Pineoblastoma-The Experience at St. Jude Children's Research Hospital.
BACKGROUND
Pineoblastomas are rare, supratentorial, primitive neuroectodermal tumors.


OBJECTIVE
To document outcomes with multimodal therapy and evaluate the impact that the degree of surgical resection has on outcome.


METHODS
A departmental brain tumor database was queried to identify all patients with pathologically proven pineoblastoma who were treated from January 1997 to June 2015 at St. Jude Children's Research Hospital. For each patient, we recorded demographic, pathological, radiological, surgical, and clinical follow-up data. The effect of degree of surgical resection on survival outcomes was analyzed.


RESULTS
Forty-one patients (21 male, 20 female) treated for pineoblastoma were identified. The median age at diagnosis was 5.5 years (range 0.4-28.1) and the median follow-up was 34.5 months. Nineteen patients experienced tumor relapse with a median progression-free survival of 11.3 months, and 18 ultimately succumbed to their disease. Patients who died or experienced treatment failure were younger (median, 2.69 vs 6.5 years, P = .026) and more likely to have metastatic disease at diagnosis (12 [63.2%] vs 5 [22.7%], P = .012). When analyzing only patients 5 years of age or older with focal disease at presentation, those who had a gross total resection or near-total resection-compared with subtotal resection or biopsy-had greater overall survival (75.18 vs 48.57 months), with no patients dying as a result of their cancer.


CONCLUSIONS
Poor prognostic variables for children with pineoblastoma include young age, metastatic disease at presentation, and tumor relapse. For patients older than 5 years with focal disease, maximal tumor resection should be the goal.
School of Medicine, Louisiana State University Health Science Center, New Orleans, Louisiana.|Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee.
Kara A
KA
Parikh
Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee.|Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee.
Garrett T
GT
Venable
Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee.
Brent A
BA
Orr
Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee.|Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital, Memphis, Tennessee.|Department of Radiology, University of Tennessee Health Science Center, Memphis, Tennessee.
Asim F
AF
Choudhri
Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee.|Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital, Memphis, Tennessee.|Semmes Murphey Clinic, Memphis, Tennessee.
Frederick A
FA
Boop
Department of Oncology, Division of NeuroOncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
Amar J
AJ
Gajjar
Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee.|Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee.|Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital, Memphis, Tennessee.|Semmes Murphey Clinic, Memphis, Tennessee.
Paul
P
Klimo
D000293:Adolescent; D000328:Adult; D001932:Brain Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D008297:Male; D010870:Pineal Gland; D010871:Pinealoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D017211:Treatment Failure; D016896:Treatment Outcome; D055815:Young Adult
Children; Pineoblastoma; Primitive neuroectodermal tumor