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[Chordomas].

Y Le Charpentier1, S Bellefqih, S Boisnic

  • 1Service Central d'Anatomie, Groupe Hospitalier Pitié-Salpêtrière, Paris.

Annales De Pathologie
|January 1, 1988
PubMed
Summary
This summary is machine-generated.

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Chordomas are rare, slow-growing malignant tumors typically found in adults, often presenting with pain due to compression. Early surgical excision is key, with radiotherapy for recurrences, and survival rates range from 50-75%.

Area of Science:

  • Oncology
  • Pathology
  • Surgical Oncology

Context:

  • Chordomas are rare, slow-growing malignant tumors originating from notochordal remnants.
  • Predominantly affecting adults aged 50-60, they commonly occur in the sacrococcygeal or spheno-occipital regions.
  • Clinical presentation often involves pain or symptoms related to compression of adjacent structures.

Purpose:

  • To describe the characteristics, diagnosis, treatment, and prognosis of chordomas.
  • To differentiate chordomas from other tumors using specific cellular markers.
  • To highlight the unique features and better prognosis of chondroid chordoma.

Summary:

  • Radiologically, chordomas exhibit osteolysis and soft tissue opacity. Microscopically, they feature epithelial-appearing cells with acid mucosubstances, glycogen, and neutral mucosubstances.

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  • Cells express a triple positivity for epithelial antigens, Vimentin, and S-100 Protein, aiding differential diagnosis.
  • Complete surgical excision is the primary treatment; radiotherapy is used for recurrences. Metastasis occurs in 10-15% of cases, with a 5-year survival rate of 50-75%.

    • Impact:

    • Accurate diagnosis through cellular marker identification is crucial for appropriate management.
    • Understanding treatment modalities like surgery and radiotherapy can improve patient outcomes.
    • The identification of chondroid chordoma as a distinct entity with a better prognosis offers hope for younger patients.