A case of posterior mediastinal paraganglioma associated with von Hippel-Lindau disease

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  • ｖｏｎ　Ｈｉｐｐｅｌ‐Ｌｉｎｄａｕ病に合併した後縦隔傍神経節腫の１例

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Abstract

A 20-year-old man was referred to our hospital due to an abnormal shadow noted on a chest radiograph during a medical check-up. It was diagnosed as a posterior mediastinal neurogenic tumor preoperatively. The tumor was resected, and the postoperative pathological diagnosis was paraganglioma. The intraoperative systolic arterial pressure transiently exceeded 200 mmHg. Postoperative 123I-metaiodobenzylguanidine scintigraphy revealed left retroperitoneal paraganglioma and right adrenal pheochromocytoma. These tumors were also resected. He also underwent the excision of cerebellar hemangioblastoma 17 months later. Finally, he was genetically diagnosed with von Hippel-Lindau disease. Mediastinal paraganglioma is a very rare disease. However, it is important to promote the preoperative suspicion of paraganglioma to avoid perioperative complications and achieve complete resection. Paraganglioma sometimes appears as a part of von Hippel-Lindau disease, and so we should search for other tumors and investigate the family history of the patient.

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