Porta Hepatis Rhabdomyosarcoma
Joseph Junewick, MD FACR
over 5 years ago
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Choroid Plexus Carcinoma - 4th Ventricle
Joseph Junewick, MD FACR
|Diagnostic Category: Neoplasia Malignant
|Created: over 6 years ago
|Updated: over 6 years ago
5 year old male with a 2 month history of daily morning emesis.
Choroid Plexus Carcinoma
MR – Axial T1, T2, T2-FLAIR, sagittal axial and coronal post-gadolinium T1 and diffusion weighted imaging of the brain show a T2 and post-gadolinium hyperintense and cellular (DWI)lesion within the 4th ventricle.
Choroid plexus carcinoma is an uncommon, aggressive, malignant, central nervous system neoplasm that typically occurs in children and presents with the signs and symptoms of cerebrospinal fluid obstruction. Within the pediatric population, the median age is approximately 2-3 years of age. Choroid plexus carcinomas are believed to arise from the choroid plexus epithelium. In general, they occur as follows: 50% lateral ventricles, 40% fourth ventricle, 5% third ventricle, and 5% multiple ventricular involvement. Although infrequent, choroid plexus carcinomas can occur in the cerebellopontine angle near the foramina of Lushka, and extremely rare cases may present as a suprasellar cistern or as an intraparenchymal mass. Choroid plexus carcinoma has been described in association with Li-Fraumeni syndrome likely related to loss of function of the tumor suppressor protein p53.
Choroid plexus carcinoma has been associated with obstruction of the cerebrospinal fluid pathways and, potentially, overproduction of cerebrospinal fluid, leading to hydrocephalus and increased intracranial pressure. An infant may present with hydrocephalus manifesting with increased head circumference, and signs and symptoms of increased intracranial pressure, including delayed development, bulging fontanelles, separated sutures, strabismus, or vomiting. An older child or adult can present with features of increased intracranial pressure, including vomiting, headache, neurologic deficits, seizures, lethargy, visual, and/or behavioral changes.
Choroid plexus carcinomas usually grow rapidly. 5-year survival rate is approximately 40%. Two thirds of these tumors disseminate throughout the cerebrospinal fluid pathway. There is currently no established protocol for the treatment of choroid plexus carcinomas however, complete resection of the tumor is the primary goal with gross total resection providing the best chance of survival.
Gopal P, Parker JR, Debski R, Parker JC. Choroid Plexus Carcinoma. Archives of Pathology & Laboratory Medicine (2008); 132(8):1350-1354.