Moutasem Alzoubi

Pediatric Neurosurgeon

King Abdullah Specialized Children’s

Hospital, NGHA

Riyadh, Saudi Arabia

Cerebellopontine angle (CPA) lesions are more commonly found in adults in which they account for 5–10% of all intracranial tumors.

with less than 1% incidence in children of all intracranial tumors

Neuroglial tissues

Cranial nerves

Meninges

Blood vessels

Bone

Cartilage

and embryonic remnants.

Schwannomas of vestibular nerves

Meningiomas

Epidermoid

Vestibular schwannoma account for 70–80 % of all CPA lesions

Meningiomas 10-15 %

Epidermoid 5% in adults.

In children, tumors in the CPA are very uncommon, Vestibular schwannoma and meningioma are also rare in childhood, except for patients with neurofibromatosis type 2 (NF2)

The majority of posterior fossa tumors in children develop in the midline, including:

The fourth ventricle

Vermis

Brain stem

Cerebellar hemispheres.

More than half of pediatric intrinsic brain

tumors develop in the posterior fossa.

Medulloblastomas and ependymomas arising in the fourth ventricle which sometimes protrude into the CPA, mimicking extra-axial tumors.

the cerebellopontine angle (CPA) is a rare site

and less than 10 % of posterior fossa tumors

in childhood were located in CPA,

tumors are often secondary to an exophytic extension of intrinsic tumors originating from the adjacent brain stem, cerebellar peduncle, and cerebellum or a direct extension from the fourth ventricle and lateral recess.

In children, CPA tumors are uncommon and have a distinct pathological spectrum compared with the adult population.

only a limited number of publications are available in the literature during past decade.

According to Holman et al., schwannomas are the most common pediatric tumor type: 57 of 87 CPA tumor.

Zuccaro and Sosa et al. indicating benign tumors are more frequent. Of their 27children with CPA tumors (excluding arachnoid cyst), schwannoma and meningioma are the most common (8 and 6, respectively), 21 (78 %) were benign histologically.

While Phi et al., of 26 children with CPA tumors, 18 (69 %) were malignant and 8 were benign. and pediatric neurosurgeons should consider a high probability of malignant histology ( 69%) for pediatric CPA tumors, especially in young children.

According to Rorke and Biegel, 52 % of ATRTs occur in the posterior fossa and they stated that the tendency to arise in the CPA with invasion of the surrounding structures is a distinct feature of this tumor.

In Tomita and Grahovac series, there were 22 malignant tumors (14 ependymomas, 4 ATRTs, 3 PNETs, and 1 glioblastoma) and 22 benign tumors (11 pilocytic astrocytomas, 4 epidermoids, 3 meningiomas, 3 nerve sheath tumors, and 1 ganglioglioma), thus malignant vs. benign tumor ratio being 1:1

50 % are malignant including ependymoma, ATRT, PNET, and glioblastoma, whereas another 50 % were benign consisting of JPA, epidermoid, schwannoma, and meningioma.

2 years old girl presented with 3 weeks history of torticollis, unsteady gait, and headache

18 month old boy presented with H/O repeated vomiting and irritability for 3 weeks

3 year old boy presented with change in level of consciousness, on/off vomiting

12 month old boy presented with seizure, left side weakness

10 year old boy presented with headache , vomiting and attaxicgait