J Rheum Dis.  2015 Apr;22(2):118-122. 10.4078/jrd.2015.22.2.118.

A Case of Thymic Carcinoma with Behcet's Disease Combined with Immunoglobulin A Nephropathy

• Park SH
• Kim JH
• Lee JY
• Shin SK
• Hong YK
• Kie JH
• Kang DY
• Lee CH

• KMID: 2222915
• DOI: http://doi.org/10.4078/jrd.2015.22.2.118

Abstract

Behcet's disease is a systemic inflammatory disorder of unknown etiology, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Renal involvement is rare in patients with Behcet's disease particularly immunoglobulin A (IgA) nephropathy. Other autoimmune diseases have been associated with increased risk of malignancy, but not Behcet's disease. Some cases of Behcet's disease accompanied by bladder cancer, thyroid cancer, stomach cancer, or hematologic malignancies have been reported. However, to the best of our knowledge, co-occurrence of Behcet's diseases with thymic carcinoma has not yet been reported. We experienced a 49-year-old male patient who had been treated for Behcet disease and IgA nephropathy, who presented with a large mediastinal mass on chest x-ray. After thymectomy, he was diagnosed with thymic carcinoma with complete resection.

Keyword

Behcet syndrome; Immunoglobulin A glomerulonephritis; Mediastinal mass; Thymoma

MeSH Terms

Autoimmune Diseases
Behcet Syndrome
Glomerulonephritis, IGA*
Hematologic Neoplasms
Humans
Immunoglobulin A
Male
Middle Aged
Skin
Stomach Neoplasms
Stomatitis, Aphthous
Thorax
Thymectomy
Thymoma*
Thyroid Neoplasms
Ulcer
Urinary Bladder Neoplasms
Uveitis
Immunoglobulin A

Figure

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  Figure 1. (A) On light microscopic finding, mesangial cell proliferaion and mesangial matrix expansion are observed (periodic acid-Schiff stain, ×400). (B) On immunofluorescent study, immnofluorescent activity for immunoglobulin A is obsereved on the mesangium (×400). (C) Electron microscopic examination reveals electron dense deposits on the mesangium and paramesangium (×4,500).

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  Figure 2. (A) A chest posterial-anterial (PA) shows a large right anterior mediastinal mass (arrows) which does not shown on 18 months ago chest PA (B). (C) A chest computed tomography demonstrates a large right anterior mediastinal mass (arrows) which shows mixed solid and cystic components.

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  Figure 3. (A) On gross examination, the mass is relatively well demarcated and on cut section, it shows multilocular cysts containing clear and mucoid fluid and partly whitish solid areas with necrosis. (B) On lower power microscopic examination, the cystic area corresponds to the thymoma and the solid area corresponds to the thymic carcinoma showing infiltrative pattern (H&E, ×40).(C) On high power field, the thymic carcinoma area shows histology of squamous cell carcinoma (H&E, ×200). (D) On immunohistochemical stain, these tumor cells are positive for p53 (×200).

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