Craniopharyngioma (metastatic)

Case contributed by Dr Aruna Pallewatte

Presentation

Head ache, bouts of vomitng and blurring of vision .

Patient Data

Age: 6 years
Gender: Male
Race: Asian
Modality: MRI

Selected MRI image at first presentation, after resection of cranipharyngioma and after onset of metastatic recurrence in right temporal fossa

MRI shows a  mass with a cystic component filled with proteinatious fluid in the suprasellar region causing compression of optic chiasma. 

This patient  underwent right parietal craniotomy for suprasellar craniopharyngioma in 2008. The histology indicated a adamantinous craniopharyngioma. 

It was believed to be completely resected surgically and the immediate post operative MRI imaging one year after surgery confirmed complete resection without residual tumor (not shown).  No radiotherapy given. He was on hormone replacement therapy with cortisol and thyroxine.

3 years after the surgery, the child again developed visual symptoms and head ache. MRI done after this second symptomatic presentation demonstrated a cystic lesion in the right temporoparietal region with an enhancing wall. There was no recurrence at the site of surgery. Since the symptoms were mild the patient was followed up for 18 months. 

Therefore  repeat right parietal craniotomy was performed to resect this lesion. The biopsy again confirmed a metastatic seedling of adamantinous craniopharyngioma.

5 years later

Modality: MRI

The MRI after 2 years demonstrated further enlargement of this implant metastasis. 

Case Discussion

Our patient had a recurrence along the surgical route in the right middle cranial fossa. The approximate time period between surgery and recurrence was two years. There were reported 17 cases in the available literature out of which 5 were under the age of 18 years. In the most of the cases  metastases have occurred along the surgical route. There were two recurrences in the spinal cord and the rest were at ectopic sites not related to surgical route 10,11.  In one case report a patient has presented with two metastases 4: one in the temporal fossa at 3 years and another in the frontal space at 10 years 7.

These patients are  usually given hormonal replacement therapy 7. Our patient was treated with thyroxine, cortisol and DDAVP. He underwent  repeat surgical resection of the metastasis  in March 2013.

Two mechanisms have been postulated for these ectopic recurrences.

  1. The first is metastasis often occurs along the surgical route, as in our case
  2. Spreading to distant locations via cerebrospinal fluid has been suggested 3. Therefore regular follow-up is necessary, even after supposedly total resection.

 

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Case Information

rID: 22216
Case created: 20th Mar 2013
Last edited: 7th Feb 2017
Inclusion in quiz mode: Included

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