Preserving Normal Function:
The Role of Imaging

National Cancer Institute, Bethesda, MD

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

But . . .

• More complicated procedures
• Less familiarity in medical profession
• Risk of recurrent disease
• Monitoring

Kidney - Adrenal - Pancreas

Kidney Imaging

VHL Kidney Lesions

• Cysts
• Cystic tumors
• Solid tumors

Some things we do know

• Cysts do not cause problems*
  • There is a solid component inside a cyst.
• Solid masses do not cause problems until they grow (become well differentiated)
• Normal appearing tissue contains tumors but...
• Most of them grow slowly

A strategy

• Solid Tumors
  • 0-3 cm Low risk of metastases
  • >3.5 cm Risk increases with size
• Cystic tumors
  • Solid component is the key
• Remove all solid tumors when they reach 3 cm.**

Nephron Sparing Approach

• Lesions are enucleated
• As many lesions are removed as possible
• Ultrasound guidance for deep lesions during the operation

Results

• No patients with tumors <3 cm. have developed metastatic disease
• To date no patients have had to go on dialysis

What is the best imaging test?

• Computed Tomography (CT)
  • Accurate, reproducible, available
  • Needs iodinated contrast media, $$
• Ultrasound
  • No radiation, available, less $
  • Operator dependent, insensitive for small lesions
• Magnetic resonance imaging
  • No radiation, safe contrast agent, flexible
  • Availability, expertise, $$$
  • Increasing use, especially in
    • Decreased renal function
    • Allergies to iodinated contrast

The Adrenal

Consists of

• Medulla, which produces catecholamines
• Cortex, which produces Cortisol, Aldosterone, and Androgens

Adrenal Imaging

• Enhancing Masses
  • Pheochromocytomas
• Other lesions (not normally in VHL)
  • Adenomas, metastatic lesions
• Verify with
  • MRI
  • MIBG studies

Some things we know . . .

• Small tumors are silent
• Increased risk of function with size
  • Arrhythmias
  • Hypertension
  • Personality changes
• Cortex is normal

Adrenal Sparing Surgery

• Remove tumor
• Leave residual adrenal
• Monitor for recurrence

Adrenal Imaging

• Identify adrenal lesion
  • Enhances on CT
  • Hot on MIBG
  • Bright on MRI
• Identify normal adrenal tissue

Results

• 9 out of 13 patients with adrenal sparing surgery have recovered adrenal function
• 5 procedures done laparoscopically

The Pancreas

Imaging of the Pancreas

• Cysts
• Cystadenomas
• Solid (Islet Cell Tumors)

Some Things We Know . . .

• 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.