Macedo Joana Espiga, Abreu Isabel, Marques Margarida, Henrique Rui, Araújo António
Onco-hematology Service, Portuguese Institute of Oncology, Oporto, Portugal.
J Thorac Oncol. 2007 Mar;2(3):259-60. doi: 10.1097/JTO.0b013e318031b633.
Angiofollicular lymph node hyperplasia, or Castleman's disease, is a rare disorder involving lymphoid tissue proliferation that was first described by Castleman in 1956. The etiology of Castleman's disease is unclear; many origins have been proposed, such as immunocompromised states, chronic inflammation or infection, and autoimmune processes. The disease has been classified on clinical grounds (unicentric or multicentric) and by histological appearance (hyaline vascular pattern, plasma cell predominance, or mixed lesions). In more than 70% of cases, Castleman's disease presents as a solitary mediastinal or cervical mass with an indolent course. Whereas the unicentric form is usually benign and curative resection is possible, patients with the multicentric form often have systemic symptoms and a clinically more aggressive, malignant course. We report a case of unicentric Castleman's disease and re-emphasize its importance in the differential diagnosis of mediastinal tumors.
血管滤泡性淋巴结增生症，即卡斯尔曼病，是一种涉及淋巴组织增生的罕见疾病，于1956年由卡斯尔曼首次描述。卡斯尔曼病的病因尚不清楚；已提出多种起源，如免疫功能低下状态、慢性炎症或感染以及自身免疫过程。该疾病已根据临床情况（单中心或多中心）和组织学表现（透明血管型、浆细胞为主型或混合性病变）进行分类。在超过70%的病例中，卡斯尔曼病表现为孤立的纵隔或颈部肿块，病程进展缓慢。虽然单中心型通常为良性，可通过手术治愈，但多中心型患者常有全身症状，临床病程更具侵袭性，呈恶性。我们报告一例单中心卡斯尔曼病病例，并再次强调其在纵隔肿瘤鉴别诊断中的重要性。
