Objectives: To explore the clinical characteristics and differential diagnosis for patients with thoracic Castleman's disease, thus to improve doctor's cognition and therapeutic level for the disease.
Methods: The clinical data of 12 patients with thoracic Castleman's disease were analyzed retrospectively, including clinical features, laboratory examination, imaging finding, pathological examination, treatment outcome, and prognosis.
Results: All 12 patients were diagnosed by mass or lymph code biopsy. Seven patients with unicentric Castleman's disease were asymptomatic or oligosymptomatic. Of them, the lung imaging features were mostly solitary and well-defined pulmonary hilar soft tissue mass and infrequently invasive mass. Pathologically, 7 patients were all hyaline vascular type. Six patients underwent surgery of complete mass resection, and 1 received chemotherapy because the nidus could not be resected completely. The prognosis of 7 patients was good. The clinical manifestations of 5 patients with multicentric Castleman's disease were diverse with negative prognosis. Their radiological features mainly showed multiple mediastinal/hilum lymph node enlargement, multiple serous cavity effusion, and hepatosplenomegaly, along with multifocal lymphadenopathy. Pathological features of 3 patients' mass were hyaline vascular type, 1 was plasma cell type, and 1 was mixed type. Three patients received comprehensive treatment on the basis of chemotherapy and another 2 patients did not receive any therapy.
Conclusions: Unicentric Castleman's disease involving the chest is mainly manifested as mediastinal or hilar soft tissue mass with single clinical manifestation, while multicentric Castleman's disease involving the chest is mainly manifested as diffuse mediastinal/hilar lymph node enlargement, multifocal lymphadenopathy and multiple clinical manifestation. For patients with mediastinal/hilum mass or with multifocal lymphadenopathy, Castleman's disease should be considered the differential diagnosis. And early mass or lymph code biopsy is of great significance for diagnosis and treatment of Castleman's disease.
目的: 探讨累及胸部的Castleman病(Castleman’s disease，CD)的临床特点及鉴别诊断方法，以提高对该病的认识和诊治水平。方法: 回顾性分析12例累及胸部的CD患者的临床资料，包括临床表现、实验室检查、影像学、病理学、治疗及预后资料。结果: 12例CD患者均经肿块或淋巴结活体组织检查(以下简称活检)确诊。7例单中心CD型患者临床无症状或少症状，影像学主要表现为孤立、边界清楚的纵隔或肺门软组织肿块影，少见浸润性肿块，病理组织类型均为透明血管型；6例行手术切除肿块治疗，1例因肿块无法完整切除行化学药物治疗(以下简称化疗)，7例患者均预后较好。5例多中心型患者临床表现多样，影像学主要表现为纵隔/肺门多发肿大淋巴结、多浆膜腔积液、肝脾肿大并伴全身其他多部位淋巴结肿大；病理组织类型3例为透明血管型，1例浆细胞型，1例混合型；3例行以化疗为基础的综合性治疗，2例未治疗，5例患者预后不良。结论: 累及胸部的单中心型CD以纵隔或肺门软组织肿块为主要表现，多中心型CD以弥漫性纵隔/肺门淋巴结肿大伴全身多发淋巴结肿大为主要表现。对有纵隔/肺门肿块或有全身多发淋巴结肿大表现者的鉴别诊断应将CD考虑在内，早期肿块或淋巴结活检对CD诊断和治疗具有重要意义。.
Keywords: Castleman’s disease; biopsy; lymphadenopathy.