CASE REPORT article
Front. Oncol.
Sec. Surgical Oncology
Volume 15 - 2025 | doi: 10.3389/fonc.2025.1633911
This article is part of the Research TopicRare Diseases and Aging: A New Frontier in HealthcareView all articles
Von Hippel-Lindau Syndrome with Multisystem Involvement: A Therapeutic Dilemma
Provisionally accepted
- 1Southwest Hospital, Army Medical University, Chongqing, China
- 2Hainan Medical University, Haikou, China
- 3Chengdu Fifth People's Hospital, Chengdu, China
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We present a rare case of von Hippel-Lindau (VHL) syndrome type 2B, characterized by multisystem involvement including Stanford type B aortic dissection, pheochromocytoma, renal cell carcinoma (RCC), cerebellar hemangioblastoma with obstructive hydrocephalus, and extensive visceral cysts. This case highlights critical therapeutic dilemmas: urgent aortic repair versus risks of catecholamine surge from pheochromocytoma resection, and neurosurgical hazards of cerebellar lesions. We describe the diagnostic and therapeutic challenges associated with VHL syndrome, culminating in the development of a preliminary treatment strategy following multidisciplinary team (MDT) discussions. This study underscores the critical importance of early recognition and systematic MDT management to optimize clinical outcomes in VHL syndrome.
Keywords: von Hippel-Lindau syndrome, case report, aortic dissection, multidisciplinary management, early recognition
Received: 23 May 2025; Accepted: 30 Sep 2025.
Copyright: © 2025 He, Wang, Yang, Shen, Zeng, Zheng and Wang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Xiaozhu Zeng, zengxiaozhu916@163.com
Jun Zheng, zhengjun10000@126.com
Yongquan Wang, wangyongquan@aliyun.com
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