MALIGNANT BRAINSTEM GLIOMAS -- CHILDHOOD

Childhood Malignant Brainstem Glioma; Malignant Brainstem Glioma- Childhood Diffuse Intrinsic; Malignant Brainstem Glioma; Diffuse Intrinsic Pontine Gliomas; DIPG; Malignant Brain Tumor; Pediatric Malignant Brain Tumor; Malignant Brain Tumor – Children

Malignant Brainstem Gliomas are a common type of brain tumor that occurs in the region of the brain referred to as the brainstem. Approximately 80% of malignant pediatric brainstem gliomas arise within the pons. The majority of pontine tumors, diffuse intrinsic pontine gliomas (DIPG), are usually high-grade, aggressive, locally infiltrative, and have a uniformly poor prognosis. Diffuse intrinsic pontine gliomas meet the criteria in the listings upon confirmed diagnosis alone.

Brainstem gliomas are classified into four grades. Grades I and II are considered low grade; grades III and IV are considered high grade. Grades I and II are the slowest growing and least aggressive. In children, Grade II brain stem tumors meet the criteria in listing 113.13 C 2 if they are progressive or recurrent following initial anticancer therapy. Grade I tumors are generally considered benign, and we evaluate them under the neurological listing 111.05. Grade III and Grade IV childhood brain stem tumors are the fastest growing and most aggressive and meet the criteria in listings 113.13 A and B.

Diagnostic testing: MRI scans are the preferred tool to evaluate a brainstem tumor, although a CT scan may be performed in the rare circumstances where MRI is unavailable. A biopsy is seldom performed outside specialized biomedical research protocols for DIPG, unless the diagnosis of this tumor is in doubt.

Tumors also are characterized on the basis of:

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  Site of origin;

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  Direction and extent of tumor growth;

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  Degree of brainstem enlargement;

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  Presence or absence of cysts;

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  Necrosis;

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  Hemorrhage; and

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  Hydrocephalus.

Physical findings: Although not pathognomonic of DIPG, signs and symptoms are related to location of the tumor and may include:

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  Cranial nerve deficits;

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  Loss of balance, difficulties with walking, worsening handwriting, or abnormal speech;

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  Lack of coordination;

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  General weakness or weakness on one side of the face;

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  Unusual sleepiness or changes in energy level;

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  Double vision;

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  Increased intracranial pressure; and

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  Headaches.

ICD-9: 191.7

Standard anticancer therapy for brainstem glioma has not been established. Surgery is not usually performed because of the tumor’s infiltrating location in the brainstem; however, surgical procedure to reduce pressure inside the skull caused by hydrocephalus is common. Surgery may be performed if the tumor extends into the fourth ventricle.

Radiation is used to shrink the tumor, improve, stabilize or prolong life. New therapies have yielded little benefit over conventional treatment with radiotherapy alone. Unfortunately, recurrence usually occurs after 6 to 9 months of treatment.

Adjuvant chemotherapy is generally not used in children because efficacy has not been proven. Data suggest that pre-radiation chemotherapy may improve survival in diffuse intrinsic pontine gliomas. The effectiveness of chemotherapy at relapse is uncertain, but it may benefit some patients.

Individuals who have difficulty swallowing or diminished gag reflex may require gastrostomy tube placement. Individuals with frequent upper respiratory infections, pneumonia, or altered voice may require post-op ventilator assistance.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and examination that describe the diagnostic features of the impairment;

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  Imaging tests;

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  Pathology reports;

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  Surgical reports;

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  Pertinent treatment records; and

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  Up-to-date progress notes.

Suggested Listings for Evaluation:

REMARKS

Malignant brainstem gliomas occur more frequently in children. Grade III and Grade IV brainstem cancers in children, such as diffuse intrinsic pontine gliomas,; meet the criteria in listing 113.13 upon confirmed diagnosis alone.