Case Study Series: Recurrent Craniopharyngioma

Dr. Ramin Javahery
Jul 24, 2017 · 3 min read

This patient is a 9 year-old with a history of a craniopharyngioma. His tumor was initially diagnosed in 2010 and he was managed at a academic hospital. He had a partial resection in 2010 for his tumor. The tumor recurred and in 2013 he needed a second resection of his tumor. He did not receive radiation therapy after either resection. Unfortunately, he was blind in the left eye as a result of the tumor and the first surgery but the vision in the right eye was still good.

Craniopharyngiomas are benign tumors that occur at the base of the brain in the area of the pituitary gland going up to the 3rd ventricle. Even though they are benign, they behave very aggressively. The treatment involves surgery with the hope of achieving a gross total resection. If we can resect it completely, then no further treatment is needed unless it regrows. If we cannot resect it completely, then patients will need radiation therapy to destroy the residual disease.

The treatment of these tumors can have significant morbidity (complications) including vision loss, hormonal dysfunction (very frequent and almost an expected consequence of treatment in most large tumors), and behavioral/memory changes related to manipulation of the hypothalamus (base of the brain).

There are multiple different approaches that can be used to approach these tumors depending on the exact location of the tumor. The main approaches include: endoscopic endonasal (through the skull base), interhemispheric transcollosal (through the top of the head between the two sides of the brain), and transsylvian (through the side of the brain).

His surgeries had been transsylvian, which for his tumor, I believe, was the best approach. Unfortunately, his tumor had consistently recurred.

He presented with vision loss earlier this year. He basically lost the vision he had in the right eye one week prior to seeing us. He was completely blind. As a matter of fact, the outside surgeons had recommended against surgery. They recommended moving to radiation therapy but his Mom brought her to Miller Children’s Hospital for another opinion.

The ophthalmologists at Miller Children’s Hospital saw him and felt that we could not save his vision. I agreed with them. I did recommend that we resect the tumor given its size. The goal was tumor control, not to regain vision. Surgery was high risk because he had had two prior surgeries and these tumors are very difficult to resect if they recur. They tend to wrap around the nerves and arteries of the skull base which is why the outside neurosurgeons did not want to operate on his tumor.

We decided to proceed with surgery after discussing all the associated risks. I again made it clear to his mom that surgery would not bring back his vision. He underwent his 3rd craniotomy for resection of the tumor. We were fortunately able to achieve a gross total resection.

After surgery he had no new neurological issues and amazingly, his vision returned in the right eye. I was astonished but happy to be wrong!

He is currently completing his radiation therapy, mainly because I was not sure I did not leave some small amount of tumor on the pituitary gland/stalk, and has returned to his baseline.

Chief of Adult and Pediatric Neurosurgery at Long Beach Memorial/Miller Children’s & Women’s Hospital Long Beach http://www.cna-ca.com/cna-doctors/ramin-j-javah

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