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Pinealoblastomas in children.

Vicente Cuccia1, Facundo Rodríguez, Fernando Palma

  • 1Unit of Pediatric Neurosurgery, Hospital Nacional de Pediatría, "Prof. Juan P. Garrahan", Combate de los Pozos 1881, 1245, Capital Federal, Buenos Aires, Argentina. vcuccia@fibertel.com.ar

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|March 24, 2006
PubMed
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Pinealoblastomas (PB) are rare pediatric brain tumors with a poor prognosis. This study analyzed 12 PB cases, highlighting aggressive tumor behavior, treatment challenges, and proposing a management algorithm for improved outcomes.

Area of Science:

  • Pediatric neuro-oncology
  • Central nervous system tumors
  • Pediatric oncology

Background:

  • Pinealoblastoma (PB) is an extremely rare central nervous system (CNS) tumor primarily affecting children.
  • PB is often grouped with other pineal parenchymal tumors (PPT), leading to inadequate characterization.
  • This study focuses on a series of pediatric PBs to better understand their clinical aspects.

Purpose of the Study:

  • To analyze clinical features, treatment strategies, prognosis, recurrence, and mortality in pediatric pinealoblastomas.
  • To characterize the behavior of PB in a pediatric cohort.
  • To propose a treatment algorithm for pediatric PB patients.

Main Methods:

  • Retrospective analysis of 12 pediatric pinealoblastoma (PB) cases from a larger cohort of 1,350 CNS pediatric tumors.

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  • Evaluation of clinical presentation, diagnostic imaging (CT/MRI), surgical interventions, adjuvant therapies (radiotherapy, chemotherapy), and outcomes.
  • Assessment of tumor recurrence, survival rates, and patient mortality.

    • Main Results:

    • Pinealoblastoma constituted 0.89% of CNS pediatric tumors in the series (12/1350).
    • The mean age of affected children was 7 years, with a male-to-female ratio of 8:4.
    • All patients presented with increased intracranial pressure; 8/12 had ocular symptoms. Hydrocephalus was universal (12/12).
    • Recurrences were observed in 8/12 cases, with a mean survival of 29.55 months for deceased patients.
    • One- and 5-year survival rates were 66.6% and 50%, respectively.

    Conclusions:

    • Pinealoblastomas (PB) exhibit aggressive behavior and a poor prognosis in pediatric patients.
    • Early diagnosis and aggressive multimodal treatment are crucial for managing pediatric PB.
    • A proposed treatment algorithm aims to optimize management strategies for this rare tumor.