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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Harry Kellermier, M.D.
Describe this T1 weighted MRI and give a radiologic differential.
Destructive lesion involving the clivus. The radiologic differential includes chordoma, chondrosarcoma, and less likely metastasis.
Describe the microscopic findings.
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This neoplasm is composed of hepatoid trabeculae of epithelioid cells with eosinophilic and bubbly cytoplasm in a myxoid matrix. Some areas appear somewhat chondroid.
What is your histologic differential diagnosis?
Chordoma, Chondrosarcoma, Adenocarcinoma
What immunostains would you order?
Keratins (AE1/AE3, Cam 5.2, or Pankeratin), S100, EMA, CEA
Based on the following immunohistochemical results, what is your diagnosis?
What are the "bubbly" cells called in this tumor?
Physaliphorous cells (Greek - bubble bearing)
From what developmental structure does this tumor arise?
What anatomic locations do they typically involve?
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.
What is the name of the benign developmental remnant of notochord that histologically resembles a chordoma?
Ecchordosis physaliphora. These lesions are small, well-circumscribed gelatinous masses adherent to the brainstem. They behave in a benign manner.
How do chordomas clinically behave?
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.
Name 2 other chordoid neoplasms of the CNS.
Chordoid glioma of the third ventricle and Chordoid meningioma.