19例以头颈部肿块为主要表现的木村病的临床诊治

Clinical diagnosis and treatment of Kimura’s disease mainly presenting with head and neck masses: A report of 19 cases

摘要:

目的 探讨分析木村病(KD)的临床特征及诊治经过，为临床诊疗提供参考。方法 回顾性分析了湖南省人民医院、南华大学附属南华医院2006年1月—2024年6月收治的19例经病理检查诊断为KD患者的病史、实验室检查、影像学检查、治疗方案以及预后，并通过Pubmed查阅相关文献了解KD研究的最新进展。19例患者临床表现为头颈部肿块，部分患者合并其他部位肿块，包括腋窝淋巴结、锁骨下淋巴结、腹股沟淋巴结以及上臂肿块。外周血嗜酸性粒细胞比例或计数增高13例；19例患者行超声检查，表现为边界清楚的低回声或无回声肿块；18例患者行CT检查，表现为均匀低密度肿块，边界清，呈均匀轻、中度强化。18例患者行手术治疗，1例患者行糖皮质激素+环磷酰胺治疗。结果 术后患者均经病理确诊，病理组织学表现为大量嗜酸性粒细胞浸润、嗜酸性微脓肿形成、淋巴滤泡增生、滤泡间区扩大以及滤泡溶解。随访1~10年，19例患者中6例在原切口部位存在肿块复发(31.6%)，其中5例予以再次手术治疗，均得到控制，其中1例患者辅以放疗，总剂量30 Gy，随访3年未复发；1例患者糖皮质激素+环磷酰胺治疗2周后肿块消退，停药5个月后复发，予以手术治疗后随访4年未复发。所有患者均未因复发而死亡。结论 木村病是一种外周血嗜酸性粒细胞升高、血清免疫球蛋白E(IgE)升高的辅助型T细胞 2(Th2)型炎症疾病，表现为无痛性肿块，以头颈部最为常见。治疗方案以手术治疗为主，KD具有术后易复发的特性，术后辅以放疗可减少复发。复发患者可予以糖皮质激素及免疫治疗。针对Th2型炎症细胞因子的生物制剂可能为KD的治疗带来突破。

Abstract:

Objective To analyze the clinical characteristics of Kimura’s disease (KD), and to explore the effective measures for the diagnosis and treatment of the disease. Methods A retrospective analysis was conducted on the medical history, laboratory tests, imaging examinations, treatment plans and prognosis of 19 patients, who were diagnosed by KD by pathological examination. The 19 patients were admitted to Nanhua Hospital Affiliated to University of South China from January 2006 to June 2024. Relevant literatures were reviewed through Pubmed to understand the latest progress in KD research. The clinical manifestations of 19 patients were head and neck masses. Some patients have masses in other parts of the body, including axillary lymph node, subclavian lymph node, inguinal lymph node and upper arm mass. The proportion or count of eosinophils in peripheral blood increased in 13 cases. Ultrasound examinations were performed on 19 patients, showing a low-echo or anechoic mass with clear boundaries. CT examinations were performed in 18 patients, presenting a uniform low-density mass with clear boundaries and uniform mild to moderate enhancement. Eighteen of the 19 patients underwent surgical treatment, and one of the 19 patients received glucocorticoid + cyclophosphamide treatment. Results All patients were pathologically diagnosed after the operation. Histopathological features were massive eosinophil infiltration, eosinophilic micro-abscess formation, lymphoid follicle hyperplasia, enlargement of interfollicular area, and follicular lyses. Nineteen patients were followed up for 1 to 10 years. There were 6 cases of the 19 patients(31.6%) with tumor recurrence at the original incision site. Five cases of the tumor recurrence were treated with reoperation, all of whom were controlled. One case of the reoperation received adjuvant radiotherapy with a total dose of 30 Gy, who was no recurrence after a follow-up of 3 years. One patient’s mass subsided after 2 weeks of treatment with glucocorticoids and cyclophosphamide. The mass recurred 5 months after drug withdrawal. Then the patient underwent a surgical treatment without recurrence during a 4-year follow-up. None of the patients died due to recurrence. Conclusions KD is a T helper 2 cell (Th2) type inflammatory disease characterized by increased peripheral blood eosinophils and elevated serum immunoglobulin E (IgE) levels, presenting as painless masses, most commonly in the head and neck region. The treatment mainly involves surgical intervention, but KD has a tendency to recur postoperatively; adjuvant radiotherapy can help reduce recurrence. In patients with recurrence, corticosteroids and immunotherapy may be administered. Biologics targeting Th2 type inflammatory cytokines may offer breakthroughs in the treatment of KD.