Pineoblastomas

Introduction
 Pineal Gland is midline structure located in the epithalamus, near the
centre of the brain, between the two hemispheres, in a groove where the two
halves of the thalamus join.

 Pineal region neoplasm's constitute only 0.3-2.7% of intracranial tumors.

 They are considered an important clinical entity because of their strategic

location.
Pineal calcification: seen in approximately 40% of normal people by
the age of 20 years and usually less than 1 cm in diameter. Larger
calcifications should raise concerns for underlying tumor.
 Pineoblastomas
• Pineoblastomas comprise approximately 1 percent of all childhood brain
tumors

• Although the tumor may occur at all ages, pineoblastomas are most likely to
arise early in life.

• As is the situation with other embryonal tumors, there is a male predominance.

 Pathology
• Pineoblastomas are indistinguishable from Medulloblastoma, which is why
they have been classified by others as embryonal tumors

• The tumor consists of densely packed, small round cells with minimal cytoplasm and
frequent mitoses
 CLINICAL PRESENTATION
• Compression of the third ventricle
• Hydrocephalus
• Vomiting
• Headaches
• Somnolence
• As they enlarge, they also frequently compress or infiltrate the tectum of the
midbrain, resulting in Parinaud’s syndrome which includes inability to
look upward, double vision and nystagmus
• Due the aggressive nature of the tumor, it may also infiltrate the thalamic area and
present with motor or sensory abnormalities.
 Diagnosis

CT Scan
On CTscan, they are characteristically hyperdense, with calcifications
being seen in approximately one-third of cases.
Axial, post-contrast CT demonstrates an enhancing mass in the pineal
region
MRI
• isointense lesion on T1-weighted images

• heterogeneity on the T2-weighted images

Axial T1 weighted MRI with contrast showing pineoblastoma
 MANAGEMENT
• Pineoblastomas are difficult-to-manage tumors
• Surgical resection
Is difficult and, since the majority of patients undergo an incomplete resection or
biopsy because of the vascularity of the pineal region which means that the degree
of tumor resection can be difficult to determine, especially as regards near-total or
total resection.

• Post surgery
• craniospinal and local boost radiotherapy
• The chemotherapy
 OUTCOME

• Survival rates as high as 70 percent have been noted after treatment with
radiotherapy and chemotherapy, many reports have been much less favorable, with
overall survival rates of 40 percent or less at 5 years.

• Outcome is even poorer, especially in those with disseminated disease at the time of
diagnosis.
Thank You