HPI: 23 year-old male presents with a 3-month history of progressive diplopia and decreased acuity.
PMH: No significant past medical or surgical history.
- AAO x 3. PERRL.
- Mild bitemporal hemianopsia by confrontation (confirmed with automated visual field testing)
- Fundoscopic evaluation reveals no papilledema or optic atrophy.
- Other cranial nerves intact.
- No gross motor or sensory deficits.
Initial evaluation and course
- Endocrinological workup is unremarkable.
- MR-imaging is performed (shown) and oral Decadron is initiated which results in resolution of diplopia.
MRI Brain reveals a suprasellar mass that is isointense on T1W images (A). There is slight hyperintesity in both T2W (B) and FLAIR images (C). Edema along the right optic tract is seen in both the T2W and FLAIR images. There is intense and slightly heterogenous enhancement on T1W Gadolinium contrasted images (D). The coronal (E) and sagittal (F) MRI images reveal the suprasellar location of the mass along with the relationship to the third ventricle. CT head without contrast through the lesion reveals no calcification (G).
1. In your opinion, what is the most likely diagnosis in this patient?
2. After a thorough pre-operative assessment is completed and no pathognomonic findings identified, a decision to proceed with surgery is made. Which approach would you utilize for this patient?
3. During the surgical procedure, you indenitfy the mass within the optic chiasm with no extra-axial component. A small tissue specimen is obtained. The frozen section biopsy returns as favor low grade glioma. How would you proceed?
4. Which of the following describes you?
5. I practice in one of the following locations.
6. Please add any suggestions or comments regarding this case:
- Good interesting case case
- Need to see the whole study
- Nil Specific
- Skull base approach with ozo
- the key here is the evidence of tumor extension/edema along the right optic tract. this is a tumor seen more often in children and not necessarily always with NF-1. Likely no operation needed in the first place
- Subfrontal/ pterional should be one approach option.
- Interesting case....pituutary seen separate from the lesi8on.transcranial shiuld b the way to go.
- I might consider an internal decompression particularly if the tumor is discernible from the optic apparatus
- very interesing clinical situation.Radiosurgery can be a better option for its management.
- Even if low-grade gioma is confirmed, resection should be better, as far as the optic nerves can be dissected and preserved.
- It was interesting to think about this case. More MR images would have been more helpful for decision making. Intra-op well localized, nodular lesion would favor gross total resection.
- I am actually in Jamaica where we have no access to stereotactic radio surgery/therapy but spent most of my professional life in Canada in a large centre This patient would have to travel abroad if he could afford it or accept close follow up -+ conventional external beam radiotherapy there is limited access to MRI for follow up as most are private so again his resources would be relevant
- Teratoma should be included in the differential diagnosis
- The pathologists in our community lack expertise in neuropathology and thus a frozen section diagnosis based upon one small specimen that is inconsistent with my pre-operative image-based and intra-operative visual-based diagnosis would be questioned everytime by me and lead me to obtain further biopsies for intra-operative confirmation of diagnosis in order to make further treatment decisions before closure.
- More t1with contrast axial and coronal are needed for proper assessment
- The management depends on the set up. However working in a government set up I would like to confirm the diagnosis and if it is consistent with optic pathway Glioma, I will like to decompress the lesion. If not possible to separate the tumor from the optic pathway, i will subject the patient to radiotherapy and follow the patient with visual fields and imaging.
- Excellent question.
- for resection of optic glioma it depends on the consistency of the tumor. If it is possible tobe aspirated itis better to remove as much as possible. After histology need to be consulted with radiotherapy as well.
- Would try to stay within the tumour thru the small opening made in the chaism and core the tumour from within as much as possible. This was not part of your options.
- If we know the tumour is low grade glioma the management strategy should be chemo- and radiosurgery.
- need radiation therapy
- Since the patient has a NMR with evidence of edema in the rigth optic tract we can think in a glioma of the optic tract.
- Very interesting case and helpful to make decisions in the real live.
- no comment.
- fig 2 wont enlarge
- Consideration to follow the lesion initially, versus surgery, could be entertained.
- nice case
- I donothave personal experiencewith Hypothalamic hammartomas butI believe the resection can be done by an expeienced neurosurgeon.