• Takeshi Namekawa, Takanobu Utsumi, Takashi Imamoto, Koji Kawamura, Takashi Oide, Tomoaki Tanaka, Naoki Nihei, Hiroyoshi Suzuki, Yukio Nakatani, and Tomohiko Ichikawa.
• Department of Urology, Graduate School of Medicine, Chiba University, Chiba, Japan.
• Asian J Surg. 2016 Jul 1; 39 (3): 187-90.

AbstractAdrenal tumors with more than one cellular component are uncommon. Furthermore, an adrenal tumor composed of a pheochromocytoma and a malignant peripheral nerve sheath tumor is extremely rare. A composite pheochromocytoma with malignant peripheral nerve sheath tumor in a 42-year-old man is reported here. After adequate preoperative control, left adrenalectomy was performed simultaneously with resection of the ipsilateral kidney for spontaneous rupture of the left adrenal tumor. Pathological findings demonstrated pheochromocytoma and malignant peripheral nerve sheath tumor in a ruptured adrenal tumor. To date, there have been only four reported cases of composite pheochromocytoma with malignant peripheral nerve sheath tumor, so the present case is only the fifth case in the world. Despite the very poor prognosis of patients with pheochromocytoma and malignant peripheral nerve sheath tumors reported in the literature, the patient remains well without evidence of recurrence or new metastatic lesions at 36 months postoperatively. Copyright © 2012. Published by Elsevier Taiwan.

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