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Neural Tumours

Pathology of Myxopapillary Ependymoma

Dr Sampurna Roy MD                

 

 

Pathology Quiz Case 4: Case history and images

Diagnosis : Subcutaneous Myxopapillary Ependymoma

 

                                                                                                                      

 

Myxopapillary ependymoma is a distinctive variant of ependymoma which was first described as a separate pathological entity by Kernohan JW  (Primary tumours of the spinal cord and intradural filum terminale - Cytology and cellular pathology of the nervous system. Vol 3 , 1932: 993-1025).

These tumours are usually restricted to the conus medullaris and filum terminale.

Extraspinal (soft tissue) myxopapillary ependymomas are rare tumours  which occur as direct extension into the soft tissue of the sacrococcygeal area from a primary ependymoma of the spinal cord, cauda equina or filum terminale.

Alternatively these may occur as primary pre-sacral, pelvic or abdominal tumours or as  primary tumours of the skin and subcutaneous tissue without direct connection to the spinal cord or filum terminale.

These tumours originate from extramedullary ependymal rests representing remnants of the coccygeal medullary vestige, an ependyma lined cleft, and is a derivative of the caudal neural tube persisting beneath the skin of the postanal pit .

These slow growing tumours usually affect patients in the 3rd and 4th decades and are slightly more common in males.

Clinically, this lesion is often misdiagnosed as a  pilonidal sinus or cyst.

The differential diagnosis also includes sacrococcygeal teratoma, neurogenic tumour, soft tissue sarcoma and metastatic carcinoma.

When the tumour involves sacrum and coccyx, tumours such as chordoma and chondrosarcoma must also be excluded.

Macroscopically the tumour usually presents as a well circumscribed, encapsulated, ovoid mass.

The cut surface has a greyish white, lobulated appearance .

In some cases cut surface shows mucoid areas, together with areas of hemorrhage, calcification, fibrosis and cystic changes.

Histopathology Image1 ; Image2 ; Image3 ; Image4 ; Image5
 

Myxopapillary ependymoma has a distinctive appearance consisting of cuboidal , columnar  and spindle cells separated by basophilic mucinous material and characteristic papillary arrangement around hyalinized blood vessels. 

Histologically the differential diagnosis includes chordoma and schwannoma. Immunopositivity of the tumour cells with GFAP helps in confirming the diagnosis of myxopapillary ependymoma.  

These are indolent tumours and in most cases are completely curable following total surgical resection.

Following incomplete removal of the tumour there is an increased risk of recurrence.

The tumour has the potential to metastasize to the lung, liver and lymph node.

Prolonged post operative follow up is necessary as late recurrences may occur several  years after the primary tumour is removed.

 

Further reading:

Subcutaneous myxopapillary ependymal rest: a potential sign of spinal dysraphism.

Subcutaneous sacrococcygeal myxopapillary ependymoma in asian female:a case report.

Sacrococcygeal extraspinal myxopapillary ependymoma.

Case report: primary subcutaneous sacrococcygeal ependymoma: a case report and review of the literature.

Lumbosacral ependymomas: a review of the management of intradural and extradural tumors.

Intraspinal extradural myxopapillary ependymoma of the sacrum arising from the filum terminale externa. Case report.

Myxopapillary ependymoma of the ischioanal fossa.

Fine needle aspiration (FNA) cytology of primary subcutaneous sacrococcygeal myxopapillary ependymoma.

Subcutaneous sacrococcygeal myxopapillary ependymoma.

Extraspinal ependymoma in the sacrococcygeal region. A case report with ultrastructural, immunohistochemical and cytophotometric studies.

Subcutaneous sacrococcygeal ependymoma with inguinal lymph node metastasis. Case report.

Sacrococcygeal extraspinal myxopapillary ependymoma.

Extradural myxopapillary ependymoma report of two cases and review of the literature.

Extraspinal ependymoma presenting as a subcutaneous mass posterior to the sacrococcygeal region.

Soft tissue ependymoma : a report of three cases.

Subcutaneous myxopapillary ependymoma and ependymal rests in infants and children.

Extraspinal ependymoma.

Immunophenotype of myxopapillary ependymomas.

Cervical extradural and extraspinal ependymoma mimicking dumb-bell schwannoma: an unusual tumor.

 

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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