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Malignant choroid plexus papilloma of the IV ventricle
Abstract:
We report a case of malignant choroid plexus papilloma (MCPP) of the IV ventricle in a child with prominent extraventricular expansive growth, mostly into the cerebellopontine angle. Interestingly enough, the tumor was entirely covered by thin, smooth, membranous, fibrous tissue, probably derived from the pia mater. In addition, hydrocephalus was not observed. The reason for the lack of hydrocephalus remains speculative, but it is possible that the presence of pure, nonexpansive, fibrous covering on the tumor might have suppressed the tumor growth to some extent, resulting in progressively increased intratumoral pressure. This, in turn, caused the suppression of excessive cerebrospinal fluid production by the tumor cells. From the diagnostic standpoint, immunohistochemical studies, using antiepithelial membrane antigen (EMA) and antitissue polypeptide antigen (TPA), were demonstrated to be useful for diagnosing the tumor.
Insights
A rare malignant choroid plexus papilloma in a child presented with unusual extraventricular growth and no hydrocephalus. Immunohistochemical markers like EMA and TPA aided in diagnosis.
Area of Science:
- Pediatric neuro-oncology
- Cerebrospinal fluid dynamics
- Surgical neuropathology
Background:
- Malignant choroid plexus papilloma (MCPP) is a rare pediatric brain tumor.
- Typical MCPP can cause obstructive hydrocephalus due to its location in the ventricles.
Observation:
- A pediatric case of MCPP in the IV ventricle exhibited significant extraventricular growth into the cerebellopontine angle.
- The tumor was uniquely encapsulated by a thin, fibrous membrane, likely of pial origin.
- Notably, hydrocephalus was absent despite the tumor's expansive growth.
Findings:
- The fibrous encapsulation may have suppressed tumor expansion and cerebrospinal fluid (CSF) production, preventing hydrocephalus.
- Immunohistochemical analysis using epithelial membrane antigen (EMA) and tissue polypeptide antigen (TPA) proved valuable for diagnosis.
- Intratumoral pressure might have increased due to the nonexpansive capsule, potentially inhibiting CSF hypersecretion.
Implications:
- This case highlights an atypical presentation of MCPP and its potential impact on CSF dynamics.
- Understanding the role of tumor encapsulation is crucial for predicting clinical presentation and outcomes.
- EMA and TPA are confirmed as useful diagnostic markers for MCPP, aiding in accurate neuropathological assessment.