancer,2013,60:175-184.
[11] PERRY A,GRAFFIEO C S,MACELLINO C,et al.Pediatric Pituitary Adenoma:Case Series,Review of the Literature,and a Skull Base Treatment Paradigm[J].J Neurol Surg B Skull Base,2018,79:91-114.
[12] CHOBY G W,MATTOS J L,HUGHES M A,et al.Delayed Nasoseptal Flaps for Endoscopic Skull Base Reconst,ruction:Proof of Concept and Evaluation of Outcomes[J].Otolaryngol Head Neck Surg,2015,152:255-259.
[13] CASLER J D,DOOLITTLE A M,MAIR E A.Endoscopic surgery of the anterior skull base[J].Laryngoscope,2005,15:16-24.
[14] MCCOUL E D,ANAND V K,SCHWARTZ T H.Improvements in site-specific quality of life 6 months after endoscopic anterior skull base surgery:a prospective study[J].J Neurosurg,2012,117:498-506.
[15] 张秋航.鼻内镜颅底外科的适应症[J].中国医学文摘(耳鼻咽喉科学),2006,21(2):76-78.
[16] 李大伟,张庆丰,李梅.影像导航引导经鼻内镜在鼻颅底肿瘤切除中的应用[J].临床耳鼻咽喉头颈外科杂志,2015,29(3):249-251.
[17] LOCATELLI D,MASSIMI L,RIGANTE M,et al.Endoscopic endonasal transsphenoidal surgery for sellar tumors in children[J].Int J Pediatr Otorhinolaryngol,2010,74:1298-1302.
-
计量
- 文章访问数: 197
- PDF下载数: 60
- 施引文献: 0