Posterior cerebral fossa medulloepithelioma: report of a case

Nezha Oumghar,Mouna Khouchani,Hanane Rais,Abdelhamid El Omrani,Fatima Ezzahra Hazmiri

doi:10.1186/s12907-017-0064-x

Nezha Oumghar, Mouna Khouchani + Show 3 more

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https://doi.org/10.1186/s12907-017-0064-x

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Abstract

BackgroundMedulloepithelioma is a rare primitive neuroectodermal tumor of the central nervous system, usually developing in childhood. Due to its rarity, the optimal management is still unknown. The prognosis is poor, especially when resection is incomplete. Adjuvant radiochemotherapy is often indicated.Case presentationWe report a rare case of infratentorial medulloepithelioma in a 3 year old girl. She presented symptoms of increased intracranial pressure. On examination, she had coordination problems, ptosis and exotropia of the right eye. Magnetic resonance imaging demonstrated a large cerebellar vermix tumor. Immuno-histochemistry revealed a diffuse positivity for Vimentin and focal positivity for the epithelial membrane antigen, but Glial Fibrillary Acidic Protein and Synaptophysin were negative, the MIB-1 antibody was very high. She received postoperative craniospinal irradiation and died 7 months later.ConclusionWe describe the features (epidemiological, clinical, histological, immunohistochemical and therapeutic outcomes) of our case and confront it to literature data.

Highlights

Medulloepithelioma is a rare primitive neuroectodermal tumor of the central nervous system, usually developing in childhood
We report a case of infratentorial medulloepithelioma in a 3-year-old girl
Brain magnetic resonance imaging (MRI) showed a large cerebellar vermis lesion compressing the 4th ventricle with surrounding edema and triventricular hydrocephalus, the mass demonstrated a heterogeneous low signal on T2-weighted (Fig. 1)

Summary

Background

Medulloepithelioma of the central Nervous System (CNS) is an uncommon primitive neuroectodermal tumor (PNET), characterized by a highly malignant behavior [1] It is a rare tumor usually located in the supratentorial area [2], with the peak incidence between 6 months to 5 years of age. Case presentation A 3 year-old girl was admitted to the emergency department with intracranial hypertension picture without fever of 30 days duration. On examination, she had coordination problems, ptosis and exotropia of the right eye. The clinical picture is nonspecific and is based on the seat of the lesion Most often, it is a syndrome of intracranial hypertension with or without a neurological deficit.

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