Metastatic pigmented neuroectodermal tumor with cranial CT-pathology correlation.

Abstract

Pigmented neuroectodermal tumors are a relatively rare clinicopathologic entity, presenting in a variety of locations. Their histogenesis suggests a transformation from common pleuripotential neural crest cells with varying degrees of differentiation to neural elements and melanocytes. We present a case of just such a tumor, which presented initially in the mandible of a leukemic patient who received irradiation to the skull base, as well as systemic chemotherapy, for suspected cranial leukemic involvement. Subsequent development of intracranial spread was documented both by imaging and pathology. Extracranial spread was also found at autopsy. This unusual presentation and widespread dissemination of the neuroectodermal tumor in our case confirms its aggressiveness and potential for multifocality.