Extra axial medulloblastoma in the cerebellar hemisphere Hasan H.

Al-Muhammed

Basrah Journal Case report

Of Surgery Bas J Surg, June, 22, 2016

EXTRA-AXIAL MEDULLOBLASTOMA IN THE

CEREBELLAR HEMISPHERE; A REPORT OF A RARE
CASE WITH LITERATURE REVIEW
Hasan Hadi Al-Muhammed
MB,ChB, FICMS, Department of Neurosurgery, Al-Sadir Teaching Hospital, Basrah, IRAQ.

Abstract
Extra-axial medulloblastoma is quite rare tumor. This report concern a 13 year old girl who
presented with headache, nausea and vomiting of two months duration. Radiological findings
demonstrate heterogeneous mass involving the left cerebellar hemisphere and compressing the
vermis and the 4th ventricle causing acute hydrocephalus. Patient was treated with venriculo-
peritoneal shunt for hydrocephalus followed by posterior fossa craniectomy and gross total
tumor excision. The diagnosis of medulloblastoma is confirmed by histopathology.
This is the first reported case of total extra-axially located medulloblastoma in Iraq. Although
extremely rare, medulloblastoma should be considered in the differential diagnosis of extra-axial
cerebellar hemisphere lesions in children.

Introduction

M edulloblastoma is a common tumor of the posterior fossa in children, representing

20-25% of all pediatric primary malignant central nervous system neoplasms1-3.
The tumor often occur in the cerebellar vermis and at the apex of the 4th ventricle1,4.
The extra-axial site of this tumor remain a rarity1,2,4,5. Previously, 33 cases of
medulloblastoma located extra-axially have been reported worldwide4,6 but most of
these medulloblastomas located at the cerebello-pontine angle (CPA). This is the 1st
reported case in Iraq in which a medulloblastoma was entirely located in the cerebellar
hemisphere.
Case report
A 13 year old girl presented with nausea and vomiting for 2 months. The initial
neurological examination revealed ataxia, dysmetria and advanced bilateral
papilledema. Brain CTS showed a well-defined, oval mass in the left posterior fossa,
compressing the 4th ventricle causing acute hydrocephalus (Figure 1). An emergency
operation was performed, ventriculo-peritoneal shunt, to control the increased
intracranial pressure secondary to hydrocephalus. Patient showed improvement after
surgery.

Figure 1

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Few days later, a brain MRI was performed which revealed presence of an oval,
complex, predominantly solid mass with small cystic foci, enhanced heterogeneously
following contrast injection, about 4x3.3x2.5 cm in size involving the left cerebellar
hemisphere and compressing the vermis and the 4th ventricle with pressure effect on the
left side of the pons and the left middle and superior cerebellar peduncles. Ventriculo-
peritoneal shunt seen in the right lateral ventricle and well-functioning (Figure 2).

Figure 2

A differential diagnosis of glioma or hemangioblastoma was made, and a continue for

the next session of tumor excision 10 days after the 1st operation of CSF shunting.
A left lateral sub-occipital infra-tentoreal craniectomy was performed for tumor
resection. Macroscopically the tumor was well demarcated, semisolid with areas of old
hemorrhages, extra-axially located, attached to the adjacent dura of the posterior cranial
fossa with invasion of the adjacent cerebellar tissues. Gross total excision was
performed.
The post-operative course was passed smoothly without complications and patients
symptoms improved gradually.
Histopathological examination reveals diffuse growth pattern of oval cells with
hyperchromatic nuclei, large bizarre cells and few rosettes, a picture highly suggesting
of medulloblastoma. The slides were revised with other histopathologist who do not
know about the first diagnosis, and the result was the same, a classical type of
medulloblastoma (Figure 3).

Figure 3

Post-operative contrast-enhanced brain CTS was performed and revealed no evidence

of tumor remnant in left posterior fossa (Figure 4). MRI of the spinal cord showed no
definite evidence of CSF tumor seedling.

