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Internet Search Leads Patient with Rare Pituicytoma to Cedars-Sinai for Endoscopic Removal

After a year’s search, John Siracusa of San Mateo in Northern California finds article written by neurosurgeon Adam N. Mamelak, MD, and comes to Cedars-Sinai for treatment

Los Angeles - July 1, 2013 – John Siracusa’s doctors in the San Francisco area expected to be able enter through his nostrils to biopsy and remove a tumor behind his pituitary gland. But they didn’t expect to find a very rare, vascular tumor called a pituicytoma. When the lesion started to bleed, they quickly retreated, later telling Siracusa they would have to go in again, this time through the top of his head.

But Siracusa went online and found a medical journal article written by neurosurgeon Adam N. Mamelak, MD, co-director of the Pituitary Center at Cedars-Sinai Medical Center, who specializes in endonasal surgical procedures. The article described his experience using this less invasive approach to remove a similar tumor. With the help of Congresswoman Jackie Speier, Siracusa, a Vietnam veteran, persuaded the Department of Veterans Affairs to cover the cost of the procedure.

“Pituicytomas are benign tumors in that they don’t spread to other parts of the body and they grow very slowly. But when they start causing symptoms, they need to be reduced in size or taken out, and if you don’t remove them entirely, they tend to grow back,” Mamelak said, adding that only about 55 cases of pituicytomas have been published in the medical literature. As the tumors grow, they can damage the pituitary and rob the patient of normal hormone production.

“The standard approach – opening the cranium and going down through the top of the head – carries significant risks, including blindness, because surgeons must work directly between the optic nerves and other critical structures that lie above the tumor’s position. The elegance of the endonasal approach is that it avoids all those structures. We go in underneath them,” he said.

Mamelak is one of a few neurosurgeons who use an endoscope – a camera lens at the tip of a long tube – and minimally invasive tools to remove tumors near the pituitary. The scope fits through a nostril and provides a panoramic view of the surgical site. But the location of Siracusa’s tumor made his surgery even more challenging.

“This is a much more involved operation than the more common pituitary surgery. It’s called an expanded endonasal approach because it is really intracranial surgery, where we go deep into the brain structures. We open up a much larger area of bone and open the membrane – the dura – that separates the brain from the skull. We’re working deep inside in an area called third ventricle. It takes a series of specialized techniques to get in and another series of techniques at the end to rotate tissue from inside the nose to function like a flap to cover the opening,” Mamelak said. “The method has tremendous advantages over a craniotomy for these sorts of tumors, but in about 8 percent of cases spinal fluid may leak through the patch, setting up the possibility of infection. With careful observation, these usually can be detected and corrected quickly.”

Siracusa didn’t know he had a tumor until he wanted to get a new eyeglass prescription two years ago. The doctor, unable to correct his vision to 20/20, referred Siracusa for an MRI, which revealed the growth. Doctors at a hospital in Northern California made the first attempt to remove it.

“When they opened me up, they saw that the tumor was vascular and they knew right away they couldn’t do it. They closed me up because, they said, there was no way to get that out through the nose,” Siracusa recalled, adding that two other neurosurgeons agreed. But the open craniotomy made no sense to him.

“It took a while before I found Dr. Mamelak – about a year. But through the Internet, in September of last year I came across an article that Dr. Mamelak had written in 2007 about operating on a patient with a pituicytoma. He had successfully removed the entire tumor. When I read that, I was thrilled,” Siracusa said. “Dr. Mamelak’s email address was on the article and I simply emailed him and, surprisingly, he emailed me back and said, ‘John, if you can send me your MRI, I’ll take a look and let you know if I can do this for you.’ I sent him my MRI, and he wrote back, ‘Yes, it can be done.’”

With medical documentation provided by Mamelak and the support of Speier, VA officials agreed to cover the cost of Siracusa’s surgery, performed on Jan. 8 at Cedars-Sinai.

“The very next day, there was no swelling. No nothing. It was as if nothing had even happened,” said Siracusa, who went home three days after the operation and eagerly resumed his life.

“John’s procedure and hospital stay went very well, and he’s doing great,” Mamelak said. “With the standard craniotomy, it’s very easy to injure the optic nerves and/or leave tumor behind. The cost, complications, length of hospitalization and recovery are very different from those associated with the endonasal approach, which I believe is not only less invasive but also more effective. It’s important for patients to seek out subspecialty centers that offer these extended procedures, because it takes a lot of experience to develop a high level of expertise.”

Siracusa, who served three years in the military and volunteered for duty in Vietnam, has worked for several companies in the years since, but now he’s semi-retired and working for himself.

“I always loved to sing. So now I sing in places like senior centers and social clubs around the Bay Area,” said Siracusa, who performs classic songs of Cole Porter, Burt Bacharach, Neil Diamond and others. “They say that if you love what you do, you never work a day in your life. So I’m doing what I love to do. And it’s very fulfilling, because I hope it gives other people a lot of pleasure, too.”