Q85.83
BillableVon Hippel-Lindau syndrome
HCC Category Mapping
V28HCC 23 — Melanoma and Other Skin Cancers
0.251V24HCC 12 — Breast, Prostate, and Other Cancers and Tumors
0.150ESRDHCC 12 — Breast, Prostate, and Other Cancers and Tumors
0.000RxHCCHCC 22 — Cancer, Other Specified Sites
0.000What This Code Means
Von Hippel-Lindau syndrome is an inherited genetic disorder that causes tumors and cysts to develop in multiple organs, particularly the brain, spinal cord, kidneys, and pancreas.
Coding Tips
- •This is a specific phakomatosis code; do not use the unspecified code Q85.9 when VHL syndrome is documented
- •Document any associated manifestations (hemangioblastomas, renal cell carcinoma, pheochromocytoma) with additional codes for complete clinical picture
Clinical Significance
Von Hippel-Lindau syndrome is a rare genetic tumor predisposition syndrome with high risk of developing multiple types of tumors and cysts throughout life, including potentially life-threatening complications like renal cell carcinoma and pheochromocytomas. The condition requires intensive lifelong surveillance and often multiple surgical interventions, significantly impacting quality of life and requiring specialized management.
Documentation Requirements
- ✓VHL gene mutation confirmation or clinical criteria
- ✓CNS hemangioblastomas documented
- ✓Retinal angiomas and ophthalmologic surveillance
- ✓Renal manifestations (clear cell carcinoma, cysts)
- ✓Pheochromocytomas or paragangliomas
- ✓Pancreatic involvement (cysts, neuroendocrine tumors)
- ✓Family history and genetic counseling
- ✓Comprehensive surveillance protocol implementation
Code Also
- manifestations
Commonly Confused Codes
Code Hierarchy
└Q85Phakomatoses, not elsewhere classified└Q85.8Other phakomatoses, not elsewhere classified└Q85.83Von Hippel-Lindau syndrome
└Q85.83Von Hippel-Lindau syndrome