Acoustic neuroma classification

Overview

Acoustic neuroma may be classified according to the findings on magnetic resonance

imaging (MRI) or it can also be classified based on microscopic histopathology, and
whether or not they are associated with neurofibromatosis type 2.

Based on microscopic histopathology, acoustic neuroma may be classified into four

subtypes:
1)Conventional schwannoma,
2)Cellular schwannoma,
3)Plexiform schwannoma
4)Melanotic schwannoma.

While acoustic neuromas are benign tumors, there is no established system for the staging
of acoustic neuromas. Koos grading scale provides four grades based on extrameatal
extension and compression of the brain stem , a reliable method for tumor classification
which is used in practice.

Classification based on

Association with Neurofibromatosis type 2

-Not associated/Sporadic
- 95% of all the cases of acoustic neuroma are sporadic. The cause of sporadic form is
unclear
-NF2 is a rare disorder and it accounts for 5% of acoustic neuromas
-Acoustic neuroma associated with neurofibromatosis type II are typically bilateral and
cause gradually progressive hearing loss, tinnitus, and balance dysfunction

MRI scan
-Entirely intracanalicular: The entire tumor is completely within the bony canal
-Intracranial extension without brain stem distortion: Intracranial portion of the tumor is
1.5 - 2.5 cm. (Some references mentioned 1 - 2 cm)
-Intracranial extension with brain stem distortion: Intracranial portion of the tumor is
greater than 2.5 cm. (Some references mentioned more than 2 cm)

Microscopic Histopathology
-Conventional schwannoma: It is the most common schwannoma
-Cellular schwannoma: It may mimic malignant peripheral nerve sheath tumor
-Plexiform schwannoma: It may mimic malignant peripheral nerve sheath tumor if
cellular, especially in childhood
-Melanotic schwannoma
Staging
Acoustic neuromas are benign tumors (WHO grade 1), but there is no established system
for the staging of acoustic neuromas. Numerous stage grading systems have been reported
according to tumor size. Tumor size is more important and can be measured by measuring
the maximum diameter of the tumor.

According to the Koos grading scale, there are 4 grades of acoustic neuroma based on the
findings on magnetic resonance imaging (MRI), extrameatal extension and compression of
the brain stem.