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Ganglioneuromas (GNs) are the rarest and most benign of the neuroblastic tumors. We experienced a case of huge retroperitoneal GN which differentiated into malignant peripheral nerve sheath tumors (MPNST) with hepatic metastasis. The tumor was located in the upper right quarter of the abdomen and pressed the right lobe of the liver, which was initially misdiagnosed as a liver carcinoma. The tumor shared blood supply with the right liver lob and had rich blood supplies from the abdominal aorta, renal artery and hepatic artery. It was also associated with skin pigment and recurrence shortly following resection. Our finding demonstrated that MPNST is a potent invasive malignant tumor and metastasis earlier with very poor prognosis.

A huge malignant peripheral nerve sheath tumor with hepatic metastasis arising from retroperitoneal ganglioneuroma