Castleman Lymphadenopathy Presenting as Axillary Mass in a 69-Year-Old Male: A Case Report with Review of Literature
DOI:
https://doi.org/10.21276/apalm.3537Keywords:
Castleman disease, Lymphadenopathy, Lymph nodes, Lymphoproliferative disorders, HistopathologyAbstract
Castleman disease (CD), first described by Benjamin Castleman, is a rare and heterogeneous disorder of unknown aetiology, characterised by massive lymphadenopathy and a range of systemic inflammatory symptoms. It shows no specific predilection for age, gender, or ethnicity and exhibits distinctive histomorphological features. Depending on the extent of involvement, CD can be classified as either unicentric or multicentric, each with distinct clinical presentations. Unicentric CD (UCD) presents as localised lymphadenopathy, usually without any other symptoms. In contrast, multicentric CD (MCD) is a progressive, systemic inflammatory condition involving multiple lymph node stations and organ systems. Histologically, two main morphological variants are recognised: the hyaline vascular (HV) type and the plasma cell (PC) type.
We herein report a case of a 69-year-old male who presented to our hospital with a painless, gradually enlarging axillary swelling, which, on imaging, appeared as a conglomerate of enlarged lymph nodes. Radiological findings were non-specific, and aspiration cytology was suggestive of Rosai-Dorfman disease.
The diagnosis of CD is generally delayed and fraught with challenges since it is rare and can mimic a broad array of entities, encompassing lymphomas, granulomatous inflammation, autoimmune disorders, among others. Histopathological analysis of the excised lymph node is considered the best modality for diagnosing and distinguishing the various subtypes of CD.
References
1. Castleman B, Towne VW. Case records of the Massachusetts General Hospital: Case No. 40011. N Engl J Med. 1954;250:26–30.
2. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymph-node hyperplasia resembling thymoma. Cancer. 1956;9(4):822–30.
3. Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer. 1972;29(3):670–83.
4. van Rhee F, Oksenhendler E, Srkalovic G, et al. International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease. Blood Adv. 2020;4(23):6039–50.
5. Simpson D. Epidemiology of Castleman disease. Hematol Oncol Clin North Am. 2018;32(1):1–10.
6. Carbone A, Borok M, Damania B, et al. Castleman disease. Nat Rev Dis Primers. 2021;7(1):84.
7. Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646–57.
8. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135(16):1353–64.
9. Murro D, Agab M, Brickman A, Loew J, Gattuso P. Cytological features of Castleman disease: a review. J Am Soc Cytopathol. 2016;5(2):100–6.
10. Murakami M, Johkoh T, Hayashi S, et al. Clinicopathologic characteristics of 342 patients with multicentric Castleman disease in Japan. Mod Rheumatol. 2020;30(5):843–51.
11. Oksenhendler E, Boutboul D, Fajgenbaum D, et al. The full spectrum of Castleman disease: 273 patients studied over 20 years. Br J Haematol. 2018;180(2):206–16.
12. Gopal S, Liomba NG, Montgomery ND, et al. Characteristics and survival for HIV-associated multicentric Castleman disease in Malawi. J Int AIDS Soc. 2015;18(1):20122.
13. Singh A, Purkait S, Mallick S, et al. Clinicopathological profile of Castleman’s disease in Indian population: experience from a tertiary care center. Indian J Hematol Blood Transfus. 2020;36:254–9.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 Prableen Kour, Anuja Sharma, Aneeta Singh Malhotra, Bawana Raina
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access at http://opcit.eprints.org/oacitation-biblio.html).
