Von Hippel-Lindau Syndrome

Implications for Nursing Care

Suzanne M. Mahon, RN, DNSc, AOCN®, APNG; Laura Waldman, MS, CGC


Oncol Nurs Forum. 2012;39(6):533-536. 

In This Article

Nursing Considerations

Understanding the variable presentation of VHL is important when extracting family history information. Recognition of VHL in patients presenting with the constellation of benign and malignant tumors associated with VHL is imperative to ensure appropriate monitoring for associated malignancies as well as benign conditions. When VHL is suspected, the family should be referred to a genetics professional for evaluation and testing. By age 25, most affected individuals have a detectable lesion of some type (Coleman, 2008).Nurses should pay attention to any history of particularly unusual tumors and refer for genetic counseling promptly. Both of the families in the case studies will benefit from the identification of other at-risk members as appropriate screening measures can be implemented (see Figure 2). In addition, assessing the family's perception of the diagnosis and providing education to correct any misconceptions is important (see Figure 3). With these steps, the mortality from complications associated with VHL tumors will ultimately be reduced and quality of life for these families will improve.

Figure 2.

Routine Evaluation of Individuals With Confirmed or Clinically Suspected von Hippel-Lindau Syndrome
Note. Based on information from Frantzen et al., 2009; Lindor et al., 2008; Teplick et al., 2011.

Figure 3.

Resources for Families of Patients With von Hippel-Lindau (VHL) Syndrome