Intrapulmonary unicentric Castleman’s Disease: A clinical analysis of 8 patients

Linling Jin; Yiting He; Chenyang Liu; Songtao Liu; Shuying Ma; Nan Li; Hui Kong; Weiping Xie; Mengyu He

doi:10.36141/svdld.2026.18073

Intrapulmonary unicentric Castleman’s Disease: A clinical analysis of 8 patients

Authors

Linling Jin Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
Yiting He Department of Respiratory and Critical Care Medicine, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu, China
Chenyang Liu Department of Respiratory and Critical Care Medicine, Affiliated People's Hospital of Jiangsu University, Zhenjiang, Jiangsu, China
Songtao Liu Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
Shuying Ma Department of Pathology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
Nan Li Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
Hui Kong Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
Weiping Xie Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China
Mengyu He Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China https://orcid.org/0000-0002-2830-8416

Keywords:

Unicentric Castleman's disease, Intrapulmonary lesion, Clinical features, Surgical resection

Abstract

Background and aims: Castleman’s disease (CD) is a rare, chronic, lymphoproliferative disorder, increasingly recognized as a clonal neoplasm of lymph node stromal cells that can affect multiple organs and tissues. Intrapulmonary unicentric Castleman’s disease (UCD) represents an exceptionally uncommon subtype, primarily involving the lung parenchyma and hilum, with only a few pathologically confirmed cases reported to date. This study aimed to characterize the clinical, radiological, and pathological features of intrapulmonary UCD and to summarize diagnostic and therapeutic experiences.

Methods: We retrospectively analyzed eight patients diagnosed with intrapulmonary UCD. Clinical manifestations, laboratory data, radiologic and bronchoscopic findings, histopathological characteristics, treatment approaches, and follow-up outcomes were systematically reviewed.

Results: No patient exhibited specific clinical symptoms or distinctive laboratory abnormalities. Chest computed tomography (CT) served as the optimal imaging modality, consistently revealing solitary, well-circumscribed, soft-tissue masses with smooth lobulated contours. Definitive diagnosis relied on histopathological examination. All patients underwent complete surgical resection, followed by regular postoperative surveillance with chest CT. During follow-up, no local recurrence or disease progression was detected.

Conclusions: Intrapulmonary UCD is an exceedingly rare form of Castleman’s disease that can be easily misdiagnosed as primary lung tumors, lymphoma, granulomatous disease, or tuberculosis due to its nonspecific clinical and radiological features. Complete surgical excision remains the mainstay of treatment and is associated with an excellent prognosis following complete resection.

References

1. Wang S, Wang R, Shang P, et al. Whole-exome sequencing reveals the genomic profile and IL6ST variants as a prognostic biomarker of paraneoplastic pemphigus-associated unicentric Castleman disease. J Invest Dermatol. 2024;144(3):585–92. doi:10.1016/j.jid.2023.07.031

2. Inada K, Hamazaki M. Localized mediastinal lymph-node hyperplasia resembling thymoma; a case report. Ann Surg. 1958;147(3):409–13. doi:10.1097/00000658-195803000-00018

3. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135(16):1353–64. doi:10.1182/blood.2019000931

4. Kligerman SJ, Auerbach A, Franks TJ, et al. Castleman disease of the thorax: clinical, radiologic, and pathologic correlation: from the radiologic pathology archives. Radiographics. 2016;36(5):1309–32. doi:10.1148/rg.2016160076

5. Hoffmann C, Hentrich M, Tiemann M, et al. Recent advances in Castleman disease. Oncol Res Treat. 2022;45(11):693–704. doi:10.1159/000526640

6. Liu Y, Chen G, Qiu X, et al. Intrapulmonary unicentric Castleman disease mimicking peripheral pulmonary malignancy. Thorac Cancer. 2014;5(6):576–80. doi:10.1111/1759-7714.12129

7. AlSheikh S, Altoijry A, Al-Mubarak H, et al. A rare presentation of unicentric Castleman's disease in the thigh: a case report and review of literature. World J Clin Cases. 2024;12(19):4003–9. doi:10.12998/wjcc.v12.i19.4003

8. Fuentes GC, López SS, González AS, et al. Unicentric Castleman's disease mimicking an hilar lung cancer. Cir Esp. 2023;101(4):298–300. doi:10.1016/j.cireng.2022.06.004

9. Pertusa Mataix R, Loaiza Cabello D, García Morillo JS. Castleman's disease, pathophysiology, advances in diagnosis and treatment. Med Clin (Barc). 2024;162(6):283–90. doi:10.1016/j.medcli.2023.10.013

10. Dispenzieri A, Armitage JO, Loe MJ, et al. The clinical spectrum of Castleman's disease. Am J Hematol. 2012;87(11):997–1002. doi:10.1002/ajh.23291

11. Haap M, Wiefels J, Horger M, et al. Clinical, laboratory and imaging findings in Castleman's disease - the subtype decides. Blood Rev. 2018;32(3):225–34. doi:10.1016/j.blre.2017.11.005

12. Hu S, Li Z, Wang H, et al. Clinical features and treatment outcomes of Castleman disease in children: a retrospective cohort in China. Eur J Pediatr. 2023;182(12):5519–30. doi:10.1007/s00431-023-05235-2

13. Pitot MA, Tahboub Amawi AD, Alexander LF, et al. Imaging of Castleman disease. Radiographics. 2023;43(8):e220210. doi:10.1148/rg.220210

14. He L, Chen Y, Tan X, et al. (18)F-FDG PET/CT and contrast-enhanced CT in the diagnosis of Castleman disease. Jpn J Radiol. 2023;41(1):98–107. doi:10.1007/s11604-022-01318-6

15. Poletti V, Petrarulo S, Piciucchi S, et al. EBUS-guided cryobiopsy in the diagnosis of thoracic disorders. Pulmonology. 2024;30(5):459–65. doi:10.1016/j.pulmoe.2023.11.008

16. Pertusa Mataix R, Loaiza Cabello D, García Morillo JS. Castleman's disease, pathophysiology, advances in diagnosis and treatment. Med Clin. 2024;162(6):283–90. doi:10.1016/j.medcli.2023.10.013

17. Molacek J, Treska V, Skalicky T, et al. Unicentric form of Castlemans disease, pitfalls of diagnosis and surgical treatment. Front Oncol. 2023;13:1057683. doi:10.3389/fonc.2023.1057683

18. Mitsos S, Stamatopoulos A, Patrini D, et al. The role of surgical resection in unicentric Castleman's disease: a systematic review. Adv Respir Med. 2018;86(1):36–43. doi:10.5603/ARM.2018.0008