Q85.03

Billable

Schwannomatosis

HCC Category Mapping

V28HCC 23 — Melanoma and Other Skin Cancers

0.251

V24HCC 12 — Breast, Prostate, and Other Cancers and Tumors

0.150

ESRDHCC 12 — Breast, Prostate, and Other Cancers and Tumors

0.000

What This Code Means

Schwannomatosis is a rare genetic disorder characterized by multiple benign tumors (schwannomas) growing on nerves throughout the body.

Coding Tips

•This is a distinct condition from NF1 and NF2; ensure proper differentiation in documentation
•Document the location and number of schwannomas when clinically relevant

Clinical Significance

Schwannomatosis is the rarest form of neurofibromatosis characterized by multiple schwannomas throughout the nervous system with chronic pain as a predominant feature, requiring specialized pain management and neurological care. Unlike NF1 and NF2, it typically presents later in life and does not involve bilateral acoustic neuromas.

Documentation Requirements

✓Multiple schwannomas confirmed by pathology
✓Absence of bilateral vestibular schwannomas
✓Chronic pain documentation and assessment
✓Genetic testing results (SMARCB1, LZTR1 mutations)
✓MRI documentation of multiple nerve tumors
✓Exclusion of NF1 and NF2 criteria
✓Family history assessment
✓Pain management interventions documented

Commonly Confused Codes

Q85.02 — NF2 (has bilateral acoustic neuromas)Q85.01 — NF1 (different tumor types and manifestations)D36.10 — Individual schwannoma without syndrome G89.29 — Other chronic pain (symptom, not underlying syndrome)

Code Hierarchy

└Q85Phakomatoses, not elsewhere classified └Q85.0Neurofibromatosis (nonmalignant)└Q85.03Schwannomatosis

└Q85.03Schwannomatosis