Pediatric choroid plexus neoplasms.

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.

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.