Extra-axial medulloblastoma in the cerebellopontine angle: Report of a rare entity with review of literature

Pant, Ishita; Chaturvedi, Sujata; Gautam, Vinay; Pandey, Pranjal; Kumari, Rima

doi:10.4103/1817-1745.199477

Cited by 12 publications

(19 citation statements)

References 8 publications

(9 reference statements)

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“…However, some characteristics of posterior fossa tumors such as progressive cerebellar signs and gait ataxia may be seen, although usually favoring an intra-axial location. Headache, nausea, and vomiting are less distinguishable symptoms commonly reported in medulloblastoma cases [17] . Additional differential diagnoses, such as posterior fossa epidermoid cyst, hemangioblastoma, pilocytic astrocytoma, and atypical tertoid-rhabdoid tumor, may also be included.…”

Section: Discussionmentioning

confidence: 91%

“…reported a study of 129 histologically verified tumors of the posterior fossa and determined that medulloblastoma frequently exhibits increased density compared with adjacent cerebellum and can be diagnosed with high likelihood when there is a central mass exhibiting well-demarcated borders [14] . Only 35 cases of medulloblastoma have been reported to be extra-axial in nature, most of which occur in the cerebellopontine angle [15] , [16] , [17] . The extra-axial finding alongside a purely hemispheric presentation of the tumor makes our patient's case quite rare in the pediatric population.…”

Section: Discussionmentioning

confidence: 99%

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Posterior fossa medulloblastoma in an atypical extra-axial location: A case report

Presutto

Chappell

Fullmer

et al. 2018

Radiology Case Reports

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Medulloblastoma is the most common posterior fossa tumor of childhood typically within the fourth ventricle. However, extra-axial medulloblastoma in posterior fossa is an uncommon diagnosis. We report a case in a 33-month-old male who presented with repeated complaints of abdominal pain, intermittent emesis, and diarrhea, and diagnosed with right cerebellar extra-axial medulloblastoma, which was surgically resected. Majority of the reported extra-axial medulloblastoma in posterior fossa in the United States are located in the cerebellopontine angle. However, to the best of our knowledge, our case is the first to document medulloblastoma occurring exclusively in the cerebellar hemispheric extra-axial space rather than the cerebellopontine angle. Although the diagnosis can present as a radiological dilemma, a systematic multimodality imaging approach can aid in narrowing the differential diagnosis and timely management. In this case report, we will discuss the imaging characteristics, differential diagnosis, and management strategies, alongside a brief review of the world literature of extra-axial medulloblastoma.

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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Posterior fossa medulloblastoma in an atypical extra-axial location: A case report

Presutto

Chappell

Fullmer

et al. 2018

Radiology Case Reports

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“…It has been suggested that extra-axial MBs located at CPA may originate either by the proliferating remnants of the external granular layers of the cerebellar hemispheres including the flocculus or from germinal cells or their remnants present at the posterior medullary velum. [ 8 ] Akay et al . suggested that extra-axial MBs at CPA arise by the lateral extension from the fourth ventricle through the foramen of Luschka or there may be direct exophytic growth from the site of origin at the surface of cerebellum or pons.…”

Section: Discussionmentioning

confidence: 99%

“…suggested that extra-axial MBs at CPA arise by the lateral extension from the fourth ventricle through the foramen of Luschka or there may be direct exophytic growth from the site of origin at the surface of cerebellum or pons. [ 8 9 ]…”

Section: Discussionmentioning

confidence: 99%

Extraaxial cerebellopontine angle medulloblastoma with multiple intracranial metastases in adult: A rare case report with a review of literature

Singh¹,

Kumar

2020

Asian J Neurosurg

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Medulloblastoma (MB) is very rare malignant primary brain tumor in adults and its location at cerebellopontine angle (CPA) is even rarer. There are only few case reports of CPA MBs in literature. Most of them are reported in pediatric age groups which are mostly intraaxial. Only 11 cases of extra-axial CPA MBs have been reported so far. The author is reporting a very rare case of adult extra-axial CPA MBs with multiple intracranial metastases. We are also reviewing clinical and radiological features and unusual way of its presentation along with surgical management. Keeping MBs as one of the differential diagnosis for extra-axial CPA tumors is going to increase our spectrum of diagnosis which can lead to alteration in management.

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“…Disease dissemination is an early event in MB, with up to ∼40% of patients showing metastases at diagnosis, with poor survival. Metastatic disease and tumor recurrence are responsible for the low survival rates, and those who survive frequently show treatment-related adverse effects (5,20,35,36,38,40,(50)(51)(52)(53).…”

Section: Discussionmentioning

confidence: 99%

Methylation Profiling of Medulloblastoma in a Clinical Setting Permits Sub-classification and Reveals New Outcome Predictions

et al. 2020

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Medulloblastoma (MB) is the most common childhood malignant brain tumor and is a leading cause of cancer-related death in children. DNA methylation profiling has rapidly advanced our understanding of MB pathogenesis at the molecular level, but assessments in Saudi Arabian (SA)-MB cases are sparse. MBs can be sub-grouped according to methylation patterns from FPPE samples into Wingless (WNT-MB), Sonic Hedgehog (SHH-MB), Group 3 (G3), and Group 4 (G4) tumors. The WNT-MB and SHH-MB subgroups are characterized by gain-of function mutations that activate oncogenic cell signaling, whilst G3/G4 tumors show recurrent chromosomal alterations. Given that each subgroup has distinct clinical outcomes, the ability to subgroup SA-FPPE samples holds significant prognostic and therapeutic value. Here, we performed the first assessment of MB-DNA methylation patterns in an SA cohort using archival biopsy material (FPPE n = 49). Of the 41 materials available for methylation assessments, 39 could be classified into the major DNA methylation subgroups (SHH, WNT, G3, and G4). Furthermore, methylation analysis was able to reclassify tumors that could not be sub-grouped through next-generation sequencing, highlighting its superior accuracy for MB molecular classifications. Independent assessments demonstrated known clinical relationships of the subgroups, exemplified by the high survival rates observed for WNT tumors. Surprisingly, the G4 subgroup did not conform to previously identified phenotypes, with a high prevalence in females, high metastatic rates, and a large number of tumor-associated deaths. Taking our results together, we demonstrate that DNA methylation profiling enables the robust sub-classification of four disease sub-groups in archival FFPE biopsy material from SA-MB patients. Moreover, we Alharbi et al. Clincal Methylation Profiling of Medulloblastomashow that the incorporation of DNA methylation biomarkers can significantly improve current disease-risk stratification schemes, particularly concerning the identification of aggressive G4 tumors. These findings have important implications for future clinical disease management in MB cases across the Arab world.

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Extra-axial medulloblastoma in the cerebellopontine angle: Report of a rare entity with review of literature

Cited by 12 publications

References 8 publications

Posterior fossa medulloblastoma in an atypical extra-axial location: A case report

Posterior fossa medulloblastoma in an atypical extra-axial location: A case report

Extraaxial cerebellopontine angle medulloblastoma with multiple intracranial metastases in adult: A rare case report with a review of literature

Methylation Profiling of Medulloblastoma in a Clinical Setting Permits Sub-classification and Reveals New Outcome Predictions

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