Intraventricular neurocytoma: A diagnostic challenge with prognostic value.

ABSTRACT

Intraventricular neurocytoma is a low incidence central nervous system tumor. It predominantly affects young adults with no apparent gender predilection. The main symptoms include headache, nausea and vomiting. These result from hydrocephalus due to the obstruction of cerebrospinal fluid flow. On diagnostic imaging, neurocytoma can be suspected by some features, such as peripheral cysts, lobulated contours and septa that bridge the ventricular wall, giving a "scalloped" appearance. There are other characteristics, but they are less specific for the diagnosis. The atypical variant of neurocytoma is even rarer and leads to a worst prognosis. Atypical neurocytomas develop higher proliferative potential identified by the Ki-67 biomarker and higher recurrence rate. There are few studies about the imaging characteristics of atypical neurocytomas. At this point, there are no reliable distinctive features to differentiate atypical neurocytomas, especially due to their low incidence. We present the case of a 20-year-old female patient with symptoms of intracraneal hypertension. CT and MRI of the brain revealed a mass occupying the body of the left lateral ventricle, adjacent to the foramen of Monro. The mass was primarily solid with discrete peripheral cyst and a few scalloped areas. It also showed signs of supratentorial obstructive hydrocephalus. The tumor was partially removed because of bleeding and compromise of vascular structures. Immunohistochemistry revealed positive synaptophysin, elevated Ki-67 (7%), increased number of blood vessels and moderate nuclear atypia. After surgery, the patient persisted with signs of intracranial hypertension, not improving with clinical management and requiring aggressive surgical procedures. While rare, atypical neurocytoma requires a better characterization, especially through imaging, to optimize immediate management and explore new therapeutic options.