J Korean Ophthalmol Soc.  2009 May;50(5):769-773. 10.3341/jkos.2009.50.5.769.

Two Cases of Orbital Castleman's Disease

• Cha DM
• Kim NJ

• KMID: 2212322
• DOI: http://doi.org/10.3341/jkos.2009.50.5.769

Abstract

PURPOSE:To report two cases of multicentric Castleman's disease involving the orbital area.
CASE SUMMARY
A 52-year-old man presented with discomfort of the right eye on eyeball movement that began 4~5 months ago. He had been diagnosed as Castleman's disease upon the biopsy of his neck lymph nodes 2 years ago. Proptosis was found in his right eye, and orbital computed tomography revealed an enlarged mass in the inferior orbit compared to the former image. An incisional biopsy confirmed Castleman's disease. A 57-year-old man presented with left eyelid swelling that began 3 months ago. Orbital and chest computed tomography revealed a mass in the left superotemporal orbit and the enlarged lymph nodes of the mediastinal area. Castleman's disease was confirmed by biopsy of the lymph nodes in the mediastinal area.
CONCLUSIONS
Steroid therapy was prescribed for the 52-year-old man. The 57-year-old man was placed under close observation.

Keyword

Castleman's disease; Hyaline-vascular type; Orbit

MeSH Terms

Biopsy
Exophthalmos
Eye
Eyelids
Giant Lymph Node Hyperplasia
Humans
Lymph Nodes
Middle Aged
Neck
Orbit
Thorax
Giant Lymph Node Hyperplasia

Figure

• 

  Figure 1. (A, B): Photographs of the patient 1. Note the proptosis in the right eye. (C, D): Orbital MRI of the patient 1. On axial view of T2-weighed orbit MRI, 2.2×2.5×1.7 cm sized mass (white arrow) was found at the right inferior orbital cavity (C). On coronal view of T2-weighed orbit MRI, the mass (white arrow) looked like an inferior rectus muscle per se, not separable from the inferior rectus muscle (D). (E, F): Microscopic photographs of the specimens (E: neck lymph node, F: orbit) obtained from patient 1. Hyalinized germinal center was surrounded by lymphocytes, producing the “onion-skin appearance”(E, H&E, ×200). Although not as typical as the photograph E, regressed germinal center and broad mantle zones of small lymphocytes were found in the follicle (F, H&E, ×200).

• 

  Figure 2. (A) Photograph of patient 2. Note the proptosis of the left eye. (B, C) Orbital MRI of patient 2. 2.0×1.0×1.0 cm sized mass with high signal intensity was found in the left superotemporal orbital cavity (white arrows). (D) On chest computed tomography, contrastenhanced soft tissue mass was found at the left hilar area (black arrow). (E) Microscopic photograph of the specimen obtained from patient 2. Hyalinized germinal center surrounded by lymphocytes was found in the follicle (H&E, ×200).

Reference

References

1. Gittinger JW. Ocular involvement in Castleman's disease. Ophthalmology. 1989; 96:1646–9.

2. Snead MP, James JN, Snead DR, et al. Orbital lymphomas and Castleman's disease. Eye. 1993; 7:84–8.

3. Kurokawa T, Suzuki S, Kawaguchi K, et al. Castleman's disease presenting with ophthalmic signs and symptoms. Am J Ophthalmol. 1999; 128:114–6.

4. Alyahya GA, Prause JU, Heegaard S. Castleman's disease in the orbit. A 20-year follow-up. Acta Ophthalmol Scand. 2002; 80:540–2.

5. Koppens JM, Pon JA, Allen J, Sloan BH. Castleman's disease of the lacrimal gland. Clin Experiment Ophthalmol. 2004; 32:108–10.

6. Castleman B, Iverson L, Menendez V. Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer. 1956; 9:822–30.

7. Gaba AR, Stein RS, Sweet DL, Variakojis D. Multicentric giant lymph node hyperplasia. Am J Clin Pathol. 1978; 69:86–90.

8. Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the media-stinum and other locations. Cancer. 1972; 29:670–83.

9. Frizzera G. Castleman's disease and related disorders. Semin Diagn Pathol. 1988; 5:346–64.

10. 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.

11. Bowne WB, Lewis JJ, Filippa DA, et al. The management of unicentric and multicentric CD: A report of 16 cases and a review of the literature. Cancer. 1999; 85:706–17.

12. Chronowski GM, Ha CS, Wilder RB, et al. Treatment of unicentric and multicentric Castleman disease and the role of radiotherapy. Cancer. 2001; 92:670–6.

13. Herrada J, Cabanillas F, Rice L, et al. The clinical behavior of localized and multicentric Castleman disease. Ann Intern Med. 1998; 128:657–62.

14. Bower M, Powles T, Wiliams S, et al. Brief communication: rituximab in HIV-associated multicentric Castleman disease. Ann Intern Med. 2007; 147:836–9.