PERIPHERAL NERVE CANCER – METASTATIC OR RECURRENT

ALTERNATE NAMES

Malignant Neoplasm of the PNS; Malignant Neurilemmoma; Malignant Neuroma; Malignant Peripheral Nerve Sheath Tumor; Malignant PNS Neoplasm; Malignant PNS Tumor; Malignant Schwannoma; Malignant Tumor of the PNS; MPNST; Neurogenic Sarcoma; Neurosarcoma

DESCRIPTION

Peripheral nerve cancer is a rare malignant tumor that develops in the tissue (sheath) covering the peripheral nerves. The peripheral nervous system includes the nerves that travel from the brain and spinal cord (central nervous system) to other parts of the body. The nerve sheath is the soft tissue that covers the nerve. This type of cancer occurs most commonly along the nerves that run from the buttocks to the legs (sciatic nerves), neck to the arms or within the pelvis. Peripheral nerve cancer that is metastatic or recurrent indicates that the malignant tumor has spread to other parts of the body, and has come back after treatment. This type of cancer generally occurs in adulthood between the ages of 20 and 50 years of age and may occur in childhood. Survival rates for metastatic and recurrent disease are poor, regardless of patient age.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: Diagnostic testing for peripheral nerve cancer include:

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  Magnetic resonance imaging (MRI);

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  X-rays;

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  Computed tomography (CT) scans;

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  Positron emission tomography (PET) scans; and

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  Bone scan to determine the location, size, and shape of the tumor and metastasis.

The only definitive diagnosis of peripheral nerve cancer is a biopsy of the tumor.

Physical findings: Most peripheral nerve cancers do not present with neurological symptoms.

ICD-9: 237.7; 237.9

ICD-10: C47

PROGRESSION

Peripheral nerve cancers are highly aggressive tumors. The prognosis for people with unresectable tumors (tumors that cannot be surgically removed) is poor. Survival rates vary depending on the location and extent of the tumor, including any metastasis.

TREATMENT

Malignant peripheral nerve cancers are aggressive tumors that require a combination of surgery, chemotherapy or radiation. Complete resection of the tumor carries the longest survival rate.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  A pathology report of the tumor biopsy and of any metastasis; and

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  If a pathology report is unavailable, a surgical report or radiological studies especially X-rays, MRI scans, CT scans, or PET scans may be substituted.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.13 B

Primary adult peripheral nerve cancer meets the criteria in listing 13.13 B and requires documented metastases or recurrence.

Equals

113.13 C

Primary pediatric peripheral nerve cancer medically equals the criteria in listing 113.13 C and requires documented metastases or recurrence.