Endolymphatic Sac Tumors Associated With von Hippel-Lindau: A Case Report Highlighting Opportunity for Novel Orphan Drug Therapy.
| Autor: | Tan D; Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Texas., Fujiwara RJT; Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Texas., Tan C; University of California Irvine School of Engineering, Irvine, California., Isaacson B; Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, Texas., Hunter JB; Department of Otolaryngology-Head and Neck Surgery, Jefferson Health, Philadelphia, Pennsylvania. |
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| Jazyk: | angličtina |
| Zdroj: | Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology [Otol Neurotol] 2024 Oct 01; Vol. 45 (9), pp. e644-e646. Date of Electronic Publication: 2024 Aug 21. |
| DOI: | 10.1097/MAO.0000000000004307 |
| Abstrakt: | Objective: To discuss the potential benefit of belzutifan therapy in a patient with von Hippel-Lindau (VHL) disease-associated endolymphatic sac tumor (ELST). Patients: Case report. Interventions: Clinical details of a patient with residual ELST after hearing preservation surgery who initiated belzutifan therapy postoperatively for concurrent renal cell carcinoma, as well as literature review of belzutifan and ELST. Main Outcome Measures: The patient remained without radiologic evidence of growth of her residual tumor at 17 months post-initiation of belzutifan. It is unknown whether this represents therapeutic drug effect, nonviability of residual tumor, or slow tumor growth not captured radiographically within the duration of follow-up. Conclusions: Belzutifan could have direct therapeutic benefit in patients with VHL-associated ELST. Competing Interests: The authors otherwise have no relevant disclosures with regard to financial support, acknowledgement, or conflicts of interest. (Copyright © 2024, Otology & Neurotology, Inc.) |
| Databáze: | MEDLINE |
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