Craniopharyngioma

Growing up, Nancy Dell was hypersensitive to light and sound. As a busy wife and mother of four, she suffered from severe migraine headaches lasting three or four days. At 45, doctors performed an emergency hysterectomy on her, thinking she had ovarian cancer. Her tumors turned out to be benign, but recovery was slow and she began having vision problems. More tests revealed she had another tumor – this one in her brain.

Living with a Benign Brain Tumor

By Marcia Swearingen

After another MRI, Nancy learned this tumor was also benign and unrelated to the others. Dr. Timothy Strait, a neurosurgeon with the Neurosurgical Group of Chattanooga, called it a craniopharyngioma. This little tumor with a big name often goes unnoticed until it’s big enough to throw its weight around. According to Dr. Strait, Nancy probably had it since birth.

Craniopharyngiomas are tumors located at the base of the skull near the pituitary gland. They occur most commonly in childhood and adolescence and account for 5 percent to 13 percent of brain tumors in children. In adults, they represent 2 percent to 4 percent of primary brain tumors, usually occurring after age 50.

“It is normal anatomy that gets trapped and ends up being in the wrong place during the process of embryology,” Dr. Strait explains. “It’s not abnormal tissue. It can act as a tumor because it can enlarge, but it’s not a tumor in the sense that we generally attribute. A craniopharyngioma is like the lining of the gut, which constantly grows and sloughs and sheds, and when it does, you’ve got a sack full of old dead cells with no place to go.”

Once craniopharyngiomas grow large enough to press against nearby structures like the optic nerve, pituitary gland or the brain, symptoms manifest and vary depending upon the tumor’s location. The most common symptoms are headache (55 percent to 86 percent), endocrine dysfunction (66 percent to 90 percent) and visual disturbances (37 percent to 68 percent).

If pressure from the tumor causes a disruption of hormone production by the pituitary gland, the patient can experience: growth failure, delayed puberty, loss of normal menstrual function or sexual desire, increased sensitivity to cold, fatigue, constipation, dry skin, nausea, low blood pressure and depression. Visual disturbances can result from pressure to the optic nerves. Involvement of the hypothalamus at the base of the brain may cause obesity, increased drowsiness, trouble regulating body temperature and diabetes insipidus. Other symptoms may include personality changes, confusion and vomiting.

After an MRI or CT scan of the pituitary region confirms the diagnosis of a craniopharyngioma, surgery is the initial treatment. One option is a craniotomy, a surgical operation in which a bone flap is removed from the skull to provide access to the brain. Another option is to access the craniopharyngioma through the nose and sinuses with an endoscope and surgical tools. The treatment option depends upon the tumor and its location.

“These (tumors) can be solid or cystic or a combination of the two,” says Dr. Strait. “A lot of it depends upon the consistency, as well as the location. If you can remove it all, it won’t grow; but the problem is that you are basically born with it. Most of the time, you can’t really separate that capsule off the brain without causing serious injury. What we do is make a window in that capsule and clean out what’s on the inside. There are some cleansing agents that can irrigate the inside and hopefully kill off the remaining functioning cells so they don’t keep replicating, but only about 60 percent of these tumors can be completely removed.”

Because Nancy’s tumor was attached to her hypothalamus, a traditional craniotomy was performed. “After surgery, I felt 110 percent better,” she says. “I instantly got my vision back. I felt better than I had in years.”

However, a year and a half later, Nancy’s headaches started again and she was losing her vision. She went in for tests and discovered that an offshoot of the original tumor was causing the trouble. Nancy had surgery a second time, and Dr. Strait put in a port to administer chemotherapy to inhibit growth.

Dr. Strait explains: “Even though they are benign tumors, they can be malignant in the sense that they can be in a location that is a ‘high rent’ district in the brain. It’s stuck to the hypothalamus and you can’t remove it, and you still have this sack. You’ve made a window in it and it continues to replicate itself. You just have to keep treating it, and sometimes these can be very problematic. They don’t spread like cancer, they don’t metastasize, they just expand from their original core.

“We’ve got a catheter in that tumor, so if it gets bigger, we can always try to draw the fluid out,” explains Dr. Strait, concerning Nancy’s situation. “The chemo probably cut down on the number of viable cells that are still capable of putting out a material that can fill up that space again.”

Because of the tendency of craniopharyngiomas to recur, repeat MRIs or CT scans are advised at least once a year for the first several years after surgery, and sometimes radiation is necessary to make sure the tumor does not return. The good news is that the long-term prognosis is good.

“Because it’s benign, it should never really take your life,” says Dr. Strait. “There is no reason to try to do something to completely take care of it where you end up jeopardizing someone’s life. We’re pretty conservative when it comes to these tumors – very rarely do you take a real aggressive approach.”

Nancy will have another MRI in the near future to see if her tumor has grown since her last scan a few months ago. After the second surgery, she says her recovery was harder and longer. She lost some vision and developed diabetes; however, her vision has stabilized and her diabetes is currently under control. She can drive and is able to be a full-time grandmother to her 11-month-old granddaughter, and she is excited to have another grandchild on the way.

“It’s not fun, but it’s not a death sentence,” Nancy says of her experience. “The struggle of it will definitely test your metal, but it is something you can live with. You just have to make adjustments.”

Nancy credits her success to the support of her doctors, family and friends. Humor also helps this grandmother cope: “You just have to put your big girl panties on and go – it’s not what I envisioned, but it’s still a good life.”

Marcia Swearingen has lived in Chattanooga for 30 years. She has a Bachelor of Science degree in journalism from the University of Tennessee at Knoxville and is currently a board member of the Chattanooga Writers Guild. Marcia and her husband, Jim, have one daughter and live in Hixson, Tenn.