Treatment

• affected individuals are at risk of developing various benign and malignant tumours of the central nervous system, kidneys, adrenal glands, pancreas, and reproductive adnexal organs
• a multidisciplinary approach to treatment is required because of the complexities associated with management of the various types of tumours in this disease

Treatment depends upon the organ affected and symptomatology, for example:

• cryotherapy or photocoagulation for retinal lesions
• surgery for phaeochromocytoma or renal adenocarcinoma
• surgery for spinal haemangioblastomas 
  • there is evidence that spinal cord hemangioblastomas can be safely removed in the majority of patients with VHL disease. Generally in these patients, hemangioblastomas of the spinal cord should be removed when they produce symptoms or signs (1)

Reference:

1. Lonser RR et al (2003). Surgical management of spinal cord hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 98(1):106-16.