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Extra-axial chordoma.

Lisa M DiFrancesco1, Cristobal A Davanzo Castillo, Walley J Temple

  • 1Department of Pathology and Laboratory Medicine, University of Calgary, Alberta. lisa.difrancesco@cls.ab.ca

Archives of Pathology & Laboratory Medicine
|December 8, 2006
PubMed
Summary
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Extra-axial chordomas are rare bone tumors found outside the axial skeleton. This study presents a case and reviews literature, confirming their existence and distinct features.

Area of Science:

  • Oncology
  • Pathology
  • Skeletal Tumors

Background:

  • Chordomas are typically low-grade malignant bone tumors originating in the axial skeleton.
  • Extra-axial chordomas are rare variants with similar histologic and immunohistochemical features but occur outside the axial skeleton.
  • Differential diagnoses include parachordoma and myxoid chondrosarcoma.

Observation:

  • A case of an 8.3 cm extra-axial chordoma in the pubic bone of a 41-year-old woman is presented.
  • Gross, microscopic, and immunohistochemical findings were identical to classic chordoma.
  • Parachordoma and myxoid chondrosarcoma were excluded.

Findings:

  • Review of five previously reported cases and the current case confirms extra-axial chordomas share clinical and pathologic features with axial chordomas.

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  • Despite their location, these tumors exhibit characteristics specific to chordoma.
  • Histologic mimics must be differentiated due to differing treatment and prognosis.

    • Implications:

    • Extra-axial chordoma is a distinct entity that should be recognized in clinical and pathologic practice.
    • Management strategies for extra-axial chordoma should align with those for conventional chordoma.
    • Accurate diagnosis is crucial for appropriate treatment and patient outcomes.