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Pediatric choroid plexus neoplasms.

E Chow1, D A Reardon, A B Shah

  • 1Department of Radiation Oncology, St. Jude's Children's Research Hospital, Memphis, TN 38105, USA.

International Journal of Radiation Oncology, Biology, Physics
|April 13, 2000
PubMed
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Choroid plexus papilloma (CPP) has a good prognosis with gross total resection (GTR). Malignant transformation occurred in 2 CPP patients with less than GTR, highlighting the importance of complete surgical removal for these rare childhood brain tumors.

Area of Science:

  • Pediatric neuro-oncology
  • Neurosurgery
  • Pediatric oncology

Background:

  • Choroid plexus tumors (CPT) are rare childhood neoplasms with debated classification and treatment standards.
  • Defining the role of adjuvant therapy is crucial for improving outcomes in pediatric CPT patients.

Purpose of the Study:

  • To contribute to the knowledge base of choroid plexus tumors (CPT).
  • To further define the role and efficacy of adjuvant therapy in managing CPT.

Main Methods:

  • Retrospective review of 14 pediatric patients with choroid plexus neoplasms (10 CPC, 4 CPP).
  • Analysis of treatment strategies including surgery (gross total resection [GTR] vs. subtotal resection [STR]), chemotherapy (CT), and craniospinal irradiation (CSI).
  • Evaluation of patient outcomes based on tumor type, extent of resection, and adjuvant therapy.

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Main Results:

  • Choroid plexus papilloma (CPP) patients treated with GTR had a good prognosis.
  • Two CPP patients with less than GTR underwent malignant transformation to choroid plexus carcinoma (CPC).
  • Localized CPC patients treated with GTR showed favorable outcomes with adjuvant CT and/or irradiation; CSI may not be routinely indicated for M0 status.
  • For CPC patients treated with STR, CT and CSI appeared necessary.
  • Six of 12 survivors experienced significant neuropsychological sequelae.

Conclusions:

  • Gross total resection (GTR) is associated with a favorable prognosis for choroid plexus papilloma (CPP).
  • Adjuvant therapy, including chemotherapy (CT) and craniospinal irradiation (CSI), is essential for localized choroid plexus carcinoma (CPC) following GTR, and particularly for subtotal resections (STR).
  • Craniospinal irradiation (CSI) may not be routinely indicated for M0 CPC patients after GTR, but salvage radiation therapy can be successful.