表现为颈部肿块的Castleman病

Castleman’s Disease Presenting as a Neck Mass

目的探讨表现为颈部肿块的Castleman病(Castleman disease,CD)的临床特点和诊治方法。方法报道1例并总结分析39例颈部CD患者的临床资料。结果颈部CD临床极为少见,10岁以下儿童更为罕见。临床主要表现为颈中上部无痛肿块,易与头颈部常见疾病混淆造成误诊甚至误治。该病的CT和MRI影像表现有一定特点。该病穿刺活检一般难以确诊,术中冷冻切片有较高诊断价值,最终确诊依靠术后常规病检。其病理类型多为透明血管型,彻底的手术切除效果良好。结论对于初步排除颈部常见疾病又疑为颈部CD的患者,应该先取活检,如被冷冻切片结果证实,再行彻底手术切除。彻底手术切除是治疗颈部CD最为有效的方法,术后随访极少复发。

Objective To investigate the clinical features of Castleman＇ s disease （CD） presenting as a neck mass and explore its diagnostic/therapeutic methods. Methods A case of CD in the neck was reported and the clinical data of 39 similar cases were analyzed. Results CD in the neck is extremely rare, especially in children under the age of ten. It is mainly manifested as a painless mass in the middle or upper neck. Since it is easily confused with some common head and neck diseases, misdiagnosis and even mistreatment are often caused. The CT and MRI images of the disease display some specific characteristics. Aspiration biopsy is usually insufficient for final diagnosis of this disease. The diagnostic value of intraoperative frozen section analysis is higher. Final diagnosis of this disease is generally achieved through postoperative pathological examination. Hyaline-vascular type is the more frequently encountered pathological type. Complete surgical resection shows good efficacy. Conclusion If patients are preliminarily excluded to be common neck diseases and suspected to be CD in the neck, biopsy examination should be first conducted. If the diagnostic results from frozen section analysis could confirm this disease, complete surgical resection should be performed. Complete surgical resection is the most effective way to treat CD in the neck since rare recurrence is observed in the postoperative follow-up.