PDF(Pubmed)
Abstract：
BACKGROUND: Olfactory neuroblastomas are rare sinonasal tumors that arise from the olfactory epithelium. The authors presented a case of an olfactory neuroblastoma with extensive cranial invasion that demonstrated dramatic response to sorafenib, a tyrosine kinase inhibitor.
METHODS: A 54-year-old man with history of prostate cancer and melanoma presented with left-sided proptosis and was found to have a 6.5-cm Kadish stage D olfactory neuroblastoma with cranial invasion that was refractory to chemotherapy and everolimus. However, it demonstrated dramatic response to sorafenib, causing extensive skull base defects that prompted operative repair. Genomic analysis of the tumor revealed mutations in TSC1 and SUFU. The patient developed disease progression with liver metastases 35 months after starting sorafenib, prompting a change to lenvatinib. He experienced progression of his olfactory neuroblastoma 10 months following this change and died in hospice 1 month later.
CONCLUSIONS: The authors reviewed the clinical presentation and management of a large olfactory neuroblastoma with dramatic response to sorafenib. They highlighted prior uses of targeted therapy in the management of refractory olfactory neuroblastoma within the context of current standard treatment regimens. Targeted therapies may play a vital role in the management of refractory olfactory neuroblastoma.
METHODS: A 54-year-old man with history of prostate cancer and melanoma presented with left-sided proptosis and was found to have a 6.5-cm Kadish stage D olfactory neuroblastoma with cranial invasion that was refractory to chemotherapy and everolimus. However, it demonstrated dramatic response to sorafenib, causing extensive skull base defects that prompted operative repair. Genomic analysis of the tumor revealed mutations in TSC1 and SUFU. The patient developed disease progression with liver metastases 35 months after starting sorafenib, prompting a change to lenvatinib. He experienced progression of his olfactory neuroblastoma 10 months following this change and died in hospice 1 month later.
CONCLUSIONS: The authors reviewed the clinical presentation and management of a large olfactory neuroblastoma with dramatic response to sorafenib. They highlighted prior uses of targeted therapy in the management of refractory olfactory neuroblastoma within the context of current standard treatment regimens. Targeted therapies may play a vital role in the management of refractory olfactory neuroblastoma.
摘要：
背景：嗅觉神经母细胞瘤是起源于嗅觉上皮的罕见鼻窦肿瘤。作者介绍了一例嗅觉神经母细胞瘤，伴有广泛的颅骨侵袭，表现出对索拉非尼的戏剧性反应。酪氨酸激酶抑制剂.
方法：一名54岁的男性，有前列腺癌和黑色素瘤病史，表现为左侧突出，被发现患有6.5cm的KadishD期嗅神经母细胞瘤，并伴有颅骨浸润，对化疗和依维莫司均难以治疗。然而,它对索拉非尼表现出戏剧性的反应，导致广泛的颅底缺损，促使手术修复。肿瘤的基因组分析显示TSC1和SUFU突变。患者在开始索拉非尼治疗35个月后出现疾病进展并伴有肝转移，促使更改为lenvatinib。在这种变化后10个月，他经历了嗅觉神经母细胞瘤的进展，并在1个月后死于临终关怀医院。
结论：作者回顾了对索拉非尼有显著反应的大型嗅神经母细胞瘤的临床表现和治疗。他们强调了在当前标准治疗方案的背景下，靶向治疗在难治性嗅觉神经母细胞瘤的治疗中的先前用途。靶向治疗可能在难治性嗅觉神经母细胞瘤的治疗中起着至关重要的作用。
方法：一名54岁的男性，有前列腺癌和黑色素瘤病史，表现为左侧突出，被发现患有6.5cm的KadishD期嗅神经母细胞瘤，并伴有颅骨浸润，对化疗和依维莫司均难以治疗。然而,它对索拉非尼表现出戏剧性的反应，导致广泛的颅底缺损，促使手术修复。肿瘤的基因组分析显示TSC1和SUFU突变。患者在开始索拉非尼治疗35个月后出现疾病进展并伴有肝转移，促使更改为lenvatinib。在这种变化后10个月，他经历了嗅觉神经母细胞瘤的进展，并在1个月后死于临终关怀医院。
结论：作者回顾了对索拉非尼有显著反应的大型嗅神经母细胞瘤的临床表现和治疗。他们强调了在当前标准治疗方案的背景下，靶向治疗在难治性嗅觉神经母细胞瘤的治疗中的先前用途。靶向治疗可能在难治性嗅觉神经母细胞瘤的治疗中起着至关重要的作用。
