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Year : 2022  |  Volume : 27  |  Issue : 1  |  Page : 109-111

Adrenal castleman's disease: Case report and review of literature

1 Department of Surgical Oncology, Cancer Institute (WIA), Chennai, Tamil Nadu, India
2 Department of Surgical Oncology, Fortis Malar Hospital, Chennai, Tamil Nadu, India

Date of Submission14-Aug-2020
Date of Decision22-Sep-2020
Date of Acceptance14-Sep-2021
Date of Web Publication11-Jan-2022

Correspondence Address:
Dr. Anand Raja
Department of Surgical Oncology, Cancer Institute (WIA), No. 38 Sardar Patel Marg, Adyar, Chennai - 600 020, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_282_20

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Castleman's disease is a rare lymphoproliferative disorder of poorly understood etiology. It is most commonly located in the mediastinum. Castleman's disease mimicking adrenal neoplasm is a very rare differential diagnosis and unusual presentation. We report a rare suprarenal unicentric hyaline vascular variant of the disease in a 16-year-old boy with atypical symptoms mimicking an adrenal neoplasm.

Keywords: Adrenal gland neoplasm, Castleman's disease, lymphoproliferative disorder

How to cite this article:
Raja A, Malik K, Mahalingam S. Adrenal castleman's disease: Case report and review of literature. J Indian Assoc Pediatr Surg 2022;27:109-11

How to cite this URL:
Raja A, Malik K, Mahalingam S. Adrenal castleman's disease: Case report and review of literature. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2023 Jan 31];27:109-11. Available from: https://www.jiaps.com/text.asp?2022/27/1/109/335566

   Introduction Top

Castleman's disease is a rare disorder, first defined by Castleman in 1956 as a distinct clinicopathological entity.[1] Two-thirds are located in the mediastinum and rest occur at extrathoracic locations such as axilla, neck, groin, mesentery, and retroperitoneal space. It is classified based on the clinical presentation and histopathological variant. The clinical subtypes are unicentric and multicentric. The histopathological variants are hyaline vascular, plasma cell, and mixed type.[2] We hereby report a case of hyaline vascular variant at the adrenal gland.

   Case Report Top

A 16-year-old boy presented with dull pain in the left upper abdomen with weight loss of 10 kg over 4 months. He had no significant past medical or surgical history. Physical examination revealed nontender, soft abdomen, without palpable mass or peripheral lymphadenopathy. Contrast enhanced computer tomography (CECT) scan of chest and abdomen showed a lobulated, well-circumscribed, enhancing mass of 53 mm × 51 mm × 55 mm with necrotic area, occupying the left suprarenal region [Figure 1]. There is no significant mediastinal mass or retroperitoneal lymph nodes. Laboratory tests and 24-h urine vanillylmandelic acid and metanephrines and plasma metanephrines were normal. He was taken up for surgery with a provisional diagnosis of nonfunctioning adrenal neoplasm. Intraoperatively, a mass of 5.5 cm × 5 cm × 6 cm was located in the left suprarenal region with multiple feeding vessels from the aorta and the left renal artery. Adrenal gland was not separately made out from the mass and it was removed en bloc. Grossly, the mass was well encapsulated with grayish white cut surface area. Histopathology revealed lymphoid follicles with thickened mantle zones and lymphocytes arranged in concentric rings [Figure 2]. There was vascular proliferation and hyalinization of vessel wall, based on which diagnosis of hyaline vascular Castleman's disease was made. His postoperative recovery was uneventful and no adjuvant therapy was considered. He is on regular follow-up without recurrence and disease free at 6 months.
Figure 1: Contrast enhanced computed tomography of abdomen showing lobulated, circumscribed, enhancing left suprarenal mass with area of necrosis. Adrenal gland is not separately seen

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Figure 2: Photomicroscopy shows lymphoid follicles arranged in a concentric fashion with hyalinization and adjacent hyalinized blood vessels. (Hematoxylin and Eosin, ×10)

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   Discussion Top

Castleman's disease is a rare neoplasm occurring most commonly in the mediastinum. Only 12% cases occur in the abdomen of which only 12 cases have been reported previously in the adrenal region [Table 1].[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] The etiology of Castleman's disease is poorly understood with one possible hypothesis being a defect in the immune regulation.[2]
Table 1: Reported cases of adrenal region Castleman's disease

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The age of presentation in suprarenal disease has ranged from 16 years to 65 years without gender predilection. The most common presenting symptom for unicentric disease in suprarenal region has been abdominal pain although many have presented as incidentalomas.[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] In our case, the patient is a 16-year-old male and presented with weight loss and abdomen pain which is an uncommon presentation for the localized unicentric hyaline vascular type Castleman's disease. In view of age, symptoms and high geographical burden, a differential diagnosis of tuberculosis was also kept, however, majority of patients with active or recently acquired tubercular disease (<2 years) have bilateral adrenal enlargement, while calcification and atrophy are visualized in more remote infection or inactive disease.[14]

