Congenital Neuroblastoma; Sympathicoblastoma; Stage IVS Neuroblastoma; Pepper’s syndrome; Schwannian Stroma-Poor Neuroblastoma
Neuroblastoma is a malignant solid tumor that develops from the nerve tissues that form the sympathetic nervous system (the part of the nervous system that controls body functions, such as heart rate, blood pressure, digestion, and levels of certain hormones). The cause of neuroblastoma is unknown. Most neuroblastomas begin in the abdomen near the adrenal glands, or next to the spinal cord, or in the chest. Child Neuroblastoma with distant metastases or recurrent is considered an aggressive tumor because it often quickly spreads to other parts of the body.
The first symptoms are usually fever, a general sick feeling (malaise), and pain. There may also be loss of appetite, weight loss, and diarrhea. More specific signs and symptoms depend on the type of neuroblastoma.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: Diagnostic tests include urine catecholamine levels (vanillylmandellic acid and homovanillic acid levels), imaging tests (X-ray, ultrasound, CT scan, MRI, radionuclide bone scan), tumor biopsy, bone marrow biopsy, and bone marrow aspiration.
Physical findings: Presentation signs vary depending on the site of the tumor, such as abdominal or liver mass, high blood pressure and a fast heart rate (caused by adrenal gland tumors), and swollen lymph nodes.
ICD-9: 194.0, Neuroblastoma, unspecified site
ONSET AND PROGRESSION
Neuroblastoma occurs in infants and young children, and is frequently diagnosed before age 5. It may occur in older children but only rarely.
Stage IV is considered advanced if the tumor has spread to other parts of the body, most commonly the skin, liver or bone marrow. Stage IVS applies only to children younger than 1 year old. However, despite the extent of metastasis, babies with Stage IVS have a good chance of recovery, and sometimes the condition sometimes resolves without any treatment.
Patients older than 18 months usually present with metastatic disease and have poor outcomes despite intensive therapy. Most recurrences occur during the first 2 years following treatment.
Treatment varies depending on location of the tumor, the stage of the cancer, and patient’s age. The types of treatment may include surgery, chemotherapy, and radiation therapy. Stem cell transplantation may be needed for patients with high-risk disease.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for evaluation: The adjudicator needs medical evidence from treating sources and hospitals. It should include clinical history and examination that describe the diagnostic features of the impairment, imaging tests, biopsies, pathology reports, surgical procedures, pertinent treatment records, and up-to-date progress notes.
Suggested Listings for Evaluation:
113.21B or 113.21C
Requires documentation of metastasis or recurrence.
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