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Case Reports
. 2023 May 17:14:1181929.
doi: 10.3389/fendo.2023.1181929. eCollection 2023.

Analysis of characteristics of four patients with adrenal unicentric Castleman disease

Hao Yu  1   2 , Yuepeng Wang  1   2   3 , Yijun Li  1 , Jin Du  1 , Qinghua Guo  1 , Weijun Gu  1 , Zhaohui Lyu  1 , Jingtao Dou  1 , Yiming Mu  1 , Li Zang  1
Affiliations
Case Reports

Analysis of characteristics of four patients with adrenal unicentric Castleman disease

Hao Yu et al. Front Endocrinol (Lausanne). .

Abstract

Background: 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.

Method: 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.

Results: 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.

Conclusion: 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.

Keywords: adrenal gland; adrenal incidentaloma; castleman disease; lymphoma; pheochromocytoma.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Magnetic resonance imaging of the Adrenal CD of Case 1 (white arrow). (A) Axial T1WI; (B) Axial T2WI; (C) DWI; (D) Enhanced T1WI (cortex phase); (E) Enhanced T1WI (delayed phase); (F) Coronal Enhanced T1WI. MRI scan and dynamic enhancement showed an 87 × 79 × 85 mm mass with partial hyper-intensity on T2WI and mild to moderate heterogeneous enhancement at the edge and septum of the lesion in the right adrenal region.
Figure 2
Figure 2
Magnetic resonance imaging of the Adrenal CD of Case 2 (white arrow). (A) Axial T1WI; (B) Axial T2WI; (C) Enhanced T1W1; (D) Coronal Enhanced T1W1. MRI scan and dynamic enhancement showed a 46× 33× 40 mm mass with hypo-intensity signal on T1WI and hyper-intensity on T2WI and with mild to moderate heterogeneous enhancement in the left adrenal region.
Figure 3
Figure 3
Adrenal CD, plasma cell variant histopathology in case 1. (A-C) Atretic germinal centers were traversed by sclerotic penetrating vessels and hyalinization, like lollipop follicles. Mantle zones were thickened with lymphocytes arranged in layers. Mantle zones may fuse and contain more than one germinal center, and follicular dendritic cells showed dysplastic features. Interfollicular areas and medulla contain sheets of small, mature plasma cells; (D) Clusters of plasma cells in the interfollicular areas were positive for CD138; (E) Immunohistochemical stain for CD38 identifies plasma cells; (F) The CD20 staining showed the expanded mantle zones.
See this image and copyright information in PMC

References

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