Editing Management of Localized and Locally Advanced Disease (section)

=== Patients with VHL or other familial syndromes ===

* '''Appropriate surgical treatment of RCC in VHL requires excision of all solid and cystic renal lesions.'''
* '''Nephron-sparing strategies, including tumor enucleation when feasible, should be pursued whenever possible''', given the multifocal nature of the disease, even for centrally located tumors.
** For PN, an enucleative approach is often preferred rather than wide resection.
* '''The National Cancer Institute have defined a 3-cm threshold for intervention in patients with VHL disease.'''
** '''3-cm threshold also applies to patients with HPRCC and Birt-Hogg-Dubé syndromes. However, HLRCC and SDH-RCC are exceptions in that tumors in these syndromes are typically more aggressive and should be managed accordingly, even when <3 cm'''
* After initial management, patients with VHL disease are at much higher risk for local recurrence than patients with sporadic RCC and must be observed closely.
* If needing another intervention for recurrence, repeat PN can be challenging because of postoperative fibrosis, and TA may be preferred for local control