冯-希佩尔-林道病的综合治疗:病例报告

Cancer Innovation Pub Date : 2023-09-20 DOI:10.1002/cai2.94
Xuesong Li, Zheng Mo, Zhuo Yu
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引用次数: 0

摘要

von Hippel-Lindau(VHL)病是一种罕见的常染色体显性多器官疾病,以富含血管的多种良性和恶性肿瘤以及其他器官的囊肿为特征。为使VHL病患者获得良好的预后,临床治疗策略显得尤为重要。在此,我们报告了一例确诊为 VHL 病的 45 岁女性患者,她患有脊柱血管母细胞瘤(HB)和透明细胞肾细胞癌(ccRCC)。右肾切除术四年后,发现右肾复发 RCC,左肾也出现恶性病变。患者开始接受索拉非尼(800 毫克,每天一次)和替莱珠单抗(200 毫克,每 3 周一次)治疗。治疗 6 个月后,肾细胞癌的体积显著缩小,肾功能得到改善。更重要的是,她在整个治疗过程中获得了部分应答。显微镜下,她接受了髓内肿块切除术,并切除了胸椎T4-5节的HB。麻木和疼痛等神经症状明显缓解。此外,保肝药和左旋甲状腺素分别缓解了替舒利珠单抗引起的肝脏转氨酶水平升高和甲状腺功能减退。总之,综合治疗策略可使VHL患者受益,尤其是HB和ccRCC患者。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Comprehensive treatment of von Hippel-Lindau disease: A case report

Comprehensive treatment of von Hippel-Lindau disease: A case report

von Hippel-Lindau (VHL) disease is a rare autosomal dominant multiorgan disease characterized by several benign and malignant tumors rich in vascular, as well as cysts in other organs. A great clinical treatment strategy is significantly warranted for good prognosis of patients with VHL disease. Herein, we reported a case of a 45-year-old woman diagnosed with VHL disease with spinal hemangioblastoma (HB) and clear cell renal cell carcinoma (ccRCC). Four years after the resection of the right kidney, a recurrent RCC in the right kidney and a malignant lesion in the left kidney were observed. This patient was started on sorafenib (800 mg, daily) and tislelizumab (200 mg per 3 weeks). After 6 months of treatment, the size of renal cell carcinoma was dramatically reduced and renal function improved. More importantly, she achieved partial response during the whole treatment. Microscopically, intramedullary masses resection was done and the HB in T4-5 thoracic spinal was removed. Neurologic symptoms such as numbness and pain were remarkably alleviated. Additionally, tislelizumab-induced elevation in liver transaminase levels and hypothyroidism were revered by hepatoprotector and levothyroxine, respectively. In short, comprehensive treatment strategies may benefit patients with VHL disease, especially with HB and ccRCC.

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