Gupta Saksham, Bi Wenya Linda, Annino Donald J, Dunn Ian F
Center for Skull Base and Pituitary Surgery, Department of Neurosurgery, and.
Department of Otolaryngology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts; and.
J Neurosurg Case Lessons. 2022 Jun 13;3(24):CASE21663. doi: 10.3171/CASE21663.
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.
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 and . 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.
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.
嗅神经母细胞瘤是一种罕见的鼻窦肿瘤，起源于嗅上皮。作者报告了一例伴有广泛颅侵犯的嗅神经母细胞瘤病例，该病例对酪氨酸激酶抑制剂索拉非尼表现出显著反应。
一名有前列腺癌和黑色素瘤病史的54岁男性出现左侧眼球突出，经检查发现患有一个6.5厘米的卡迪什D期嗅神经母细胞瘤，伴有颅侵犯，对化疗和依维莫司耐药。然而，它对索拉非尼表现出显著反应，导致广泛的颅底缺损，促使进行手术修复。对肿瘤的基因组分析显示在[具体基因1]和[具体基因2]中存在突变。患者在开始使用索拉非尼35个月后出现肝转移导致疾病进展，促使改用乐伐替尼。在此改变10个月后，他的嗅神经母细胞瘤病情进展，1个月后在临终关怀中去世。
作者回顾了一例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情况。他们强调了在当前标准治疗方案背景下，靶向治疗在难治性嗅神经母细胞瘤治疗中的既往应用。靶向治疗可能在难治性嗅神经母细胞瘤的治疗中发挥重要作用。
