| 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:
                   
                     
                        
                           • 
                              Magnetic resonance imaging (MRI);
                        
                     
                        
                           • 
                              Computed tomography (CT) scans;
                        
                           • 
                              Positron emission tomography (PET) scans; and
                        
                           • 
                              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: 
                     
                        
                           • 
                              A pathology report of the tumor biopsy and of any metastasis; and
                        
                           • 
                              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.
                   | 
            
               
               | * 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.
                   |