Dr. Choyke described the results of years of research at the National Institutes of Health, leading to strategies for keeping normal function of kidneys, adrenals, and pancreas and thereby improving quality of life for people with VHL.
Organ Sparing in VHL
A life long perspective:
Maintain normal function
Quality of life, Improve outcome
Reduce risk of cancer by monitoring with imaging and operating early
Reduce the total number of operations by safely prolonging interval between surgery
Cysts can grow and interfere with pancreatic function but otherwise are benign
Cystadenomas behave like cysts
Solid tumors usually grow slowly but can metastasize to liver (3-8 cm)
Solid tumors (Islet cell tumors) are rarely functional
Location
Strategy
No role for surgery with cysts
For Islet Cell tumors in the Tail of the pancreas <3 cm, cut off
For Islet Cell tumors in the Head of the pancreas 1.5-2 cm, cut off
Intraoperative ultrasound
Identify other lesions
Identify the relationship of duct to pancreas
Results
18 patients with Islet cell tumors
6 had enucleations with preserved pancreatic function
7 had distal pancreatectomy with preserved pancreatic function
5 had metastatic disease at diagnosis
Conclusions
An Organ Sparing strategy is possible for kidney, adrenal, and pancreatic manifestations of VHL
Imaging is used to monitor and localize tumors before and during surgery
Non-invasive methods are being used to take this strategy the next step
The History of treatment of these organs has gone from
Total removal of organ
to Partial removal of organ
to Enucleation
to Laparoscopic enucleation
to Percutaneous ablation
The role of imaging has increased throughout this progression, leading to better quality of life for patients.
Talk presented at the 4th International Patient/Provider Conference on Von Hippel-Lindau, Seattle, Washington, June 1998. Go to the agenda for the meeting.
