A diagnostic and therapeutically challenging presentation of unicentric mesenteric Castleman disease: A case report

Hua,Lixin; Yin,Zhibin; Yang,Ruirui

doi:10.3892/etm.2025.12830

A diagnostic and therapeutically challenging presentation of unicentric mesenteric Castleman disease: A case report

Authors:
- Lixin Hua
- Zhibin Yin
- Ruirui Yang
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Affiliations: Department of General Surgery, Affiliated Huishan Hospital of Xinglin College, Nantong University (Wuxi Huishan District People's Hospital), Wuxi, Jiangsu 214000, P.R. China, Department of Science and Education, Affiliated Huishan Hospital of Xinglin College, Nantong University, Wuxi, Jiangsu 214000, P.R. China
Published online on: February 21, 2025 https://doi.org/10.3892/etm.2025.12830
Article Number: 80

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Abstract

Castleman disease (CD) is a rare lymphoproliferative disorder primarily manifesting as either Multicentric CD (MCD) or Unicentric CD (UCD), with Unicentric Mesenteric CD (UMCD) representing a less common subtype within the UCD category. The present study presented an encounter with a 29‑year‑old male patient afflicted by UMCD, presenting with significant morbidity attributed to a sizable mesenteric mass. The diagnostic and therapeutic management of this condition posed notable challenges. In the absence of any additional abnormalities detected in auxiliary examinations, a distinct soft tissue density lesion in the abdominal region was revealed by computed tomography (CT). Despite the patient's reluctance to pursue further diagnostic procedures such as fine needle aspiration, a surgical approach was adopted under the suspicion of malignancy to establish a definitive diagnosis and implement treatment, confirming the condition as UMCD. Subsequent adjuvant chemotherapy was performed postoperatively. Fortunately, the patient achieved complete recovery, with no tumor recurrence observed during the 5‑year follow‑up period post‑surgery. Due to the special location of UMCD, its preoperative diagnosis posed challenges and the most effective treatment remains a topic of debate. The prevalent instances of delayed diagnosis and misdiagnosis underscore a deficiency in comprehending the etiology and features of the disease, essential for advancing novel therapeutic strategies. CT imaging and pathological examination both play a crucial role in UMCD diagnosis. The present study supported surgery as the primary treatment modality for UMCD, with chemotherapy and immunotherapy offering additional benefits for appropriately selected patients.

