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

机译:腹膜后神经节神经瘤引起的巨大恶性周围神经鞘瘤伴肝转移

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摘要

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.
机译:神经节神经瘤(GNs)是神经母细胞瘤中最罕见和最良性的。我们经历了一例巨大的腹膜后GN,该病例分化为伴有肝转移的恶性周​​围神经鞘瘤(MPNST)。肿瘤位于腹部右上四分之一处,并压迫肝脏的右叶,最初被误诊为肝癌。肿瘤与右肝叶共享血液供应,并具有来自腹主动脉,肾动脉和肝动脉的丰富血液供应。切除后不久,它也与皮肤色素和复发有关。我们的发现表明,MPNST是一种有效的浸润性恶性肿瘤,较早转移,预后很差。

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