Rapidly growing, multifocal, benign choroid plexus tumor in an infant: case report

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Choroid plexus papillomas (CPPs) are rare, benign tumors that can arise in young children. Most pediatric patients present with signs of hydrocephalus and require immediate treatment. The natural history of choroid plexus tumors in children without hydrocephalus is poorly defined. In this report, the authors present the very rare case of a child without hydrocephalus but with two intraventricular choroid plexus tumors discovered shortly after birth. Initial imaging had been performed for seizures and showed agenesis of the corpus callosum and enhancing tumors in the third and left lateral ventricles. Sequential imaging demonstrated rapid growth of both tumors. The lateral tumor was removed when the child was 3 months of age. A histological examination of the specimen showed benign features with an elevated mitotic rate. Given the patient’s age of under 3 years, the diagnosis was WHO grade I CPP. The third ventricle tumor grew rapidly. A second surgery was performed and this tumor was resected. Again, the pathological diagnosis was WHO grade I CPP. The authors present this rare case and discuss the current relevant literature.

ABBREVIATIONS aCPP = atypical CPP; CPC = choroid plexus carcinoma; CPP = choroid plexus papilloma; CSF = cerebrospinal fluid.

Article Information

Correspondence Daniel H. Fulkerson: Beacon Children’s Hospital, South Bend, IN. dhfulkerson@beaconhealthsystem.org.

INCLUDE WHEN CITING Published online February 22, 2019; DOI: 10.3171/2018.12.PEDS18453.

K.A.M. and Z.G.O. contributed equally to this paper.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.



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    Axial T1-weighted MR image (A) with gadolinium contrast showed multiple enhancing lesions in a 5-day-old infant. A T2-weighted image (B) showed the lesions within the left lateral and third ventricles (arrows). MRI also demonstrated agenesis of the corpus callosum.

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    Axial T1-weighted MRI with gadolinium contrast performed at 2 months of age, showing interval growth in both tumors (A). The left lateral ventricular was removed 1 month later. Postoperative MRI showed gross-total resection of the left tumor, with interval growth of the third ventricular tumor (B).

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    H & E sections revealed a papillary neoplasm with arborizing architecture (A). The well-differentiated epithelial tumor shows a single layer of uniform columnar cells and scattered mitoses (B). The Ki-67 proliferation index reached 20% (C). Up to 5 mitoses were identified in a single high power field on PHH3 immunostaining (D). Original magnification × 4 (A), × 10 (B and C), × 20 (D). Figure is available in color online only.

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    T1-weighted MRI with gadolinium contrast performed when the child was 4.5 months of age, showing growth of the third ventricular tumor in the axial (A) and coronal (B) planes, measuring 44 × 46 mm. There was also a new nodule in the atrium of the right lateral ventricle, measuring 7 × 15 mm. Postoperative axial T1-weighted MRI with contrast demonstrated gross-total resection of the tumor (C). An MR image obtained 8 months after surgery showed no evidence of recurrence of the third or left lateral ventricular tumors (D).





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