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Infantile Choroid Plexus Papilloma with Multiple Peritumoral Cysts
Shoichi Horinouchi1, Takashi Watanabe1, Hideo Takeshima1
1Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.
Insights
Infantile choroid plexus papilloma (CPP) with peritumoral cysts is rare. Surgical resection of the solid tumor component effectively treated cysts and hydrocephalus, avoiding permanent CSF diversion.
Area of Science:
- Pediatric Neurosurgery
- Pediatric Oncology
- Neuropathology
Background:
- Infantile choroid plexus papilloma (CPP) is a rare brain tumor.
- A variant of CPP associated with multiple peritumoral cysts presents unique clinical challenges.
- Optimal management strategies for this rare variant remain largely undefined.
Purpose of the Study:
- To describe the clinical presentation, management, and outcomes of a rare variant of infantile choroid plexus papilloma associated with peritumoral cysts.
- To evaluate the efficacy of surgical resection of the solid tumor component in managing associated cysts and hydrocephalus.
Main Methods:
- Case presentation of a 9-month-old boy with a left lateral ventricle tumor.
- Detailed description of tumor characteristics, associated cysts, and hydrocephalus.
- Surgical resection of the solid tumor component and follow-up imaging.
Main Results:
- Total resection of the hypervascular solid tumor component led to shrinkage of peritumoral cysts and resolution of hydrocephalus.
- Histological examination confirmed CPP in the solid component and reactive gliosis in the cyst walls, without neoplastic cells.
- 12-month follow-up MRI showed stable cyst size, indicating long-term resolution.
Conclusions:
- Resection of only the solid part of the tumor is a viable management strategy for infantile CPP with nonenhancing peritumoral cysts.
- This approach can successfully resolve associated hydrocephalus and cysts without the need for permanent cerebrospinal fluid diversion.
- This case highlights a potentially less invasive management option for this rare pediatric brain tumor variant.
Abstract:
Infantile choroid plexus papilloma (CPP) associated with multiple peritumoral cysts is a rare variant of CPP, and clinical course and optimal management are largely unknown. A 9-month-old boy presented with a large solid tumor in the left lateral ventricle associated with multiple peritumoral cysts, arachnoid cysts, and hydrocephalus containing xanthochromic fluid with high protein content. Shrinkage of these cysts and resolution of hydrocephalus were achieved after total resection of the hypervascular solid part of the tumor. Histological examination confirmed the solid part of the tumor as CPP and showed that the wall of the peritumoral cysts consisted of reactive gliosis without neoplastic cells. Follow-up magnetic resonance imaging 12 months after surgery revealed that these cysts remained stable. CPP with nonenhancing peritumoral cysts can be managed by resection of only the solid part of the tumor without permanent cerebrospinal fluid diversion.
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