PERIPHERAL NERVE
CANCER - METASTATIC OR
RECURRENT
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ALTERNATE NAMES
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Malignant Peripheral Nerve Sheath Tumor; Malignant Neurilemmoma; MPNST; Malignant
Schwannoma; Malignant Tumor of the PNS; Malignant Neoplasm of the PNS; Malignant PNS
Tumor; Malignant PNS Neoplasm; Neurosarcoma; Neurogenic Sarcoma; Malignant Neuroma
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: Diagnostic testing for peripheral nerve cancer include:
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-
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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
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PROGRESSION
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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.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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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.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.13 B
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Primary adult peripheral nerve cancer meets the criteria in listing 13.13 B and requires
documented metastases or recurrence.
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Equals
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113.13 C
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Primary pediatric peripheral nerve cancer medically equals the criteria in 113.13
C and requires documented metastases or recurrence.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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