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Spinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report
Ramin A Morshed1, Darryl Lau1, Peter P Sun2
1Department of Neurological Surgery, University of California, San Francisco; and.
Insights
Benign choroid plexus papillomas (CPPs) can rarely metastasize, even in children. This case highlights the need for total neural axis imaging to detect rare drop metastases from CPPs.
Area of Science:
- Neuro-oncology
- Pediatric Neurosurgery
Background:
- Choroid plexus papillomas (CPPs) are typically benign intracranial tumors.
- While generally benign, CPPs have been reported to metastasize in both adult and pediatric patients.
Observation:
- A 14-year-old boy presented with obstructive hydrocephalus due to a fourth ventricular mass.
- Spinal MRI revealed a concurrent intradural sacral lesion, concerning for a drop metastasis.
- Both lesions were surgically resected and histologically confirmed as benign CPP (WHO Grade I).
Findings:
- This case represents a rare instance of metastasis from a benign CPP in a pediatric patient.
- A review of literature identified prior cases of benign CPP metastasis in both pediatric and adult populations.
- Metastatic lesions exhibited benign or atypical pathology.
Implications:
- Patients diagnosed with even benign CPPs may require comprehensive initial and follow-up neuroimaging.
- Routine imaging of the entire neural axis is recommended to identify rare drop metastases.
- This approach aids in the early detection and management of potential CPP spread.
Abstract:
Choroid plexus papillomas (CPPs) are typically benign tumors that can occur in any age group but are more commonly found in pediatric patients. Although these tumors are benign, there are several reports in adult patients of distant metastases present either at the time of diagnosis or occurring months to years after initial resection. Here, the authors report the case of a 14-year-old boy who presented with symptoms of elevated intracranial pressure due to obstructive hydrocephalus that was caused by a large fourth ventricular mass. Preoperative imaging included a full MRI of the spine, which revealed an intradural lesion that encased the distal sacral nerve roots at the tip of the thecal sac and was concerning for a drop metastasis. The patient underwent gross-total resection of both the fourth ventricular and sacral tumors with histology of both lesions consistent with benign CPP (WHO Grade I). In addition, the authors review prior reports of both pediatric and adult patients in whom benign CPPs have metastasized with either benign or atypical pathology found at a distant site. Taking into account this unusual case and reports in the literature, patients with even benign CPPs may warrant initial and routine follow-up imaging of the total neural axis in search of the rare, but possible, occurrence of drop metastasis.
