Background: As chordomas are slow growing and locally invasive with high recurrence rates, initial recommendations include complete surgical resection with or without radiation therapy. A large proportion of recurrences occur years after initial resection necessitating lengthy follow-up. The novel biomarker brachyury and the repurposing of pharmaceutical products have the potential to substantially impact long-term recurrence rates.
Case Presentation: A 43-year-old woman presented with an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root underwent a C3-5 laminectomy, C3-5 lateral mass screw instrumentation, and mass resection. All symptoms resolved by the 12-month postoperative follow-up visit.
Conclusions: This is the first report of an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root, and this case adds to the previous six Type IV chordomas in the literature. Unfortunately, the very rare form of extraosseous intradural chordoma is poorly understood: the lack of detailed knowledge in how they are differentiated from other forms of chordoma confounds the development of optimal treatment strategies and follow-up guidelines.
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http://dx.doi.org/10.1186/s41016-019-0170-y | DOI Listing |
Neurochirurgie
July 2024
Department of Neurosurgery, La Timone Hospital, Aix-Marseille University, Marseille, France.
Intradural Extraosseous Ewing sarcoma (IEES) is an infrequent occurrence. We report a case of a 66-year-old male who presented with a 2-month history of low back pain and bilateral S1 sciatica, with acute sphincter dysfunction. Imaging studies revealed an intradural extramedullary lesion in the cauda equina spanning from level L4 to S1.
View Article and Find Full Text PDFJ Neurosurg Case Lessons
January 2024
Departments of1Neurosurgery, and.
Background: Ewing's sarcoma is an uncommon, aggressive malignancy that typically presents as an osseous lesion, most commonly in children and adolescents. Very rarely Ewing's sarcoma can present as an intradural extramedullary mass mimicking more common tumors.
Observations: A 32-year-old female had a left L3 nerve root-associated lesion identified in the setting of recent-onset radiculopathy.
Ann Neurosci
October 2023
Department of Pathology and Lab Medicine, All India Institute of Medical Science Bhopal, Saket Nagar, Habib Ganj, Bhopal, Madya Pradesh, India.
Ewing's Sarcomas (ES)/Peripheral neuroectodermal tumour (pPNET) are heterogenous group of rare, highly malignant, undifferentiated primitive round-cell neoplasms of neuroectodermal origin. pPNETs are seldom observed to involve the spine of which Spinal Intradural Extramedullary Extraosseous Primary ES/pPNET are extremely rare. We report a case of a 23-year-old male with complaints of low backache and hip pain radiating to the left inguinal region for four months.
View Article and Find Full Text PDFNeuropathology
April 2023
Department of Neurosurgery, Yokohama City Minato Red Cross Hospital, Yokohama, Japan.
Other iatrogenic immunosuppressive-associated lymphoproliferative disorders (OIIA-LPDs) rarely occur in the central nervous system (CNS). Additionally, they almost always present as lymphoma and withdrawal by cessation of immunosuppressive treatment. We report a case of primary CNS OIIA-LPD that presented as extraosseous plasmacytoma (EP) with a progressive clinical course in spite of immunosuppressive treatment cessation.
View Article and Find Full Text PDFCureus
July 2022
Neurosurgery, University of Puerto Rico, Medical Sciences Campus, San Juan, PRI.
Primary spinal extraosseous Ewing sarcoma (ES) is a rare mesenchymal tumor characterized by high malignancy, occurring in a few patients with ES. The occurrence of this tumor in the intradural extramedullary spinal region is infrequent. This systematic review examines primary extraosseous intradural extramedullary ES in the cervical region to provide specific outcomes and evaluate the role of adjuvant chemoradiation in overall prognosis.
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