《Slide 166.》Chordoma, Sacrum

A. Brief Descriptions：

1. Definition: a notochord-derived tumor of the skull and spine.

B. Gross Findings：

1. Infiltrative， osseodestructive，often lobulated lesion with mucoid appearance.

2. 極少見intratumoral hemorrhage.

C. Micro Findings：

1. Oval or round lobules of vacuolated cell tumors delineated by fibrous septa in myxoid matrix in cording pattern.

2. Tumor cells in epithelial or “hepatocyte-like” rows or cords or strands.

3. Other tumor cells have abundant eosinophilic vacuolated cytoplasm (physaliferous cells) and indistinct cell boundaries (resulting in a syncytial quality).

4. Between the cell cords is abundant extracellular mucoid matrix.

5. 有時可見malignant foci，病理特徵是hyperchromatism、pleomorphism、mitotic activity.

D. Others:

1. Notochordal tumors沒有benign的，它是屬於malignant nonvascular tumors。

2. 臨床特色：

   • Distributed most often in the midline along the craniospinal axis， approximately half arise in the sacrum，one third in the spheno-occipital region or clivus，and the remainder in the articulating vertebrae

   • Male predominate and most often over 30 years old

   • Sacral chordoma是具有破壞性的intrasacral tumors，臨床上因為壓迫到其他神經，所以會引起疼痛、擴約肌功能障礙等神精學上問題。

   • 而生長在腦內的（Intracranial lesions）會引發unilateral cranial nerve signs的臨床症狀。

   • 轉移的可能地方: lung，lymph node，skin.

3. Treatment

   • Wide excision and postoperative radiotherapy.

4. Prognostic factors

   • extent of resection.

   • patient age（40歲以下預後較好）

   • presence of mitotic activity.

5. 可能轉成的惡性腫瘤有undifferentiated spindle cell tumor、malignant fibrous histiocytoma、chondrosarcoma.

E. Reference：

1. Robbins Pathologic Basis of Disease, 6^th ed.  P.1584-1585.

　

 

 

 

【 Fig. 166-1 (LP)】Lobulated pattern of tumor with myxoid stroma.

　

 

 

【 Fig. 166-2 (LP)】Myxoid stroma.

　

　

 

【 Fig. 166-3 (HP)】Tumor cells in epithelial rows or cords with myxoid stroma.

　

 

【 Fig. 166-4 (HP)】Physaliferous cells with abundant vaculated cytoplasm.