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Case Study 6

Case Study 6. Harry Kellermier, M.D. Question 1. Describe this T1 weighted MRI and give a radiologic differential. Answer. Destructive lesion involving the clivus.  The radiologic differential includes chordoma, chondrosarcoma, and less likely metastasis. Question 2.

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Case Study 6

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  1. Case Study 6 Harry Kellermier, M.D.

  2. Question 1 Describe this T1 weighted MRI and give a radiologic differential.

  3. Answer Destructive lesion involving the clivus.  The radiologic differential includes chordoma, chondrosarcoma, and less likely metastasis.

  4. Question 2 Describe the microscopic findings. Click here to view slide.

  5. Answer This neoplasm is composed of hepatoid trabeculae of epithelioid cells with eosinophilic and bubbly cytoplasm in a myxoid matrix.  Some areas appear somewhat chondroid.

  6. Question 3 What is your histologic differential diagnosis?

  7. Answer Chordoma, Chondrosarcoma, Adenocarcinoma

  8. Question 4 What immunostains would you order?

  9. Answer Keratins (AE1/AE3, Cam 5.2, or Pankeratin), S100, EMA, CEA

  10. Question 5 Based on the following immunohistochemical results, what is your diagnosis?

  11. AE1/AE3

  12. CAM5.2

  13. PANKERATIN

  14. EMA

  15. S100

  16. Answer • Chordoma - Keratin positive, EMA positive, S100 positive, CEA negative • Chondrosarcoma would be S100 positive, keratin negative, EMA negative; Adenocarcinoma would be Keratin positive, EMA positive, CEA positive, and usually S100 negative.

  17. Question 6 What are the "bubbly" cells called in this tumor?

  18. Answer Physaliphorous cells (Greek - bubble bearing)

  19. Question 7 From what developmental structure does this tumor arise?

  20. Answer The notochord

  21. Question 8 What anatomic locations do they typically involve?

  22. Answer Chordomas involve the midline of the axial skeleton.  30%-40% involve the base of the skull (clivus in the region of the spheno-occipital synchondrosis), approximately 49% involve the sacrum, and approximately 15% involve the vertebral column, usually the cervical spine.

  23. Question 9 What is the name of the benign developmental remnant of notochord that histologically resembles a chordoma?

  24. Answer Ecchordosis physaliphora.  These lesions are small, well-circumscribed gelatinous masses adherent to the brainstem.  They behave in a benign manner.

  25. Question 10 How do chordomas clinically behave?

  26. Answer Although they grow slowly, chordomas are characterized by local destruction and multiple recurrences.  Complete surgical resection may be curative, but is extremely difficult to attain.  Radiation therapy may improve survival.

  27. Question 11 Name 2 other chordoid neoplasms of the CNS.

  28. Answer Chordoid glioma of the third ventricle and Chordoid meningioma.

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