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Presented by Edward McCarthy, M.D. and prepared by Jeffrey Seibel, M.D. Ph.D.
Case 5: A four year old boy had pain in his skull for 6 months.
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1. Question
Week 78: Case 5
A four year old boy had pain in his skull for 6 months./images/031102case5a.jpg
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/images/031102case5e.jpgCorrect
Answer: Fibrous dysplasia
Histology: Radiographs demonstrate a lesion in the superior portion of the orbit. The lesion is associated with some reactive bone formation. Histologically, there is a fibroblastic stroma with seams of new bone deposited in an “alphabet soup pattern.” There is no atypia in the fibrous stroma. In addition, there are zones of aneurysmal bone cyst with dilated blood-filled lakes and fibrous septae.
Discussion: Fibrous dysplasia is common in the cranial skeleton and may involve both the calvarium and/or the facial bones. When present in the skull, fibrous dysplasia is often accompanied by extensive aneurysmal bone cyst formation. Histologically, there is a fibroblastic stroma with seams of new bone deposited in an “alphabet soup pattern.” There is no atypia in the fibrous stroma. In addition, there are zones of aneurysmal bone cyst with dilated blood-filled lakes and fibrous septae.
The lesion is not a fibrosarcoma or an osteosarcoma because the cellular pleomorphism present in both these tumors is absent in this case.
Incorrect
Answer: Fibrous dysplasia
Histology: Radiographs demonstrate a lesion in the superior portion of the orbit. The lesion is associated with some reactive bone formation. Histologically, there is a fibroblastic stroma with seams of new bone deposited in an “alphabet soup pattern.” There is no atypia in the fibrous stroma. In addition, there are zones of aneurysmal bone cyst with dilated blood-filled lakes and fibrous septae.
Discussion: Fibrous dysplasia is common in the cranial skeleton and may involve both the calvarium and/or the facial bones. When present in the skull, fibrous dysplasia is often accompanied by extensive aneurysmal bone cyst formation. Histologically, there is a fibroblastic stroma with seams of new bone deposited in an “alphabet soup pattern.” There is no atypia in the fibrous stroma. In addition, there are zones of aneurysmal bone cyst with dilated blood-filled lakes and fibrous septae.
The lesion is not a fibrosarcoma or an osteosarcoma because the cellular pleomorphism present in both these tumors is absent in this case.
