MALIGNANT BRAINSTEM GLIOMAS --
CHILDHOOD
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ALTERNATE NAMES
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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
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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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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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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Physical findings: Although not pathognomonic of DIPG, signs and symptoms are related to location of
the tumor and may include:
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Loss of balance, difficulties with walking, worsening handwriting, or abnormal speech;
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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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Increased intracranial pressure; and
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ICD-9: 191.7
ICD-10: C71.7
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PROGRESSION
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The average age at diagnosis is 5 to 9 years of age. DIPG has a high rate of recurrence
or progression. DIPG often follows an inexorable course of progression, despite therapy.
A large majority of children die within a year of diagnosis.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describe the diagnostic features of the impairment;
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Pertinent treatment records; and
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Up-to-date progress notes.
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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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113.13 A and B
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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.
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113.13 C 2
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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.
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Equals
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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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