A Case of Retroperitoneal Malignant Schwannoma Resected with Right Lobe of Liver and Inferior Vena Cava in a Patient with von Recklinghausen's Disease

Abstract

We report a retroperitoneal malignant schwannoma resected with the right liver lobe and inferior vena cava in a patient with von Recklinghausen's disease. A 29-year-old woman was admitted with right abdominal tumor and pain. Abdominal computed tomography (CT) showed a mass about 25 cm in diameter in the retroperitoneal space that ventrally depressed the inferior vena cava. Venography showed a defect in the inferior vena cava from the third vertebra and presence of collateral circulation. Under a diagnosis of retroperitoneal malignant schwannoma, the tumor was extirpated. Specifically, the liver was split anteriorly and the tumor resected together with the right liver lobe and inferior vena cava. The inferior vena cava was not reconstructed due to stable blood circulation following resection. The woman was discharged on postoperative day (POD) 24 without complication. Retroperitoneal malignant schwannoma is rare without subjective symptoms and has a poor prognosis, partcularly in patients with von Recklinghausen's disease. Treatment requires complete surgical extirpation.