Dr. Jonathan Finlay is a world-renowned pediatric neuro-oncologist specializing in the management of children, adolescents, and young adults with brain tumors. Dr. Finlay’s work aims at minimizing the long-term side effects of radiation therapy through his Head Start clinical study protocols, now in their fourth iteration, which have demonstrated significantly improved survival and quality of life for pediatric brain tumor patients around the world. Most recently, Dr. Finlay was awarded the Lifetime Achievement Award at the biennial International Pediatric Neuro-oncology Symposium (ISPNO) in Hamburg. Below, Dr. Finlay sheds light on childhood cancer diagnoses to improve the lives of children worldwide.
First, ALL childhood cancers are UNCOMMON!
Acute lymphoblastic leukemia is the most common type of childhood cancer. It is not difficult to diagnose, and it is rarely a source of debate amongst pediatric hematologists and oncologists.
However, brain cancer—the second most common type of childhood cancer and a major cause of cancer-related and disease-related death amongst children and adolescents up age to 21—is often very difficult to diagnose, even amongst experienced institutions and institutional pathologists.
Diagnosing Brain Cancer in Children and Adolescents Can Be Difficult, Even for The Most Experienced
Providers seek information to make their diagnosis. For example, is the brain tumor low-grade or high-grade (malignant)? Is it a glial tumor or a neuronal tumor (or some combination of both)? The answers to these and other questions are crucial, as they dictate the prognosis and outcome for the child. They also dictate the best therapy required: Observation only? Or surgical resection, radiation therapy, chemotherapy—or some combination of all three?
Our understanding of molecular genetic differences between different tumors has grown rapidly. As a result, we can more accurately diagnose tumors, predict the outcome, and specify specific treatments—including highly specific tumor-targeted therapies.
However, to paraphrase a saying of Socrates: The more we think we know, the more we don’t know.
Why Second Opinions are Crucial for Children with Uncommon Cancers
Permit me to provide you with an example that highlights the pivotal need for second opinions, including second pathological opinions for uncommon childhood cancers:
A four-month-old child became acutely ill with marked irritability and vomiting. A brain MRI revealed severe hydrocephalus or fluid on the brain due to a small tumor blocking the normal flow of cerebrospinal fluid. Cerebrospinal fluid, known as CSF, is the fluid that bathes and protects the brain at the level of the brain known as the tectum. A neurosurgeon at a major U.S. institution placed a shunt to permit the CSF to drain into the child’s abdomen, and the child made a rapid and complete recovery.
Because tumors of the tectum commonly present in this manner, are very low-grade, and require no further treatment, an approach of observation only was recommended. A follow-up MRI was performed three months later, at which time the hydrocephalus had completely resolved, but the tumor appeared much larger. The same neurosurgeon undertook a stereotactic tumor biopsy, yielding a tiny fragment that appeared to be a low-grade astrocytoma. This is when everyone involved became a bit too clever. The tumor fragment was sent to a well-known pathology center to determine, with fairly simple but novel methods, the tumor’s molecular characteristics. The tumor was reported to display a rare but highly lethal mutation which, under then-new guidelines, would render the tumor highly malignant.
At that point, the family sought the opinions of FOUR different leading pediatric brain tumor centers in North America. One center recommended palliative (non-curative) radiotherapy; two others recommended 18 months of standard chemotherapy; and the fourth recommended a highly intensive course of chemotherapy, including a marrow transplant. All four believed that the molecular abnormality was indeed predictive of a highly malignant cancer and likely unfavorable outcome.
The family then sought my opinion on how to manage their child’s medical care. My local pediatric neuroradiology expert and I did not feel the tumor had grown. Rather, it only appeared so because the relief of the marked pressure on the brain had permitted the tumor to breathe and thus to enlarge—but not due to tumor growth! In addition, in my 30+ years as a pediatric brain tumor doctor, I had never heard of nor encountered an infant with a MALIGNANT tumor of the tectum. But who was I to question this all-new supposedly powerful molecular genetic diagnostic tool?
I sought the opinion of two other pediatric brain tumor specialists with large practices and almost as many grey hairs on their heads as on mine. They both concurred that they had never encountered an infant with a malignant tumor in this location of the brain at such a young age. So, at this point, I contacted an internationally renowned pediatric brain tumor pathologist who concurred that malignant gliomas do not arise in this location of the brain. Moreover, the pathologist agreed that we should be very skeptical of the report of this particular mutation in the tumor as identified by this simple methodology, because it can be very inconclusive, especially in tiny tumor tissue samples. Some years later, I had the original tumor reviewed by the original neuropathologist, who agreed that, at best, the molecular diagnosis was inconclusive.
My recommendation to the family was to observe their child—nothing more. I am pleased to report that several years later, the tumor, rather than growing, has spontaneously shrunk. The child is growing and developing normally, avoiding both radiotherapy and chemotherapy.
The best specialists to perform such second opinions are, therefore, not only individuals who have the experience and expertise to recognize when a diagnosis fits—or does not fit—all the clinical and imaging characteristics (or, when is a tiger really a kitty cat!), but one who also has the humility to seek additional input from colleagues when encountering uncommon diagnoses.
About the Author
Jonathan Finlay, MD, ChB, FRCP, is the Pediatrician-in-Chief of MORE Health for Kids. He is an Emeritus Professor of Pediatrics at The Ohio State University College of Medicine and is the Principal Investigator of NEXT / Head Start 4 Consortium Trial at Nationwide Children’s.
