Bonekamp David, Hruban Ralph H, Fishman Elliot K
The Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins University, Baltimore, MD.
Department of Pathology, The Johns Hopkins University, Baltimore, MD.
Semin Ultrasound CT MR. 2014 Jun;35(3):263-71. doi: 10.1053/j.sult.2013.12.005. Epub 2013 Dec 19.
Castleman disease (CD) was first described and most commonly occurs in the form of classic hyaline-vascular-type CD with hyperenhancing localized (unicentric) nonneoplastic lymphadenopathy. CD may affect any body region including unusual locations for isolated lymph node enlargement. This makes CD a great mimicker of more common benign and malignant masses in the neck, chest, abdomen, and pelvis. CD masses commonly raise the suspicion of lymphoma, paraganglioma, neuroendocrine tumor, metastatic adenopathy, solid parenchymal tumors, and infectious or inflammatory diseases. The less common plasma cell-type CD, mixed CD, and human herpes virus-8-associated CD (HHV8-CD) are more prone to lead to generalized (multicentric) adenopathy without the typical nodal hyperenhancement and are associated with B symptoms and hematologic and immunologic manifestations. The generalized forms of CD have a worse prognosis, which can approach that of malignancies. CD has multiple known associations with neoplasms, skin diseases, blood dyscrasias, and immunodeficiency, which include certain forms of lymphoma; paraneoplastic pemphigus; polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes syndrome; and plasma cell dyscrasias. Cytokine overproduction (IL-6) is an important pathogenetic factor in the development of CD. The treatment is accordingly variable, from curative or diagnostic resection of a solitary lesion to systemic chemotherapy or anti-IL-6 therapy.
卡斯特曼病（CD）最早被描述，最常见的表现形式为经典透明血管型CD，伴有强化明显的局限性（单中心）非肿瘤性淋巴结病。CD可累及身体的任何部位，包括孤立性淋巴结肿大的不常见部位。这使得CD极易被误诊为颈部、胸部、腹部和盆腔中更常见的良性和恶性肿块。CD肿块常引发对淋巴瘤、副神经节瘤、神经内分泌肿瘤、转移性淋巴结病、实体实质肿瘤以及感染性或炎性疾病的怀疑。较不常见的浆细胞型CD、混合型CD和人类疱疹病毒8相关型CD（HHV8 - CD）更容易导致全身性（多中心）淋巴结病，且无典型的淋巴结强化，还与B症状以及血液学和免疫学表现相关。CD的全身性形式预后较差，可接近恶性肿瘤。CD与多种肿瘤、皮肤病、血液系统疾病和免疫缺陷存在多种已知关联，其中包括某些形式的淋巴瘤；副肿瘤性天疱疮；多神经病、器官肿大、内分泌病、M蛋白和皮肤改变综合征；以及浆细胞发育异常。细胞因子过度产生（IL - 6）是CD发生发展中的一个重要致病因素。相应地，治疗方法多种多样，从对孤立性病变进行根治性或诊断性切除到全身化疗或抗IL - 6治疗。
