Introduction: Diffuse pontine gliomas are truly inoperable tumors that invariably result in the early death of the patient. Misdiagnosis has devastating clinical consequences. This clinical series presents the surgical outcomes of patients who were initially diagnosed as having an inoperable tumor and who subsequently had surgical intervention. The aim of this retrospective study was to ascertain whether previous radiotherapy and/or chemotherapy had a negative impact on short and long-term outcomes.

Methods: A retrospective review was undertaken of all surgical patients who were initially diagnosed with an inoperable, diffuse brainstem tumor with at least 6 months follow-up. The patient's chart, imaging and long-term survival data, if available, were collected for review. IRB approval for the study was obtained.

Results: Of 156 patients who were operated on by the senior author, 52 fulfilled the selection criteria. Only 12 patients presented having had a previous biopsy. 28 patients had been given or were in the process of having radiotherapy and/or chemotherapy. 19 patients presented having had no medical or surgical intervention. There was no statistical difference in the 2 groups, previous intervention and no previous intervention, when looking at PFS and OS. However, there was a significant difference in quality of life measures and a trend towards longer overall survival in those patients with malignant tumors who had surgical resection before adjuvant treatment.

Conclusions: Diffuse brainstem tumors are inoperable and incurable. Focal brainstem tumors are surgically treatable and sometimes curable. Given the devastating consequences of misdiagnosis of a brainstem tumor clinicians involved in their management need to get it right. This series shows that those patients with low grade or extra-axial tumors live longer or may be cured and those with malignant tumors enjoy better quality of life by having surgery before radiotherapy.

Patient Care: This clinical research will be a timely reminder of the importance of accurate pre-operative diagnosis based on MRI imaging and the importance of following the basic surgical tenet of cytoreduction prior to the administration of radiotherapy. This will save and improve the quality of life of many patients

Learning Objectives: By the conclusion of this session, participants should be able to 1) Describe the importance of distinguishing diffuse from focal brainstem tunors...2) Discuss in small groups the ideal treatment algorithms for those patients presenting with a surgically treatable brainstem tumor...and 3) Identify operative approaches to these lesions.

References: Teo C, Siu TL. “Radical resection of focal brainstem gliomas: is it worth doing?” Childs Nerv Syst Nov;24(11):1307-14, 2008 Teo C, Broggi M. “Surgical outcome of patients considered to have “inoperable” tumors by specialized pediatric neuro-oncological multidisciplinary teams” Childs Nerv Syst 26(9): 1219-1225; 2010