Although imaging plays a key role in the identification of adrenal masses, it may not be conclusive in identifying Castleman's disease. Various authors have reported features of well-defined enhancing solid mass with homogenous soft tissue density, calcified lesion or with multiple lymph nodes on CECT.[10] Our patient had a heterogenous mass with necrotic areas. One author has described MR features of adrenal disease as hypointense in T1 and hyperintense in T2 with a delayed washout although they emphasized that similar features can be seen in lymphoma also.[3]

The hyaline vascular type accounts for 80%–90% of localized disease and is usually asymptomatic but may cause pressure effect due to large size and is rarely associated with systemic features.[4] As in mediastinum, three different disease patterns are seen in suprarenal region also: solitary noninvasive mass; a dominant infiltrative mass with associated lymphadenopathy; matted lymphadenopathy without a dominant mass.[15] In other sites, a solitary noninvasive mass is the most common presentation, but in the suprarenal region, 50% patients had adrenal gland infiltration. In our case, the mass was 6 cm with lobulations but well encapsulated without any infiltration of adjacent structures. Such findings are also seen in other adrenal malignancies also.

Castleman's disease involving a single site is usually benign, however, multicentric variety can transform into malignant lymphoma.[2] The treatment of unicentric hyaline vascular Castleman's disease is complete surgical excision which is curative.[13] Our case of 16-year-old boy with symptomatic presentation and uncommon computed tomography findings illustrates a divergent presentation of this rare pathology and need for histopathological confirmation.

   Conclusion Top

The unicentric hyaline vascular variety of Castleman's disease masquerading as an adrenal neoplasm is a very rare presentation. It is to be considered in the differential diagnosis of nonfunctional adrenal neoplasm.

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   References Top

Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer 1956;9:822-30.  Back to cited text no. 1
Henry K & Symmers WSC. Systemic Pathology: Thymus, Lymph Nodes, Spleen and Lymphatics. Vol. 7. Edinburgh: Churchill Livingstone. 1992. p. 207-18.  Back to cited text no. 2
Debatin JF, Spritzer CE, Dunnick NR. Castleman disease of the adrenal gland: MR imaging features. AJR Am J Roentgenol 1991;157:781-3.  Back to cited text no. 3
Gupta NP, Ansari MS, Chopra P, Dinda AK. Castleman's disease masquerading as an adrenal tumor. J Urol 2002;168:2524.  Back to cited text no. 4
Chen JH, Yu CY, Pai CY, Chan DC, Chen CJ, Yu JC, et al. Castleman's disease in the left upper retroperitoneal space mimicking an adrenal neoplasm: Report of a case and literature review. Jpn J Clin Oncol 2005;35:353-6.  Back to cited text no. 5
Mussig K, Horger M, Wehrmann M. Adrenal castleman's disease. Ann Hematol 2007;86:63-5.  Back to cited text no. 6
Santomauro M, Choe C, Heimbigner J, Roberts J, Auge B. Castleman's disease in the left suprarenal region, mimicking an adrenal neoplasm. Urology 2011;78:319.  Back to cited text no. 7
Otto M, Wieprzowski T, Dzwonkowski J, Ziakiewicz-Wroblewska B. Castleman's disease – An unusual indication for laparoscopic adrenalectomy. Wideochir Inne Tech Maloinwazyjne 2012;7:50-4.  Back to cited text no. 8
Cao DH, Liu LR, Xu RH, Jiang D, Lv X, Yuan HC, et al. Retroperitoneal laparoscopic management method of Castleman's disease in the adrenal gland: Two cases report and review of the literature. Int J Clin Exp Med 2014;7:5909-12.  Back to cited text no. 9
Hong SB, Lee NM, Kim S, Han GJ, Ha HK, Ku JY et al. Adrenal Castleman's disease mimicking other adrenal neoplasms: A case report. J Korean Soc Radiol 2017;76:73-7.  Back to cited text no. 10
Sundar P, Bijalwan P, Pooleri GK. Castleman's disease: A suprarenal surprise! Indian J Surg Oncol 2018;9:254-5.  Back to cited text no. 11
Chen J, Yang C, Liang C. Detection of a unicentric type of castleman-like mass at the site of adrenal gland: A case report and review of literature. World J Clin Cases 2018;6:683-7.  Back to cited text no. 12
Guo L, Sang Z, Du J, Zhang Q, Meng H, Shi B, et al. A case report of adrenal Castleman's disease. SN Compr Clin Med 2019;1:479-82.  Back to cited text no. 13
Kelestimur F. The endocrinology of adrenal tuberculosis: The effects of tuberculosis on the hypothalamo-pituitary-adrenocortical function. J Endocrinol Invest 2004;27:380-6.  Back to cited text no. 14
Meador TL, McLarney JK. CT features of castleman disease of the abdomen and pelvis. AJR Am J Roentgenol 2000;175:115-8.  Back to cited text no. 15


  [Figure 1], [Figure 2]

  [Table 1]

This article has been cited by
1 Case Report: A rare case of†primary hepatic Castlemanís disease†mimicking a liver tumor
Hong Chen, Xiaoxi Pang, Jie Li, Baixuan Xu, Yachao Liu
Frontiers in Oncology. 2022; 12
[Pubmed] | [DOI]


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