July 2018 - Presented by Dr. Dongguang Wei (Mentored by Dr. Tao Wang)

Clinical History

A 17-year-old female presented with right femur fracture and underwent intermedullary nailing and open reduction internal fixation. She complained right hip pain again 9 months after the surgery. X-ray revealed hardwire failure and non-union of the fracture. Biopsy demonstrated only vascular tissue. She underwent revision with hardware replacement. Culture of the non-union fracture comes back negative. She complained of persistent right hip pain, X-ray showed right femoral neck fracture around the nail and she was admitted again for a right femur removal of hardware and proximal femoral replacement.

Further Imaging Studies

CT and X-ray shows intraosseous hemangioma, diffuse demineralization/osteolysis of the proximal femur, underlying severe osteoporosis and tapering of bone.

Pathology Review

Multiple pathology reviews showed fragments of viable bones with remodeling changes, negative for acute inflammation; Benign lymphovascular proliferation with network of thin-walled vessels highlighted by CD31, CD34, and D2-40. Osteoclasts are noted within the adjacent bones, and demonstrate scalloped osteoclastic activity.

Images

Click on images to enlarge.

Figure 1
H&E 4x

Figure 2
H&E 10x

Figure 3
H&E 40x

  

  

Figure 4
CD31 10x

Figure 5
CD34, 10x

Figure 6
D2-40 10x


Which of the following is most likely the diagnosis?

Choose one answer and submit.






 

The correct answer is

D. Gorham-Stout Syndrome (Vanishing Bone Disease)

> Learn more about this diagnosis.