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摘要: 目的 探讨首发于头颈部的Castleman病的临床表现、病理特点、治疗及预后。方法 回顾性分析2007—2021年在南京鼓楼医院诊断和治疗的18例首发于头颈部的Castleman病患者的临床资料，其中单中心型14例，多中心型4例，对其临床特点、治疗及预后进行分析。结果 18例头颈部Castleman病患者中，位于腮腺1例，耳后1例，咽旁间隙1例，颈部Ⅰ区3例，锁骨上2例，颈部Ⅲ区2例，余均多发颈部两个亚区以上。单中心型患者术后复查颈部彩超及头颈部CT均未见肿物复发及进展; 多中心型出现多脏器功能异常，如双下肢水肿、肝脾肿大、咳嗽等。4例多中心型患者仅1例接受化疗，余3例均拒绝化疗，仅接受对症治疗。随访所有患者均存活，但多中心型患者病情明显进展，且累及淋巴结增多，部分患者新发肝脾肿大。结论 头颈部Castleman病多为单中心型，表现为颈部多个无症状肿大淋巴结，手术切除效果好，预后佳。头颈部多中心型Castleman病，临床症状复杂，随时间进展累及多脏器，需后续治疗。
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关键词:
- Castleman病 /
- 头颈部 /
- 临床特征 /
- 预后
Abstract: Objective To investigate the clinicopathological features, treatment and prognosis of Castleman disease in the head and neck.Methods The clinical and pathological data of 18 patients with Castleman disease of the head and neck in Nanjing Drum Tower Hospital from 2007 to 2021 were retrospectively analyzed. There were 14 cases of unicentric type and 4 cases of multicentric type. The clinical characteristics, treatment and prognosis were analyzed.Results Among the 18 cases of Castleman disease in the head and neck, 1 case was located in the parotid gland, 1 case was behind the ear, 1 case was in the parapharyngeal space, 3 cases were in the neck region Ⅰ, 2 cases were supraclavicular, 2 cases were in the neck region Ⅲ, the rest were located in more than two subregions of the neck. In patients with unicentric type, no tumor recurrence and progression were found in the postoperative re-examination with neck Doppler ultrasound and CT; in the multicentric type, multiple organ dysfunction, such as edema of both lower extremities, hepatosplenomegaly, and cough, were found. Of the 4 patients with multicentric type, only 1 patient received chemotherapy, and the remaining 3 patients refused chemotherapy and only received symptomatic treatment. All patients survived during follow-up, but the disease of multicentric patients progressed significantly, and the number of involved lymph nodes increased, and hepatosplenomegaly were found in some patients.Conclusion Castleman disease of the head and neck is mostly unicentric type, which is manifested as multiple asymptomatic enlarged lymph nodes in the neck. The surgical resection is effective and the prognosis is good. Multicentric Castleman disease of the head and neck has complex clinical symptoms and involves multiple organs over time, requiring follow-up treatment.-
Key words:
- Castleman disease /
- head and neck /
- clinical features /
- prognosis
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表 1 UCD组和MCD组患者的一般资料
例(%) UCD组(n=14) MCD组(n=4) 性别 男 6(42.9) 0(0) 女 8(57.1) 4(100.0) 年龄/岁 ≥46 9(64.3) 1(25.0) < 46 5(35.7) 3(75.0) 病理类型 透明血管型 11(78.6) 2(50.0) 浆细胞型 3(21.4) 2(50.0) 混合型 0(0) 0(0) 临床症状 双下肢水肿 0(0) 2(50.0) 肝脾肿大 0(0) 2(50.0) 咳嗽 0(0) 2(50.0) 实验室检查 低白蛋白血症 0(0) 3(75.0) 贫血 1(7.1) 3(75.0) 血沉升高 0(0) 3(75.0) C-反应蛋白升高 1(7.1) 3(75.0) 
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表 2 MCD患者的随访及预后情况
例序 性别 年龄/岁 颈部肿大淋巴结部位 病理类型 诊疗经过 随访时间/月 预后 1 女 37 左侧锁骨上 浆细胞型 至当地医院行化疗 96 疾病进展 2 女 15 左侧胸锁乳突肌前缘 透明血管型 术前PET-CT考虑多中心型，血液科建议行化疗，患者拒绝，后复查PET-CT提示新发肝脾肿大，且累及全身各处淋巴结，累及淋巴结数量增多，体积变大，代谢值增高。 77 疾病进展 3 女 51 耳后 浆细胞型 建议化疗患者拒绝，术后查PET-CT考虑全身多发肿大淋巴结，考虑多为新发肿大淋巴结。 61 疾病进展 4 女 42 左侧锁骨上 透明血管型 建议化疗患者拒绝，复查全身肿大淋巴结较术前明显增大，且部分淋巴结增大超过20%，并且有多发新病灶产生。 40 疾病进展
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