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1	Masaki Y, Kawabata H, Fujimoto S, Kawano M, Iwaki N, Kotani T, Nakashima A, Kurose N, Takai K, Suzuki R and Aoki S: Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan. J Clin Exp Hematop. 59:175–178. 2019.PubMed/NCBI View Article : Google Scholar
2	Castleman B, Iverson L and Menendez VP: Localized mediastinal lymphnode hyperplasia resembling thymoma. Cance. 9:822–830. 1956.PubMed/NCBI View Article : Google Scholar
3	Dispenzieri A and Fajgenbaum DC: Overview of Castleman disease. Blood. 135:1353–1364. 2020.PubMed/NCBI View Article : Google Scholar
4	Simpson D: Epidemiology of Castleman disease. Hematol Oncol Clin North Am. 32:1–10. 2018.PubMed/NCBI View Article : Google Scholar
5	Oksenhendler E, Boutboul D, Fajgenbaum D, Mirouse A, Fieschi C, Malphettes M, Vercellino L, Meignin V, Gérard L and Galicier L: The full spectrum of Castleman disease: 273 Patients studied over 20 years. Br J Haematol. 180:206–221. 2018.PubMed/NCBI View Article : Google Scholar
6	Cohen AB, Swaminathan A, Wang X, Zamora S, Repucci MA, Kelly J, Hannan MD, Sepler D, Sridharma S, Orlando A, et al: Clinical characteristics, treatment patterns, and overall survival of real-world patients with idiopathic multicentric Castleman disease. J Clin Oncol. 39 (Suppl 15)(S7048)2021.
7	Boutboul D, Fadlallah J, Chawki S, Fieschi C, Malphettes M, Dossier A, Gérard L, Mordant P, Meignin V, Oksenhendler E and Galicier L: Treatment and outcome of Unicentric Castleman disease: A retrospective analysis of 71 cases. Br J Haematol. 186:269–273. 2019.PubMed/NCBI View Article : Google Scholar
8	Wojtyś M, Piekarska A, Kunc M, Ptaszyński K, Biernat W, Zaucha JM, Waloszczyk P, Lisowski P, Kubisa B and Grodzki T: Clinicopathological comparison and therapeutic approach to Castleman disease-a case-based review. J Thorac Dis. 11:4859–4874. 2019.PubMed/NCBI View Article : Google Scholar
9	Muhammad T, Alkheder A, Mazloum A, Almooay A, Naziha L and Shaheen M: Unicentric Castleman disease: A case report of an atypical presentation and successful management. Int J Surg Case Rep. 118(109688)2024.PubMed/NCBI View Article : Google Scholar
10	Talat N, Belgaumkar AP and Schulte KM: Surgery in Castleman's disease: A systematic review of 404 published cases. Ann Surg. 255:677–684. 2012.PubMed/NCBI View Article : Google Scholar
11	Hu S, Li Z, Wang H, Chen L, Ma Y, Zhu X, Li J, Dong R, Yao W, Dong C, et al: Clinical features and treatment outcomes of Castleman disease in children: A retrospective cohort in China. Eur J Pediatr. 182:5519–5530. 2023.PubMed/NCBI View Article : Google Scholar
12	van Rhee F, Wong RS, Munshi N, Rossi JF, Ke XY, Fosså A, Simpson D, Capra M, Liu T, Hsieh RK, et al: Siltuximab for multicentric Castleman's disease: A randomised, double-blind, placebo-controlled trial. Lancet Oncol. 15:966–974. 2014.PubMed/NCBI View Article : Google Scholar
13	Zhang L, Zhao AL, Duan MH, Li ZY, Cao XX, Feng J, Zhou DB, Zhong DR, Fajgenbaum DC and Li J: Phase 2 study using oral thalidomide-cyclophosphamide-prednisone for idiopathic multicentric Castleman disease. Blood. 133:1720–1728. 2019.PubMed/NCBI View Article : Google Scholar
14	Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC and Bower M: Castleman disease. Nat Rev Dis Primers. 7(84)2021.PubMed/NCBI View Article : Google Scholar
15	Mitsos S, Stamatopoulos A, Patrini D, George RS, Lawrence DR and Panagiotopoulos N: The role of surgical resection in Unicentric Castleman's disease: A systematic review. Adv Respir Med. 86:36–43. 2018.PubMed/NCBI View Article : Google Scholar
16	Zhang MY, Jia MN, Chen J, Feng J, Cao XX, Zhou DB, Fajgenbaum DC, Zhang L and Li J: UCD with MCD-like inflammatory state: Surgical excision is highly effective. Blood Adv. 5:122–128. 2021.PubMed/NCBI View Article : Google Scholar
17	van Rhee F, Oksenhendler E, Srkalovic G, Voorhees P, Lim M, Dispenzieri A, Ide M, Parente S, Schey S, Streetly M, et al: International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Adv. 4:6039–6050. 2020.PubMed/NCBI View Article : Google Scholar
18	Abraham SS, Narayanan G, Thambi SM, Vasudevan JA, Joy Philip DS, Purushothaman PN, Nair SG and Nair R: Castleman disease: Experience from a single institution. Med Int (Lond). 3(56)2023.PubMed/NCBI View Article : Google Scholar
19	Mohan M, Meek JC, Meek ME, Broadwater R, Alapat D and van Rhee F: Combinatorial treatment for unresectable unicentric Castleman disease. Eur J Haematol. 107:484–488. 2021.PubMed/NCBI View Article : Google Scholar
20	Zhu SH, Yu YH, Zhang Y, Sun JJ, Han DL and Li J: Clinical features and outcome of patients with HIV-negative multicentric Castleman's disease treated with combination chemotherapy: A report on 10 patients. Med Oncol. 30(492)2013.PubMed/NCBI View Article : Google Scholar
21	Seo HY, Kim EB, Kim JW, Shin BK, Kim SJ and Kim BS: Complete remission in a patient with human herpes virus-8 negative multicentric Castleman disease using CHOP chemotherapy. Cancer Res Treat. 41:104–107. 2009.PubMed/NCBI View Article : Google Scholar
22	van Rhee F and Stone K: Storming the Castle with TCP. Blood. 133:1697–1698. 2019.PubMed/NCBI View Article : Google Scholar
23	Kaneko H, Ohkawara T, Aragane H, Ohkawara Y and Taniwaki M: Clinicopathological analysis of a case with mesenteric solitary Castleman's disease: Diagnostic value of radiological findings. Int Surg. 92:272–275. 2007.PubMed/NCBI