- Department of Neurosurgery, Centro Hospitalar Lisboa Central-Hospital São José, Serrano, Lisboa, Portugal, Barcelona, Spain.
- Department of Neurosurgery, Hospital Universitari Vall d’Hebron, Barcelona, Spain.
- Department of Pathology, Hospital Universitari Vall d’Hebron, Barcelona, Spain.
Department of Neurosurgery, Hospital Universitari Vall d’Hebron, Barcelona, Spain.
DOI:10.25259/SNI-96-2019Copyright: © 2019 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Lino Fonseca, Marta Cicuendez, Francisco Martínez-Ricarte, Elena Martínez-Saez, Esteban Cordero, Agustín Bescos. A rare case of an intramedullary metastasis of a myxopapillary ependymoma. 10-May-2019;10:83
How to cite this URL: Lino Fonseca, Marta Cicuendez, Francisco Martínez-Ricarte, Elena Martínez-Saez, Esteban Cordero, Agustín Bescos. A rare case of an intramedullary metastasis of a myxopapillary ependymoma. 10-May-2019;10:83. Available from: http://surgicalneurologyint.com/surgicalint-articles/9338/
Background: Myxopapillary ependimoma (MPE) is a benign slow-growing tumor, and it has been designated histologically as a Grade I neoplasm according to the 2016 World Health Organization classification. Despite the benign character, dissemination and metastasis have occasionally been reported. The retrograde dissemination to other levels of the neuraxis is extremely rare, being more frequent to the intracranial compartment.
Case Description: We hereby present a case of medullary metastasis of cauda equina MPE, with a history of having undergone a subtotal resection and postoperative adjuvant radiotherapy. The patient presents complaints of night dorsal pain attributable to intradural metastasis twenty-one years after the first surgical intervention.
Conclusion: The case reported highlights the importance of long follow-up in patients with MPE, since the possibility of secondary seeding to distant craniospinal sites or local spinal sites after surgery, and radiotherapy should be considered in metastatic disease.
Keywords: Ependymoma, intramedullary, metastasis, myxopapillary
Myxopapillary ependymoma (MPE) was first described as a distinct subtype of ependymoma by Kernohaw in 1932.[
A 43-year-old Caucasian male started experiencing progressive dorsalgia, including nocturnal pain, and progressive resistance to analgesic therapy. He had previoulsy undergone lumbar spine surgery 21 years ago for MPE of cauda equina. A subtotal resection of the tumor was done and postoperative radiotherapy was performed with follow-up scans showing no evidence of residual lesion or any new lesion in the spinal cord. He reported no motor, numbness, bowel, or bladder dysfunction. The patient’s actual neurological examination was normal with no pyramidal or cauda equina compression signs. Magnetic resonance (MR) imaging studies showed an intramedullary lesion with expansive features extending from D5 to D10. The lesion had a cranial cystic and a caudal solid component. After the injection of gadolinium, there was an intense homogenous enhancement of the solid component and several small pachymeningeal lesions throughout the dorsal region and filum terminale. MR studies of the brain and cervical spine showed no abnormality [
Preoperative magnetic resonance images (MRI). (a) Sagittal T1 sequence with gadolinium contrast showing an intramedullary lesion at D9–D10 level with homogeneous enhacement and several small dorsal implants. (b) Sagittal T1 sequence with contrast where there is no evidence of residual lesion in filum terminale of the first surgery. (c) Sagittal T2 sequence showing an intramedullary isointense lesion in D9–D10 level with hemosiderin focus. (d) Axial T1 Axial MRI T1 with gadolinium contrast at D9 level where the lesion involves the whole spinal cord.
Smears of the specimen showed a papillary pattern with myxoid background (a, H and E, ×200) and presence of globules of myxoid substance (b, H and E, ×200). The paraffin sections reflexed these findings, with a microcystic myxoid background (c, H and E, ×200) and intense glial fibrillary acidic protein (GFAP) expression (d, GFAP, ×200).
Histologically, the lesion was composed of cells with medium- sized, oval nuclei with finely granular chromatin and occasional intranuclear pseudoinclusions. Also well defined eosinophilic cytoplasm with microcystic inclusions and basophilic myxoid material under Alcian-Blue staining. There were frequent hyalinized vessels. No mitotic activity, necrosis, or vascular proliferation was observed. Neoplastic cells expressed glial fibrillary acidic protein and were negative for cytokeratin AE1/ AE3 and EMA. Ki-67 proliferation index was inferior to 2%. A diagnosis of MPE was made (2016 WHO grade I).
The patient did not experience any new postoperative neurological deficits after surgery and he had an uneventful postoperative course. Adjuvant radiotherapy was planned after surgery.
Ependymal tumors originate from ependymal cell rests and are uncommon central nervous system neoplasms with an incidence of 0.2/100,000 person-years and a slight predominance in men and Caucasians.[
Ependymomas are divided into three histological entities according to the 2016 WHO classification: ependymoma, subependymoma, and MPE.[
As already mentioned, 75% of ependymomas in the adult population occur in the spinal cord, and 50% of these lesions are MPE.[
Despite the benign histology and slow-growing nature of most MPE tumors, some behave in an aggressive manner, with local recurrence, primary seeding, and metastasis. In the management of these patients, radiation therapy and chemotherapy are a treatment option after surgery.[
Neurosurgeons should be aware of the possibility of secondary seeding of an MPE to distant craniospinal sites or local spinal sites after surgery and should consider craniospinal imaging as part of preoperative workup and postoperative follow-up. Our case highlights the importance of long follow-up in patients with MPE. Although the limited data, radiotherapy should be considered in patients with metastatic disease.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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