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July, 2007

For Pituitary Tumors, a Full Court Press
Monica Cisternelli had no inkling of the mass stealthily enlarging in her brain until her right peripheral vision plummeted, seemingly overnight. Referred by her local physician to the Wilmer Eye Institute, the 38-year-old marine biologist was stunned at how quickly neuro-ophthalmologist Paul Hoffman nailed the problem. “Within 30 seconds,” recalls Cisternelli, “he said, ‘I know exactly what you have.’”

An MRI confirmed what Hoffman had deduced—the presence of a large pituitary tumor stretching her optic nerve—and Cisternelli suddenly found herself transformed from a woman who “was never sick, never went to a doctor, never took medication” to a patient needing the expertise of a cadre of subspecialists.

Estimated to occur in one of every 10 people, pituitary growths are almost invariably benign and often clinically insignificant, says endocrinologist Roberto Salvatori. Still, some wreak havoc. They can suppress hormone secretion or launch excess hormone production, producing symptoms that range from the common (fatigue, headaches, sleep loss) to the odd (hirsutism, temper outbursts, changes in facial features). And since not all pituitary tumors are visible on imaging, their diagnosis demands not only adeptness at interpreting their heterogeneous signs but a battery of detailed blood and urine hormone tests. Treatment, which must be tailored to tumor type and size as well as the patient’s age and overall health, spans medical management, radiotherapy and surgery.

In more than two-thirds of cases, Salvatori and fellow endocrinologist Gary Wand can monitor patients for signs of progression or keep their tumors in check with medication. It was for patients like Cisternelli, however, that Salvatori co-founded Hopkins’ Pituitary Tumor Center to ensure that what can be highly complex care is well coordinated.

Cisternelli’s evaluations by Hoffman and Salvatori showed a 4-centimeter growth-hormone-secreting adenoma that had to be removed, meaning her next consultation would be with Henry Brem.

The director of neurosurgery, Brem heads a surgical team fully versed in the intricacies of operating on the pea-size gland that hangs just below the optic nerves. Among their options for resecting microadenomas (tumors smaller than 1 centimeter) are a new endoscopic technique that allows the surgeon to view and remove the mass via an incision in the lining of the nose, and the transsphenoidal hypophysectomy, in which the surgeon makes an incision behind the upper lip and drills a hole through the sphenoid bone. Today, Brem and his group use intraoperative MRI and intraoperative computer navigation to monitor their progress.

For macroadenomas such as Cisternelli’s, removal is trickier. Brem, who believes in starting with the least aggressive mode whenever possible, first debulked her tumor in a transsphenoidal operation. Several months later, because the stiff capsule that had surrounded the growth was still compressing her optic nerve, he performed a craniotomy to remove the remaining husk.

Now, Cisternelli sees Salvatori every six months for follow-up, has a semiannual MRI, and adheres to her regimen of hormone replacement therapy and monthly injections aimed at preventing tumor regrowth and controlling its hormone over-production. “There was some residual tumor after the second surgery,” she says, “but so far, it’s stable. I’m trying everything to avoid radiation therapy.”

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