Department of Pathology, University of Oklahoma Health Sciences Center

NeuroTest Question #13 Next question  Previous question

Answer: B (Associated with neurofibromatosis 2 and enhances on MRI). Level of difficulty: 3 NeuroTest NeuroLearn NeuroHelp

Discussion: 

Pathology: The tumor being illustrated here is a meningothelial meningioma. [Click here to see Gross pathology] [Click here to see histopathology] [Click here to see a related case]

Neurofibromatosis 2 (NF2): 

• NF2, also known as central neurofibromatosis, is transmitted in an autosomal dominant pattern. The gene involved is located on chromosome gene 22q12 and encode for the protein merlin. 
• The incidence of NF2 is only about one-tenth that of NF1. 
• The cardinal features of neurofibromatosis are multifocal focal hyperplasia and neoplasia in the supportive tissue throughout the entire nervous system. These include the nerve sheath elements of the cranial, spinal and peripheral nerve as well as glial and meningeal elements of the central nervous system. Schwannomas and meningiomas are most common, followed by ependymoma. Hamartomatous lesions in the central nervous system are also very frequent.
• Neoplastic transformation of ganglionic elements resulting in pheochromocytomas, peripheral neuroblastomas and ganglioneuromas may also occur.

Comment: KarMing-Fung@ouhsc.edu