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Extra axial medulloblastoma in the cerebellar hemisphere Hasan H. Al-Muhammed

Figure 4

Discussion
Brain tumors are the second most common malignancy affecting the pediatric
population and account for more childhood death than any solid tumors7.
Medulloblastoma account for 25% of all brain tumors in children and is the most
common malignant pediatric tumor of the posterior fossa8.
Medulloblastoma belong to the family of primitive neuro-ectodermal tumors9. The cell
of origin is still controversial, it may originate either from germinal cells or their
remnant situated at the end of the posterior medullary velum or from the remnant of the
external granular layer1,4,5,10. kadin et al suggested that medulloblastoma arise from
germinal cells (or their remnant) anywhere along their migratory pathway, and because
the migratory process normally proceed s in a lateral direction, a relatively higher
frequency of laterally situated tumor should be observed in adults4,11.
Medulloblastoma have long been classified into tow chief histological variants,classical
and desmoplastic. Recent update to the world health organization classification of CNS
tumors have modified the classification of medulloblastoma. The revised classification
considered all medulloblastomas to be WHO grade IV tumors. The five subtypes of
major histological variants recognized include the classical, desmoplastic/nodular,
extensive nodularity medulloblastoma, anaplastic and large cell subtype12.
The relationship between the histological type of medulloblastoma and their location is
still uncertain and should be further studied4, but many studies suggested that
desmoplastic type have been reported to occur more frequently among extra axial
tumors4,13. In this case a classical type was found which is quite unusual regarding the
site of the tumor, but it goes with the age of the patient since the classical type is more
common in children while desmoplastic type seen more commonly in adult and most of
the previously reported extra axial tumors seen in adults.
The clinical presentation of children with medulloblastoma is variable but most patients
presented with short history of progressive symptoms which are presented for less than
3 months in 75% of cases. Associated hydrocephalus is responsible for a significant
proportion of clinical presentation14, as in this case.
Controversy still exists regarding the initial management of patient with
medulloblastoma and hydrocephalus. Some advocate routine shunting of the
hydrocephalus (ventriculo-peritoneal shunt or endoscopic third ventriculostomy) as the
first step, claiming that there is a lower mortality and morbidity and a better surgical
field after the intracranial pressure is relieved with CSF shunting for a period of several
days prior to tumor excision15. Still others advocate one session surgery with external
drainage of CSF plus direct tumor excision16.
The policy at our center is to consider CSF shunting few days prior to craniectomy and
tumor excision. Lin et al17 found that 10-40% of cases demonstrate persistent
hydrocephalus after posterior fossa tumor resection alone. Due et al18 found that the low

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cure rate of hydrocephalus by tumor resection alone in patients with medulloblastoma

and ependymoma, raises the issue of whether these patients would benefit from
preoperative CSF shunting.
The surgical plane is always to try to perform a gross total resection of the tumor
because there is evidence in the literatures that this result will enhance the likelihood of
long term survival of the patients19.
Chung et al4 revised the prognosis of the extra axially located medulloblastomas in the
literatures including 33 cases reported in the cerebellopontine angle, among which 4
cases seen in the pediatric age group described by Kumar et al20 and they concluded that
there is currently no clear cut consensus as to whether extra axially located
medulloblastomas are more aggressive compared to their midline counterparts4.

Conclusion
Medulloblastoma is the most common malignant pediatric tumor in the posterior fossa,
and usually occur in the midline. Extra-axial occurrence of medulloblastoma is
extremely rare, and clinically it is difficult to be differentiated from other extra axial
posterior fossa neoplasms. This is the1st reported case of extra-axial medulloblastoma
in Iraq but still these lesions are likely to be under reported owing to the lack of
systematic case reports publication by neurosurgeons in Iraq and wing to publication
biases. Although rare, medulloblastoma should be considered in the differential
diagnosis of extra-axial posterior fossa lesions.

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