Presentation
Headache.
Patient Data
A mass in the region of the pineal gland is seen with peripheral calcifications and hydrocephalus.
An enhancing pineal mass with a surrounding rim of coarse calcification is present. The mass has relatively low ADC values (ADC 700 x 10^-6 mm^2/s). Moderate lateral and third ventricular dilatation, which anteriorly has remodeled the dorsum sellae. The pituitary is flattened within the sella. The fourth ventricle is normal in size. No transependymal CSF leakage to suggest an acute hydrocephalus.
There appears abnormal signal within the cerebral aqueduct with associated enhancement which could represent tumor extension or prolapse into the cerebral aqueduct. CSF flow studies show reduced flow in the cerebral aqueduct. The remainder of the brain parenchyma is unremarkable. Incidental right occipital developmental venous anomaly.
Conclusion
Enhancing pineal mass has a differential including pineal parenchymal tumor favored over than germ cell tumor given the peripheral distribution of calcification.
Case Discussion
The patient went on to have an endoscopic biopsy and thrid ventriculostomy.
Histology
Sections show fragments of a cellular tumor. The tumor forms sheets of cells, with only the occasional small rosette seen. The tumor cells are monomorphic, with round-to-oval nuclei, fine stippled chromatin, and eosinophilic-to-pale cytoplasm. There are between 1 and 2 mitoses per 10 high power fields. There is no necrosis and no nuclear atypia. There are plentiful calcifications.
IMMUNOHISTOCHEMISTRY: The tumor cells are diffusely and strongly positive with synaptophysin. Occasional scattered cells are positive with NFP. The Topoisomerase proliferation index is approximately 6-7%.
FINAL DIAGNOSIS: Pineal parenchymal tumor of intermediate differentiation (PPTID) (WHO Grade III)
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