RARE CASE OF MYXOPAPILLARY EPENDYMOMA FROM MEDULLA

Learning objectives

Myxopapillary ependymoma (WHO Grade I ) is a rare variant of ependymoma; this tumor was originally defined by Kernohan and it occurs almost exclusively in the conus medullaris or filum terminale.

Ependymomas have a wide morphologic spectrum and MPE constitutes a distinct clinico-pathological variant.

MPE that occurs primarily in the cerebral hemisphere or brainstem is extremely rare with only seven cases having been reported in the literature, and all of them have occurred in young patients

backgroundA 45 years old male patient presented with several episodes of syncopal attacks with right upper limb numbness

Plain CT revealed a well defined ovoid heterogeneous mildly hypodense lesion measuring ~ 3 cm filling the cisterna magna with mass effect on adjacent cerebellar hemispheres and medulla

Plain and contrast enhanced MRI revealed

A well circumscribed heterogeneous lesion in the cisterna magna and inferior aspect of fourth ventricle causing compression of medulla and splaying of inferior cerebellar peduncle with minimal expansion of cervico medullary junction

CT PLAIN

MRI T1 WI

T2WI- AXIAL

MRI - CONTRAST SAGGITAL /CORONAL VIEW

Patient had an episode of cardiac arrest and was revived and taken up for emergency decompression surgery.

Midline suboccipital craniectomy was performed

The lesion was seen arising from medulla and upper part of cervical cord with soft fleshy wall and a cystic area containing mucinous material

Partial resection of the lesion and cyst was achieved.

HISTO PATHOLOGY FINDINGS

Pathological Findings: Microscopically, the peculiar histologic features were papillae or reticular structures formed by cuboidal or flat cells with regular nuclei, and there was an abundant stroma showing conspicuous mucinous change.

The latter was strongly positive for alcian-blue and periodic acid-Schiff stain.

Multifocal hemorrhagic necrosis with fibrosis were evident.

HPE ..CONTThe tumor cells showed strong diffuse positivity for GFAP in the majority of the neoplastic cells. Focal positive reactions were also identified for S-100 protein and vimentin. However, the tumor cells were negative for cytokeratin. The Ki-67 labeling index was very low (<1%) and the tumor cells were negative for p53 protein. histopathological and clinicoradiologic studies confirmed that the

primary MPE arising in the medulla.

DISCUSSION Myxopapillary ependymoma is a sub-classification

of ependymoma that is thought to be nearly exclusive to the conus medullaris or filum terminale. primary intracerebral or brainstem myxopapillary ependymomas are very rare only seven cases of primary intracranial MPE have been reported

other regions where MPE presented

six of these tumors were locatedsupratentorial tumor group, 2 occurred in the intraventricular, 2 were intraparenchymal, 1 was transependymal, and 1 was extra-axial. the infratentorial tumor was restricted to the 4thventricle.

We present here a case of 45 years old male with Mxyo papillary ependymoma of the medulla, and we also review the relevant literature. To the best of our knowledge, this is the second documented case of the literature

Conclusion: Myxopapillary ependymoma thought to be nearly exclusive to the conus medullaris or filum terminale. but one should think about MPE intraparenchymal and brainstem and imaging/ histopathology helps us to treat the patient in advance

Patient now on radiotherapy and check MRI done for drop metastasis

References