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Multicentric Chordoma in a Child.

Shighakolli Ramesh1, Raju Subodh1, Srinadh Boppana2

  • 1Department of Neurosurgery, Kamineni Hospitals, Hyderabad, Telangana, India.

Journal of Pediatric Neurosciences
|September 15, 2017
PubMed
Summary

Multicentric chordoma, a rare bone cancer originating from notochordal remnants, is reported for the first time in a pediatric patient. This aggressive tumor requires intensive multidisciplinary treatment, highlighting the need for early diagnosis and management in children.

Keywords:
Chordomamulticentricpediatric

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Area of Science:

  • Oncology
  • Pediatric Oncology
  • Skeletal Biology

Background:

  • Chordomas are rare malignant bone tumors arising from the axial skeleton, originating from embryonic notochordal cell rests.
  • Multicentric chordoma, presenting in multiple locations simultaneously, is exceptionally rare, with only three adult cases documented previously.

Observation:

  • A 14-month-old infant presented with torticollis and left upper limb monoparesis.
  • Imaging revealed simultaneous, expansile, bone-destructive lesions in the clivus and dorsal spine.
  • Histopathology confirmed chordoma, marking the first reported pediatric case of multicentric chordoma.

Findings:

  • The pediatric case of multicentric chordoma exhibited aggressive tumor behavior.
  • Diagnosis involved imaging and histopathological confirmation of chordoma in multiple axial skeletal sites.

Implications:

  • This case underscores the possibility of multicentric chordoma in pediatric patients, a previously unreported occurrence.
  • Aggressive pediatric chordomas necessitate a multidisciplinary approach, including maximal safe surgical resection and adjuvant radiotherapy (conventional and/or proton therapy).
  • Despite comprehensive management, pediatric chordomas are associated with significant morbidity and mortality, emphasizing the need for further research and improved therapeutic strategies.