vhl alliance

Suggested Referral Criteria for VHL Clinical Care Centers

These are criteria used to REFER patients. These are NOT criteria for clinical diagnosis of VHL.

Developed and Used by Othon Iliopoulos, MD, Massachusetts General Hospital, Boston MA


1.  Any blood relative of an individual diagnosed with VHL disease

2.  Any individual with:
–  Hemangioblastoma (HB)
–  Clear cell renal carcinoma (RCC)
–  Pheochromocytoma (PHE)
–  Endolymphatic sac tumor (ELST)
–  Epididymal or adnexal papillary cystadenoma
–  Pancreatic serous cystadenomas or pancreatic neuroendocrine tumors.

3.  Any individual with ONE or more of the following:
–  CNS hemangioblastoma
–  Pheochromocytoma or paraganglioma
–  Endolymphatic sac tumor (ELST)
–  Epidiydmal papillary cystadenoma

4.  Any individual with
Clear cell renal carcinoma (RCC) diagnosed at a < 40 year old patient Bilateral and/or
multiple clear cell RCC
–  >1 pancreatic serous cystadenoma
–  >1 pancreatic neuroendocrine tumor
–  Multiple pancreatic cysts + any VHL-associated lesion