ALTERNATE NAMES

Pineoblastoma; Pineoblastoma – Child; Pinealoma – Childhood; Pinealoma – Child; Pinealoblastoma

DESCRIPTION

Pineoblastoma is an aggressive cancerous (malignant) tumor that grows in a part of the brain known as the pineal gland. It occurs mainly in children. These tumors are primary central nervous system tumors that start in the brain and can spread to the spinal cord and beyond.

Pineoblastoma’s fast growth usually causes cerebrospinal fluid (CSF) to build up in and around the brain (hydrocephalus), increasing intracranial pressure. The cause of pineoblastoma is unknown, but specific inherited variants in two genes, RB1 and DICER1 are associated with increased risk for developing pineoblastoma.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: The diagnosis of pineoblastoma is made by:

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  Magnetic resonance imaging (MRI);

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  Computerized tomography (CT) scan;

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  Positron emission tomography (PET) scan;

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  Tissue biopsy; and

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  Cerebrospinal fluid (CSF) testing.

Physical findings: The physical findings of pineoblastoma depend on where the cancer is growing in the brain. Some general symptoms are:

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  Headaches;

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  Nausea;

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  Vomiting;

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  Difficulty with eye movements (nystagmus);

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  Difficulty with balance; and

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  Difficulty walking.

ICD-9: 194.4

ICD-10: C75.3

PROGRESSION

While pineal region tumors can occur at any age, they are more common in children. All tumors, except papillary tumors of the pineal region, occur slightly more often in females than males.

The relative five-year survival rate for children is 50-60%. Many factors can affect prognosis, including tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and response to treatment.

Patients who receive radiation therapy are at risk for long-term endocrine system changes that may lead to problems such as delayed growth, fatigue, and fertility problems. Problems with hydrocephalus can persist in patients who have been successfully treated.

TREATMENT

The first treatment for pineal region tumors is surgery to remove or reduce the size of the tumor and to obtain tissue to determine the tumor type.

Treatments after surgery may include radiation therapy, chemotherapy, and/or clinical trials. Very young children are treated with a combination of chemotherapy medications to delay radiation therapy until the child is older. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available.

Suggested MER for Evaluation:

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

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  Imaging reports such as CT scan or MRI scan;

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  Biopsy or needle aspiration information found in operative notes, pathology reports, summaries of hospitalization or other medical reports that include details of the surgical and pathological findings.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

113.13 B

113.13 C

Listing level severity must be documented.

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