Primitive neuroectodermal tumour metastases mimicking neurofibromatosis type 2

Bilateral vestibular schwannomas are the diagnostic features of neurofibromatosis type 2 (NF-2), and are the most common findings associated with the disorder. We report a three-year-old boy who presented with left facial nerve palsy and weight loss with bilateral large cerebellopontine (CP) angle masses that extended into the internal auditory canal on magnetic resonance imaging. The patient also had synchronous tumours in the lateral ventricle and intradural extramedullary spinal canal. The above findings were misinterpreted as NF-2 with bilateral vestibular schwannomas, ventricular meningioma and spinal schwannomas/meningiomas. However, histological examination of the spinal masses revealed a primitive neuroectodermal tumour. Although bilateral CP angle masses are characteristic of NF-2, the possibility of diffuse craniospinal malignancy should be considered in a very young child who presents with weight loss and extensive tumours.