Abstract
Dysregulated overproduction of interleukin-6 (IL-6) from activated B cells in affected lymph nodes has been implicated in the pathogenesis of multicentric Castleman’s disease (MCD), a rare lymphoproliferative disorder accompanied by systemic manifestations. We here report the case of a 32-year-old female presenting with MCD associated with a dermoid cyst in the pelvic cavity. The co-occurrence of MCD and dermoid cyst has not been reported before. Immunohistochemical analysis of the tissue sections showed IL-6 production in CD68-positive macrophage cells, which had infiltrated the dermoid cyst. Removal of the cyst resulted in partial improvement in systemic symptoms accompanied by a decrease in serum IL-6, while complete improvement was obtained by treatment with an anti-IL-6 receptor antibody following resection of the dermoid cyst. To the best of our knowledge, this is the first study to provide evidence of IL-6 production by CD68+ cells in a dermoid cyst involved in MCD.
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References
Castleman B, Iverson L, Menendze VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer. 1956;9:822–30.
Keller AR, Hochholzer L, Castleman B. Hyalin-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer. 1972;29:670–83.
Frizzera G, Peterson BA, Bayrd ED, Goldman A. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease. Clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol. 1985;3:1202–16.
Yoshizaki K, Matsuda T, Nishimoto N, Kuritani T, Lee T, Aozasa K, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castelman’s disease. Blood. 1989;74:1360–7.
Nishimoto N, Sasai M, Shima Y, Nakagawa M, Matsumoto T, Shirai T, et al. Improvement in Castelman’s disease by humanized anti-interleukin-6 receptor antibody therapy. Blood. 2000;95:56–61.
Nishimoto N, Kanakura Y, Aozasa K, Johkoh T, Nakamura M, Nakano S, et al. Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman’s disease. Blood. 2005;106:2627–32.
Song SN, Tomosugi N, Kawabata H, Ishikawa T, Nishikawa T, Yoshizaki K. Down-regulation of hepcidin resulting from long-term treatment with an anti-IL-6 receptor antibody (tocilizumab) improves anemia of inflammation in multicentric Castleman disease. Blood. 2010;116:3627–34.
Naresh KN, Rice AJ, Bower M. Lymph nodes involved by multicentric Castleman disease among HIV-positive individuals are often involved by Kaposi sarcoma. Am J Surg Pathol. 2008;32:1006–12.
Molinié V, Diebold J. Hodgkin’s disease associated with localized or multicentric Castleman’s disease. Arch Pathol Lab Med. 1995;119:201.
Larroche C, Cacoub P, Soulier J, Oksenhendler E, Clauvel JP, Piette JC, et al. Castleman’s disease and lymphoma: report of eight cases in HIV-negative patients and literature review. Am J Hematol. 2002;69:119–26.
Curioni S, D’Amico M, Quartagno R, Martino S, Dell’Antonio G, Cusi D. Castleman’s disease with nephrotic syndrome, amyloidosis and autoimmune manifestations. Nephrol Dial Transplant. 2001;16:1475–8.
Tavoni A, Vitali C, Baglioni P, Gerli R, Marchetti G, Di Munno O, et al. Multicentric Castleman’s disease in a patient with primary Sjögren’s syndrome. Rheumatol Int. 1993;12:251–3.
Nanki T, Tomiyama J, Arai S. Mixed connective tissue disease associated with multicentric Castleman’s disease. Scand J Rheumatol. 1994;23:215–7.
Leger-Ravet MB, Peuchmaur M, Devergne O, Audouin J, Raphael M, Van Damme J, et al. Interleukin-6 gene expression in Castleman’s disease. Blood. 1991;78:2923–30.
Beck JT, Hsu SM, Wijdenes J, Bataille R, Klein B, Vesole D, et al. Brief report: alleviation of systemic manifestations of Castleman’s disease by monoclonal anti-interleukin-6 antibody. N Engl J Med. 1994;330(9):602–5.
Zaloude KC. Tumors of ovary. In: Fletcher CDM, editor. Diagnostic histopathology of tumours. 2nd ed. Churchill Livingston: Harcourt Publication Ltd; 2000. p. 611.
Van Dongen JJ, Langerak AW, Brüggemann M, Evans PA, Hummel M, Lavender FL, et al. Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: report of the BIOMED-2 Concerted Action BMH4-CT98–3936. Leukemia. 2003;17:2257–317.
Nishimoto N, Terao K, Mima T, Nakahara H, Takagi N, Kakehi T. Mechanisms and pathologic significances in increase in serum interleukin-6 (IL-6) and soluble IL-6 receptor after administration of an anti IL-6 receptor antibody, tocilizumab, in patients with rheumatoid arthritis and Castleman disease. Blood. 2008;112:3959–64.
Kishimoto T. The biology of interleukin-6. Blood. 1989;74:1–10.
Nishikawa T, Hagihara K, Serada S, Isobe T, Matsumura A, Song J, et al. Transcriptional complex formation of c-Fos, STAT3, and hepatocyte NF-1 alpha is essential for cytokine-driven C-reactive protein gene expression. J Immunol. 2008;180:3492–501.
Acknowledgments
We wish to thank the laboratory members at Yao Tokushukai General Hospital for their technical assistance.
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The authors have no financial conflicts of interest.
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Ebara, S., Song, SN.J., Mizuta, H. et al. Interleukin-6-producing dermoid cyst associated with multicentric Castleman’s disease. Int J Hematol 95, 198–203 (2012). https://doi.org/10.1007/s12185-011-0978-5
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DOI: https://doi.org/10.1007/s12185-011-0978-5