Recurrent skeletal extra-axial chordoma confirmed with brachyury: Imaging features and review of the literature Journal Article


Authors: Lantos, J. E.; Agaram, N. P.; Healey, J. H.; Hwang, S.
Article Title: Recurrent skeletal extra-axial chordoma confirmed with brachyury: Imaging features and review of the literature
Abstract: A small number of tumors bearing histological resemblance to axial chordoma arising from the bone or soft tissue outside the axial skeleton have been reported. These lesions have historically been referred to as parachordoma, chordoma periphericum (CP), or extra-axial chordoma (EAC). With the introduction of the immunohistochemical stain brachyury, a sensitive and specific marker for notochordal origin, chordomas arising in extra-axial locations (i.e., CP, EAC), are now diagnosed with more accuracy and distinguished from parachordoma, which resembles chordoma on histology. The distinction between EAC and parachordoma is clinically important because EAC confirmed by immunoreactivity for brachyury tends to grow and recur with local bone destruction. Prior to the introduction of brachyury, the diagnosis of EAC was challenging and therefore the imaging features of EAC have not been comprehensively described. We report two cases of recurrent EAC confirmed by the expression of brachyury arising from the distal femur and distal tibia and describe the imaging findings from radiography and MRI at initial diagnosis and at recurrence. © 2013 ISS.
Keywords: immunohistochemistry; adult; human tissue; protein expression; bone graft; bone tumor; unclassified drug; case report; ki 67 antigen; metastasis; differential diagnosis; tumor marker; tumor recurrence; tumor protein; radiography; protein s 100; physical examination; cytokeratin; epithelioid cell; chordoma; eosinophilic granuloma; mri; bone disease; knee radiography; ankle radiography; orthopedic surgery; vimentin; myoepithelioma; brodie abscess; bone biopsy; knee pain; whole body mri; brachyury; chordoma periphericum; extra-axial chordoma; parachordoma; protein ema; bone swelling; extraaxial chordoma; periosteal chondroma; periosteal chondrosarcoma; periosteal osteoblastoma
Journal Title: Skeletal Radiology
Volume: 42
Issue: 10
ISSN: 0364-2348
Publisher: Springer  
Date Published: 2013-10-01
Start Page: 1451
End Page: 1459
Language: English
DOI: 10.1007/s00256-013-1615-6
PROVIDER: scopus
PUBMED: 23653219
DOI/URL:
Notes: --- - "Export Date: 1 October 2013" - "CODEN: SKRAD" - "Source: Scopus"
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Narasimhan P Agaram
    190 Agaram
  2. Sinchun Hwang
    96 Hwang
  3. John H Healey
    547 Healey
  4. Joshua Lantos
    5 Lantos