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.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7398216PMC
http://dx.doi.org/10.1186/s41016-019-0170-yDOI Listing

Publication Analysis

Top Keywords

extraosseous intradural
16
isolated cervical
12
cervical extraosseous
12
chordoma attached
12
attached nerve
12
nerve root
12
intradural chordoma
8
root case
8
intradural extramedullary
8
extramedullary chordoma
8

Similar Publications

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 PDF

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.

View Article and Find Full Text PDF

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 PDF

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 PDF

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.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!