Presentation
Chronic headaches, ataxia, visual symptoms and neck pain.
Patient Data
There is a sharply defined, cystic, intra-axial mass lesion within the right cerebellar hemisphere. There is a mixed intensity (solid and cystic spaces within) and avidly enhancing eccentric mural nodule present. The wall of the lesion remains non-enhancing. There is no associated restriction on diffusion imaging. There are eccentric peripheral flow voids best identified on T1 imaging. There is perilesional edema, focal mass effect with attenuated basal cisterns and brain stem. There is cerebellar tonsillar herniation at 13mm. There is tri-ventricular obstructive hydrocephalus with periventricular trans ependymal edema. There is prominent perioptic CSF and raised intracranial pressure likely accounting for papilledema. Multiparametric MRI brain and cervical spine are otherwise unremarkable.
Microscopy revealed a cystic mass, partially lined by a proliferation of capillaries, characterized by closely packed thin-walled vessels. There are associated large neoplastic stromal cells with eosinophilic to clear foamy cytoplasm and prominent vacuolation. The cells show hyperchromatic nuclei. The cyst wall shows evidence of gliosis. There is no significant necrosis.
The immunohistochemical findings confirmed a cerebellar hemangioblastoma.
Case Discussion
A histopathologically proven case of a right cerebellar hemisphere hemangioblastoma completely excised at laparoscopic surgical intervention. There are typical MRI features as described above. The broad differential diagnosis in children always includes a pilocytic astrocytoma, ependymoma and medulloblastoma. In adults, the differential diagnosis includes glioblastoma or metastases. Arteriovenous malformations and cavernomas also need to be excluded. In this instance, the lesion is sporadic in etiology and the patient does not have von Hippel Lindau (vHL) disease.
Unable to process the form. Check for errors and try again.