Pineoblastoma - The Experience at St. Jude Children's Research Hospital

Kara A. Parikh, Garrett T. Venable, Brent A. Orr, Asim Choudhri, Frederick Boop, Amar J. Gajjar, Paul Klimo

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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. CONCLUSION: 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.

Original languageEnglish (US)
Pages (from-to)120-127
Number of pages8
JournalNeurosurgery
Volume81
Issue number1
DOIs
StatePublished - Jul 1 2017

Fingerprint

Pinealoma
Research
Neoplasms
Primitive Neuroectodermal Tumors
Recurrence
Survival
Treatment Failure
Brain Neoplasms
Disease-Free Survival
Demography
Databases
Biopsy

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology

Cite this

Pineoblastoma - The Experience at St. Jude Children's Research Hospital. / Parikh, Kara A.; Venable, Garrett T.; Orr, Brent A.; Choudhri, Asim; Boop, Frederick; Gajjar, Amar J.; Klimo, Paul.

In: Neurosurgery, Vol. 81, No. 1, 01.07.2017, p. 120-127.

Research output: Contribution to journalArticle

Parikh, Kara A. ; Venable, Garrett T. ; Orr, Brent A. ; Choudhri, Asim ; Boop, Frederick ; Gajjar, Amar J. ; Klimo, Paul. / Pineoblastoma - The Experience at St. Jude Children's Research Hospital. In: Neurosurgery. 2017 ; Vol. 81, No. 1. pp. 120-127.
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abstract = "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. CONCLUSION: 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.",
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AU - Gajjar, Amar J.

AU - Klimo, Paul

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N2 - 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. CONCLUSION: 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.

AB - 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. CONCLUSION: 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.

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