Choroid plexus
carcinomas (
CPC) are rare malignant
intracranial neoplasms usually occurring in young children. The objectives of this study were to characterize the preoperative MRI features of
CPC, determine the frequency of disseminated disease in the CNS at diagnosis, and assess patient outcomes. The preoperative cranial MR images of 11 patients with
CPC were retrospectively reviewed for lesion location, lesion size, un-enhanced and enhanced MRI signal characteristics, and presence of disseminated intracranial
tumor. Postoperative cranial and spinal MRI images were reviewed for residual, recurrent, and/or disseminated
tumor. The study group included six male and five female patients ranging in age from 5 months to 5.3 years (median= 1.8 years).
CPC were located in the lateral (n = 8), fourth (n = 1), and third (n = 1) ventricles, and foramen of Luschka (n = 1). Mean
tumor size was 5.2 cm x 4.9 cm x 5.0 cm. On short-TR images,
CPC had heterogeneous, predominantly intermediate signal with foci of high signal in 45% of lesions from areas of
hemorrhage. On long-TR/long-TE images, solid portions of
CPC typically had heterogeneous, intermediate-to-slightly-high signal. Small zones of low signal on long-TR/long-TE images were seen in 55% of the lesions secondary to areas of
hemorrhage and/or calcifications. Tubular flow voids representing blood vessels were seen in 55% of the lesions. Zones of high signal comparable to CSF were seen in 64% of
CPC secondary to cystic/necrotic zones. All
CPC showed prominent contrast enhancement. Irregular enhancing margins suggesting subependymal invasion were seen in 73% of the lesions. Findings consistent with
edema in the brain adjacent to the enhancing lesions were seen in 73% of
CPC.
CPC caused
hydrocephalus in 82% of patients at diagnosis. Two patients died from hemorrhagic complications from surgical biopsies. Disseminated
tumor in the leptomeninges was present in 45% of patients at diagnosis and was associated with a poor prognosis. The 1-year and 5-year survival probabilities were 55% and 45%, respectively. In conclusion, MRI features commonly associated with
CPC include large intraventricular lesions with irregular enhancing margins; heterogeneous signal on long TR/long TE images and short-TR images;
edema in adjacent brain;
hydrocephalus; and presence of disseminated
tumor. MRI evidence of disseminated
tumor at diagnosis is associated with a poor prognosis.