Neurosurgery paper on survival for rare tumor receives CNS paper of the year

Pediatric neurosurgeon Paul Klimo, MD, was awarded the Pediatric Paper of the Year from the Congress of Neurological Surgeons (CNS) at the 2018 CNS Annual Meeting in Houston, Texas. His paper, published in Neurosurgery, titled “Pineoblastoma – The Experience at St. Jude Children’s Research Hospital,” documented the outcomes of 41 patients with pineoblastoma who underwent multimodal therapy and evaluated the impact surgical resection had on survival.

Pineoblastomas are rare, malignant embryonal tumors that account for less than one percent of all pediatric brain tumors, according to Klimo. In addition, they are more common in young children. Klimo’s study represents the largest pediatric series of pineoblastoma cases to date. The results of the study showed the effect of age and cancer metastasis on the likelihood of death or treatment failure. Of the 41 patients studied, 18 had succumbed to their tumor and 19 had experienced tumor recurrence or progression. Children who experienced treatment failure were younger (less than 5 years old) and more likely to have metastatic disease at diagnosis. Patients 5 years or older who did not have metastatic disease and had 90 percent or more of their tumor removed had an excellent chance of survival. No patient in this subgroup died as a result of their cancer.

Klimo hopes that future studies will build on this research and uncover additional effective ways to improve outcomes for children with pineoblastoma.

“Our knowledge of molecular derangements that give rise to pineoblastomas will expand and may impact our surgical management,” said Klimo. “We hope that unraveling the molecular blueprint of pineoblastoma could lead to targeted chemotherapy and understanding genetic links.”

Le Bonheur is home to the nation’s largest pediatric surgical brain tumor program working in partnership with St. Jude Children’s Research Hospital. Approximately 200 brain tumor operations are performed each year. Children in Le Bonheur and St. Jude’s joint brain tumor program have some of the highest survival rates and have access to the most advanced technology available for treatment and support.

Kara A. Parikh, Garrett T. Venable, Brent A. Orr, Asim F. Choudhri, Frederick A. Boop, Amar J. Gajjar, Paul Klimo; Pineoblastoma—The Experience at St. Jude Children’s Research Hospital, Neurosurgery, Volume 81, Issue 1, 1 July 2017, Pages 120–128, https://doi.org/10.1093/neuros/nyx005