What preoperative tests are required for a patient with Von Hippel-Lindau (VHL) disease undergoing nephrectomy?

From the Guidelines

Before undergoing a nephrectomy, the patient with von Hippel-Lindau disease should undergo a comprehensive preoperative evaluation, including assessment of serum electrolytes, creatinine, and BUN to evaluate renal function, as recommended by the American Urological Association/Endourological Society guideline 1. The patient's preoperative workup should prioritize tests that assess renal function, given the potential for reduced renal function in patients with VHL disease.

• Evaluation of serum chemistries and renal function tests should be based upon clinical characteristics, including pertinent preoperative medications and therapies, endocrine disorders, and risk of renal dysfunction, as suggested by the American Urological Association/Endourological Society guideline 1.
• Additionally, the patient should undergo abdominal MRI or CT scan with contrast to evaluate both kidneys for additional tumors, as VHL commonly causes multiple renal cell carcinomas.
• Brain and spine MRI should be performed to check for central nervous system hemangioblastomas.
• Ophthalmologic examination is necessary to detect retinal hemangioblastomas.
• The patient also needs adrenal function testing, including plasma metanephrines or 24-hour urinary catecholamines, to rule out pheochromocytomas which occur in about 10-20% of VHL patients and could cause dangerous blood pressure fluctuations during surgery if undetected, as suggested by the ACC/AHA 2007 guidelines on perioperative cardiovascular evaluation and care for noncardiac surgery 1.
• Pancreatic imaging should be included to identify potential neuroendocrine tumors or cysts.
• Standard preoperative tests like complete blood count, comprehensive metabolic panel, coagulation studies, and cardiac evaluation are also required, considering the patient's overall health and potential risks for postoperative complications, as indicated by the ACC/AHA 2007 guidelines 1.

From the Research

Pre-Surgical Tests for Nephrectomy in Von Hippel-Lindau Disease

To determine the necessary tests before surgery for a patient with Von Hippel-Lindau disease who needs a nephrectomy, consider the following:

• The patient's overall health and the presence of any other VHL-related tumors or conditions, such as hemangioblastomas, pheochromocytomas, or pancreatic neuroendocrine tumors 2, 3
• The size and location of the renal tumor, as well as the presence of any other renal cysts or tumors 4, 5
• The patient's renal function and the potential need for renal replacement therapy after surgery 6

Recommended Tests

Some recommended tests before nephrectomy in a patient with Von Hippel-Lindau disease may include:

• Imaging studies, such as CT or MRI scans, to evaluate the size and location of the renal tumor and to assess for any other VHL-related tumors or conditions 4, 2
• Laboratory tests, such as serum creatinine and estimated glomerular filtration rate (eGFR), to assess renal function 5, 6
• Genetic testing to confirm the diagnosis of VHL and to identify any family members who may be at risk 2, 3
• Other tests, such as echocardiogram or pulmonary function tests, to assess the patient's overall health and to identify any potential risks for surgery 6

Considerations for Surgery

When considering surgery for a patient with Von Hippel-Lindau disease, it is essential to take into account the potential risks and benefits, including:

• The risk of tumor recurrence or metastasis 4, 5
• The potential need for renal replacement therapy after surgery 6
• The risk of complications, such as bleeding or infection, during or after surgery 5, 6
• The potential benefits of surgery, including the removal of the tumor and the improvement of symptoms 4, 2