Belzutifan in VHL Syndrome
A 45-year-old woman presented with lower back pain for 3 months. MRI lumbar spine showed the enhancing lesion opposite to L1-L2, which was suggestive of hemangioblastoma. The tumor was surgically removed, however, she again presented to the emergency department for acute abdomen. Her blood pressure was 180/110 mmHg. Contrast-enhanced CT (CECT) abdomen was showed unilateral heterogeneously enhancing suprarenal mass on the right side. The metaiodobenzylguanidine (MIBG) scan confirmed pheochromocytoma which was surgically removed. Sequencing of the VHL gene revealed a heterozygous VHL gene. Today she comes to you for discussing treatment for her recently diagnosed VHL syndrome. She heard about a new drug recently approved for this disease and was interested to know more about that. Which of the following is true for belzutifan?
- Mechanism of action is the same as everolimus that is mTOR inhibition.
- Belzutifan acts directly on the VHL gene and promotes its tumor suppression activity.
- The most common side effect is cardiovascular adverse events such as hypertension.
- The most common grade 3 adverse events in patients receiving belzutifan were hypoxia in the landmark trial
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V S Cheran
Mar 27, 2022
May 26, 2024
Pending Moderator approval.