What is Neurofibromatosis Type 2?

Neurofibromatosis Type 2
Neurofibromatosis Type 2. Micro-hamartomas in the brain of a 20-year-old man with NF-2. Low (a) and high (b) quality magnification of an area of the left thalamic deep gray matter in this patient with radiographic evidence of cortex dysplasia reveal dysplastic foci of glial cells without alterations of cytologic abnormalities or cortical architecture. Transverse fast fluid-attenuated inversion-recovery (FLAIR) sequences (c and d) demonstrate a cortical hyperintense lesion (arrows) suggestive of a cortical dysplasia in the left superior frontal gyrus in the same patient.Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2. Vargas WS, Heier LA, Rodriguez F, Bergner A, Yohay K - NeuroImage. Clinical (2014). Not Altered. CC.

Neurofibromatosis type 2 is a condition of the nerves where slow-growing tumors develop in both ears leading to bilateral hearing loss due to the presence of schwannomas on the 8th cranial nerve.

What is the Pathology of Neurofibromatosis Type 2?

The pathology of neurofibromatosis type 2 is:

-Etiology: The cause of neurofibromatosis type 2 is a genetic mutation.

-Genes involved: NF-2 gene.

-Pathogenesis: The sequence of events that lead to neurofibromatosis type 2 not clearly understood

-Morphology: The morphology associated with neurofibromatosis type 2 shows well-circumscribed tumors.

-Histology: The histology associated with neurofibromatosis type 2 shows Antoni A and Antoni B structures.

How does Neurofibromatosis Type 2 Present? 

Patients with neurofibromatosis type 2 typically are female and males present at the age range of 18-24 years. The symptoms, features, and clinical findings associated with neurofibromatosis type 2 include gradual hearing loss, poor balance, headaches, and tinnitus.

How is Neurofibromatosis Type 2 Diagnosed? 

Neurofibromatosis type 2 is diagnosed history taking and physical examination. MRI, CT scan, biopsy, hearing tests, vision tests, and genetic testing.

How is Neurofibromatosis Type 2 Treated? 

Neurofibromatosis type 2 is treated depending on the size and the location. It can be surgery, radiation, auditory brainstem implant, and chemotherapy. 

What is the Prognosis of Neurofibromatosis Type 2?

The prognosis of neurofibromatosis type 2 is poor due to its high mortality rate at a young age.