- A 5 y/o boy presents with recent history of increased headaches and gait difficulty.
- PMH: Dextrocardia, situs inversus, asthma, GERD (s/p fundoplication), obesity, sleep apnea.
He has had two prior episodes of apnea which required ICU admission and felt to be related to his asthma.
- Medications: asthma inhalers
- Bilateral papilledema, right lateral nystagmus, nasal voice, dysarthric speech
- Motor 5/5 bilaterally with mild increased lower extremity tone
- DTR 3+ throughout
- Sensory exam: normal
- Right intention tremor
Figure 1-4. MRI Head and Neck (Below)
Figure 5. Intraoperative pathology showed (Below)
1. What is the most likely diagnosis for this lesion?
2. What is the next best step in management?
3. Any additional or further imaging required?
4. Which intraoperative monitoring tools would you utilize during surgery?
5. Please add any suggestions or comments regarding this case:
Based on neuroimaging, the lesion appears to be a medulloblastoma (midline location. Although, closer examination of the axial MRI with contrast is suggestive of a left mural nodule). As the patient is symptomatic from hydrocephalus, I would place a ventriculostomy drain. I would obtain an MRI of the neuroaxis, to be followed by surgery. However, I would take precautions to ensure that excessive drainage of CSF does not result in upward herniation. The intaop pathology, is indicative of pilocytic cerebellar astrocytoma (eosinophilic granular bodies, Rosenthal fibers). The child will only require resection of the tumor (no radiation or chemotherapy). The prognosis is certainly much better than medulloblastoma.
the imaging features would best fit a cervico medullary brain stem glioma or possibly a cervico medullary haemangioblastoma. As initial management I would consider an endoscopic third ventriculostomy and possibly vascular imaging prior to resection/debulking.
The intraop. pathology photomicrographs is consistent with a pilocytic astrocytoma. The presence of Rosenthal fibers, microcysts and oligodendroglia indicate that, this is a Winston type A cerebellar pilocytic astrocytoma. Therefore, with regards to prognosis, such cerebellar astrocytomas have a 94% 10-year survival.
this is an interesting case. looking at radiology i was thinking this to be an ependymoma but i think it is most likely going to be pilocytic astrocytoma, as also indicated by the perop.histology slide showing rosenthal fibres(although rosenthal fibres are not pathognomonic of pilocytic astrocytomas). maximal surgical resection is indicated .on axial cuts it looks like infiltrating the stem and hence total removal is not likely to be possible.I will also put an EVD asap or do 3rd ventriculostomy in view of his signs of raised ICP as mentioned. Khalid Saeed , Manchester,UK
antenatal/ natal and post natal history? any history of c/c steroid intake with prior h/o asthma.
This is a patient with a cervicomedullary lesion that is causing rasied intracranial pressure due to obstruction of the CSF pathway. Placement of the ventricular catheter is to reduce ICP -- but this should be done under steroid coverage and a followup open surgical procedure to effect maximum tumour removal.
clear cell ependymoma?