A case of low-risk neuroblastoma refractory to chemotherapy with an image-defined risk factor

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  • IDRF陽性,化学療法不応性の低リスク神経芽腫の一例

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<p>Although gross total resection of low-risk neuroblastoma remains the current treatment recommendation, neoadjuvant chemotherapy would be administered when image-defined risk factors (IDRFs) were positive. However, in the case of low-risk tumor with IDRFs and resistant to chemotherapy, the treatment of choice is not clearly defined. We describe the case of a 5-year-old girl who presented with a localized 78×83 mm2 mass located in the left suprarenal fossa. The tumor completely involved the left renal artery, which on biopsy proved to be intermixed ganglioneuroblastoma, with favorable histology without MYCN amplification. The patient was assigned to the category of low-risk neuroblastoma with IDRFs. Despite chemotherapy for 6 weeks, the tumor showed no response. Thus, we tried debulking surgery to preserve the left kidney. However, since the bleeding was difficult to control, we performed complete resection with left nephrectomy. Histopathological findings showed ganglioneuroblastoma nodules with unfavorable histology without MYCN amplification. No adjuvant therapy was administered.</p>

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