Department of Endocrinology, The First Medical Center of Chinese PLA General Hospital, Beijing, China.
Department of Endocrinology, General Hospital of Northern Theater Command, Shenyang, China.
Front Endocrinol (Lausanne). 2023 May 17;14:1181929. doi: 10.3389/fendo.2023.1181929. eCollection 2023.
Castleman Disease (CD) is a group of diseases with characteristic lymph node histopathology, characterized by marked enlargement of deep or superficial lymph nodes. Adrenal CD is rarely reported, and an accurate preoperative diagnosis of adrenal CD is difficult.
We report four cases of CD in the adrenal gland confirmed by pathology and review the characteristics of this rare disease, highlighting the necessity of diagnostic evaluation and follow-up of the patients.
All of the patients sought medical advice because of adrenal incidentalomas. No significant abnormalities were presented in the biochemistry or endocrine systems. The imaging suggested a moderate-to-large mass with uneven moderate contrast enhancement of the adrenal region, similar to a pheochromocytoma. All cases were misdiagnosed as pheochromocytomas before operation and finally confirmed by histopathology. Three cases were pathologically diagnosed as hyaline vascular CD, and one case was diagnosed as plasma cell CD. All the patients are alive without recurrence after a median follow-up of 8 years.
The adrenal CD should be considered after excluding pheochromocytoma and malignancy in the adrenal region. The long-term prognosis of patients with complete resection of the mass is excellent.
Castleman 病（CD）是一组具有特征性淋巴结组织病理学的疾病，其特征为深部或浅部淋巴结显著肿大。肾上腺 CD 很少见报道，准确的术前诊断较为困难。
我们报道了 4 例经病理证实的肾上腺 CD，并回顾了这种罕见疾病的特点，强调了对患者进行诊断评估和随访的必要性。
所有患者均因肾上腺意外瘤就诊。生化和内分泌系统无明显异常。影像学提示肾上腺区中等至大肿块，不均匀中等程度对比增强，类似于嗜铬细胞瘤。所有病例术前均误诊为嗜铬细胞瘤，最终经组织病理学证实。3 例病理诊断为透明血管型 CD，1 例诊断为浆细胞型 CD。所有患者在中位 8 年的随访中均存活，无复发。
在排除肾上腺嗜铬细胞瘤和恶性肿瘤后应考虑肾上腺 CD。肿块完全切除患者的长期预后良好。
