C47.0

Billable

Malignant neoplasm of peripheral nerves of head, face and neck

HCC Category Mapping

V28HCC 21 — Breast, Prostate, Colorectal and Other Cancers and Tumors

0.545

V24HCC 10 — Lymphoma and Other Cancers

0.675

ESRDHCC 10 — Lymphoma and Other Cancers

0.000

RxHCCHCC 22 — Cancer, Other Specified Sites

0.000

What This Code Means

A malignant tumor arising from the peripheral nerves in the head, face, and neck region.

Coding Tips

•This code applies to nerve sheath tumors (such as malignant peripheral nerve sheath tumors) in the head and neck area.
•Ensure the neoplasm originates from peripheral nerve tissue, not from surrounding structures.

Clinical Significance

Malignant peripheral nerve sheath tumors of the head, face, and neck are rare but aggressive sarcomas that arise from Schwann cells or other nerve sheath components. These tumors are strongly associated with Neurofibromatosis Type 1 and carry a high risk of local recurrence and distant metastasis, requiring complex multidisciplinary management.

Documentation Requirements

✓Pathology confirmation of malignant peripheral nerve sheath tumor (MPNST) or other peripheral nerve malignancy
✓Specific nerve or anatomical location within head/face/neck region
✓Tumor size, grade, and surgical margins if resected
✓Neurofibromatosis Type 1 status (present in 50% of MPNST cases)
✓Imaging (MRI preferred) showing tumor extent and relationship to adjacent structures

Excludes 1 — Do NOT code together

malignant neoplasm of peripheral nerves of orbit (C69.6-)

Commonly Confused Codes

D36.11 (Benign neoplasm of peripheral nerves of face) — benign nerve tumors such as schwannomas or neurofibromas C47.9 (Malignant neoplasm of peripheral nerves, unspecified) — use C47.0 when head/face/neck is documented C72._ (Malignant neoplasm of spinal cord, cranial nerves) — cranial nerve tumors use different codes C49.0 (Malignant neoplasm of connective and soft tissue of head/face/neck) — soft tissue sarcoma, not nerve origin

Code Hierarchy

└C47Malignant neoplasm of peripheral nerves and autonomic nervous system └C47.0Malignant neoplasm of peripheral nerves of head, face and neck

└C47.0Malignant neoplasm of peripheral nerves of head, face and neck