Collaborative Management of Pediatric Brain Tumors

MRI of medulloblastoma in 4-year-old boy

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Recently Nicholas M. Wetjen, M.D., a pediatric neurosurgeon at Mayo Clinic in Minnesota, helped remove a tumor that had eroded through the bones of the skull and face of a young child.

The surgical team conducting the procedure included Dr. Wetjen and two skull-base surgeons: Michael Link, M.D., a neurosurgeon, and Eric Moore, M.D., an otorhinolaryngologist. Each contributed his particular expertise on how best to approach the tumor and reconstructive surgery.

This surgery is a typical example of the depth of expertise so often needed to address the complicated care of children with brain tumors. Mayo teams include specialists who collaborate in every aspect of pediatric care at Mayo Clinic's T. Denny Sanford Pediatric Center and the Eugenio Litta Children's Hospital.

Symptoms: Specific and Nonspecific

Recognizing and diagnosing the symptoms of brain tumor may take longer in infants and children than it does in adults. At Mayo Clinic, once a tumor is suspected, the efficient integration across pediatric neuro-oncology, neurology, neuroradiology, neurosurgery, and neuropathology smoothes the diagnostic process — a process that may take one to several days compared with weeks in many medical centers.

Some children have obvious neurologic signs, such as focal motor or sensory deficits or asymmetric motor skills, which suggest the diagnosis of a tumor. Other readily recognized neurologic symptoms are seizures, hydrocephalus, balance problems, lack of coordination, and failure to reach developmental milestones or the loss of those previously gained.

A Craniopharyngioma in 8-year-old boy

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Often symptoms, such as vomiting and irritability, however, are less specific to a neurologic disorder. For example, persistent vomiting over the course of two to three weeks without other gastrointestinal tract or respiratory tract signs or fever might be a response to pressure within the brain from a tumor. Other nonspecific symptoms are lethargy, persistent headache, failure to gain weight, and frequent thirst, drinking, and urination. In older children, the signs may include head tilt and eye deviation.

Collaborative Diagnosis

The initial work-up is conducted by one of Mayo's 10 pediatric neurologists or by one of its pediatric neuro-oncologists. Cynthia J. Wetmore, M.D., Ph.D., a pediatric neuro-oncologist, notes that "as soon as a child comes to the clinic, the patient and the patient's family meet with a pediatric oncologist, a pediatric neurologist, and often a radiation oncologist as well as a pediatric neurosurgeon." Dr. Wetjen adds, "It is rare that a new patient would not see us all in one day."

Ependymoma in an 8-month-old girl

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Imaging is critical to detect and localize brain tumors, but it can be difficult to conduct in children. Tests for brain tumors in children at Mayo include CT scan, MRI with diffusion-weighted and gradient-echo imaging, MR spectroscopy, and, in some cases, PET scans. Imaging studies require that children be sedated, with particular attention to age and severity of illness. Anesthesiologists with special expertise in pediatrics are another important adjunct to Mayo's pediatric brain tumor team.

If seizures are a presenting symptom, pediatric epilepsy specialists help determine if the tumor alone is causing the seizures. Mayo has extensive experience in video monitoring for epilepsy, should that be needed before surgery. Pediatric neuropsychologists provide behavioral and developmental testing. Specialists in pediatric endocrinology and genetics are also available as needed.

Surgical and Medical Management

Surgical resection is considered the best treatment for most types of childhood brain tumors. Because both chemotherapy and radiotherapy pose risks for the developing brain, surgically removing as much of the tumor as possible is critical. Dr. Wetjen notes, "Too often we see patients in whom the tumor has been incompletely resected or an inadequate tissue sample has been collected for biopsy. Then we are dealing with both the adverse effects of previous surgery and the need to reoperate."

At Mayo, an initial biopsy specimen can often be taken and evaluated during surgery, then sent for further pathology testing. The histology results help determine the best management, whether it be surgical resection, chemotherapy, radiotherapy, or some combination of treatments. As Dr. Wetmore points out, "Even if the tumor is completely resectable, adjunct chemotherapy or radiotherapy often improves long-term survival, except in children younger than 3 years in whom radiation is not recommended." Gamma knife surgery is rarely recommended for children as a primary treatment.

Nadia N. Laack, M.D., a radiation oncologist, and her colleagues are investigating the long-term effects of radiation on cognitive development and growth. Dr. Wetmore, who directs a basic research laboratory, and colleagues are also investigating tumor resistance to radiation in children and ways to target tumors without harming normal brain development. Mayo Clinic holds a National Cancer Institute (NCI) Specialized Programs of Research Excellence (SPORE) grant in brain cancer under which numerous other pediatric research initiatives are ongoing.

Recently joining the group is pediatric neurologist Gesina Keating, M.D., who has expertise in the care of children with tumors of the nervous system, as well as the neurologic complications of other cancers in children. Her interests range from the acute management of newly diagnosed tumors in children to their ongoing care and long-term follow-up, with plans to establish a transition program for the continued neurologic needs of patients as they reach adult age.

Mayo offers state-of-the-art neurosurgical techniques and technology, including the ability to conduct electrophysiologic and speech and language monitoring during surgery, frameless stereotactic surgery for real-time intraoperative localization and navigation, and intraoperative magnetic resonance imaging (iMRI). Mayo neurosurgeons have extensive experience in tumor resection in both the adult and pediatric practice. Experience matters. A recent study found that experience is a major factor affecting pediatric brain tumor surgical outcomes.

Unlike adults, 95 percent of children who have chemotherapy in the United States are treated through protocols specified by NCI. Most of those involved in the direct management of pediatric brain tumors at Mayo Clinic in Minnesota are members of the Children's Oncology Group (COG) through NCI. All postsurgical therapy is directed by a member of COG.

Taking Care of the Whole Patient and the Whole Family

The T. Denny Sanford Pediatric Center at Mayo Clinic in Minnesota is located in the Gonda Building. It serves more than 45,000 children a year. Every aspect of the Center, from the nature-themed environment to the specialized furniture, restrooms, and drinking fountains at several different heights, is designed for children of all ages. The Center is a colorful, welcoming environment, but more important, it is staffed by pediatric experts from every specialty who, working together in a central location, can coordinate care efficiently and to the patient's best advantage.

Mayo's Eugenio Litta Children's Hospital is staffed by more than 150 physicians. Distinct from, but located within Mayo's Saint Marys Hospital, Eugenio Litta Children's Hospital provides patients with access to all Mayo's technological advances and the expertise of Mayo's entire staff of physicians. The facility includes playrooms for all ages and a teen lounge. Tutors from the Rochester Public School system are available for children absent from their own schools for long periods. Rehabilitation specialists, including physical therapists and speech-language pathologists and others with expertise in pediatrics, work with patients as needed during recovery.

Diagnosis of a brain tumor in a child affects the entire family. During the hospital experience, social workers as well as child-life specialists who support children and families before, during, and after surgery work with patients and their families. The hospital has special family support space with laundry, kitchen, and shower facilities. Pets are permitted to visit. And because many patients travel great distances, Mayo is fortunate to have the Ronald McDonald House located only blocks from both the clinic and hospital. Supported by charitable contributions and available to patients and their families with a Mayo Clinic referral, Ronald McDonald House has 45 rooms that are available at a minimum cost and accommodate up to five persons per room, for as long as needed—be it six days or six months.

Dr. Wetmore notes that at Mayo Clinic, "We have a commitment to our pediatric brain tumor patients and their families that extends from our breadth and depth of interdisciplinary expertise to our research on brain tumor treatment and to our child-centered, state-of-the art facilities."