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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

Case Study 6

Harry Kellermier, M.D.

question 1
Question 1

Describe this T1 weighted MRI and give a radiologic differential.

answer
Answer

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

question 2
Question 2

Describe the microscopic findings.

Click here to view slide.

answer1
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.

question 3
Question 3

What is your histologic differential diagnosis?

answer2
Answer

Chordoma, Chondrosarcoma, Adenocarcinoma

question 4
Question 4

What immunostains would you order?

answer3
Answer

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

question 5
Question 5

Based on the following immunohistochemical results, what is your diagnosis?

answer4
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.
question 6
Question 6

What are the "bubbly" cells called in this tumor?

answer5
Answer

Physaliphorous cells (Greek - bubble bearing)

question 7
Question 7

From what developmental structure does this tumor arise?

answer6
Answer

The notochord

question 8
Question 8

What anatomic locations do they typically involve?

answer7
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.

question 9
Question 9

What is the name of the benign developmental remnant of notochord that histologically resembles a chordoma?

answer8
Answer

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

question 10
Question 10

How do chordomas clinically behave?

answer9
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.

question 11
Question 11

Name 2 other chordoid neoplasms of the CNS.

answer10
Answer

Chordoid glioma of the third ventricle and Chordoid meningioma.