Department of Pathology, University Medical Center Göttingen, Germany.
BMC Cancer. 2011 Aug 12;11:352. doi: 10.1186/1471-2407-11-352.
Castleman's disease is a rare form of localized lymph node hyperplasia of uncertain etiology. Although the mediastinum is the most common site of involvement, rare cases occurring in lymph node bearing tissue of other localization have been reported, including only a few intramuscular cases. Unicentric and multicentric Castleman's disease are being distinguished, the latter harboring an unfavorable prognosis.
Here, we present a case of unicentric Castleman's disease in a 37-year-old woman without associated neoplastic, autoimmune or infectious diseases. The lesion was located in the femoral region of the right lower extremity and surgically resected after radiographic workup and excisional biopsy examinations. The tumor comprised lymphoid tissue with numerous germinal centers with central fibrosis, onion-skinning and rich interfollicular vascularization. CD23-positive follicular dendritic cells were detected in the germinal centers and numerous CD138-positive plasma cells in interfollicular areas. The diagnosis of mixed cellularity type Castleman's disease was established and the patient recovered well.
In conclusion, the differential diagnosis of Castleman's disease should be considered when evaluating a sharply demarcated, hypervascularized lymphatic tumor located in the extremities. However, the developmental etiology of Castleman's disease remains to be further examined.
血管滤泡性淋巴结增生症（Castleman 病）是一种病因不明的局部淋巴结增生的罕见形式。尽管纵隔是最常见的受累部位，但也有报道罕见病例发生在其他部位的淋巴结，包括少数肌内病例。血管滤泡性淋巴结增生症分为局灶型和多中心型，后者预后不良。
本文报告了一例 37 岁女性局灶型 Castleman 病，无相关肿瘤、自身免疫或感染性疾病。病变位于右下肢股部，经影像学检查和切除活检后行手术切除。肿瘤由具有大量生发中心的淋巴组织组成，伴有中央纤维化、洋葱皮样和丰富的滤泡间血管化。生发中心检测到 CD23 阳性滤泡树突细胞，滤泡间区有大量 CD138 阳性浆细胞。诊断为混合细胞型 Castleman 病，患者恢复良好。
总之，当评估位于四肢的界限清楚、富含血管的淋巴肿瘤时，应考虑 Castleman 病的鉴别诊断。然而，Castleman 病的发病病因仍有待进一步研究。
