Tohoku J. Exp. Med., 2022 June, 257(2)

Severe Inflammatory Idiopathic Multicentric Castleman’s Disease Coexisting with Advanced Renal Cancer: A Case Report

Daigo Chiba,1 Yoshihide Kawasaki,1 Atsushi Miyagi,1 Yuki Katsumata,1 Yohei Satake,1 Shuichi Shimada,1 Hiromichi Katayama,1 Naoki Kawamorita,1 Shinichi Yamashita,1 Koji Mitsuzuka,1 Kanae Akita,2 Mika Watanabe3 and Akihiro Ito1

1Division of Preventive Medicine, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Niigata, Japan
2Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
3Department of Pathology, Tohoku University Hospital, Sendai, Miyagi, Japan

The present case study was conducted on a 74-year-old man who visited our department due to a left renal and retroperitoneal tumor on computed tomography (CT). The patient was diagnosed with left renal cancer lymph node metastasis and was hospitalized a few weeks prior to surgery due to fever, malaise, and severe appetite loss. Biochemical laboratory findings at admission showed markedly high levels of inflammation. The cause of high inflammatory response was paraneoplastic syndrome. Tumor resection was considered necessary, and left nephrectomy and lymphadenectomy were performed; however, it did not improve the inflammatory response. After operation, positron emission tomography-CT revealed hyperaccumulation of 18F-fluorodeoxyglucose in the bone marrow throughout the body. Pathological examination of the resected specimen and bone marrow aspiration revealed the coexistence of idiopathic multicentric Castleman disease (CD) and renal cancer. Prednisolone and tocilizumab were administered for idiopathic multicentric CD and a tyrosine kinase inhibitor for renal cancer; however, they had poor therapeutic effect, and the patient died. CD is characterized by systemic symptoms due to the overproduction of interleukin-6. Treatment for idiopathic multicentric CD involves steroid and anti-interleukin-6 therapy. The diagnostic criteria for CD require the exclusion of malignant tumors although there are some cases in which CD and malignant tumors coexist. The prognosis for CD is relatively good; however, as in this case, the prognosis of CD coexisting with uncontrollable renal cancer is insufficient due to poor improvement in the inflammatory response.

Keywords —— Castleman disease; inflammation; paraneoplastic syndrome; renal cancer; tocilizumab

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Tohoku J. Exp. Med 2022, 257, 127-133.

Correspondence: Yoshihide Kawasaki, Department of Urology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi 980-8574, Japan.

e-mail: kawasaki@uro.med.tohoku.ac.jp