Management of Hemophilic Pseudotumor of Thumb in a Child

Abstract

A case of hemophilic pseudotumor of the thumb in a child is reported. Combined treatment with radiation therapy and factor VIII replacement resulted in complete resolution of the lesion with no recurrence at the 6-month follow-up. The combination of radiotherapy and factor replacement should be strongly considered in the treatment of bone pseudotumors in children.

Hemophilic Pseudotumor

“…3,5,6,[20][21][22] Three patterns of hemophilic pseudotumor have been characterized. 5,7,11,23 The first type occurs within muscle or soft tissues, especially around the femur and pelvis. It arises from an arteriovenous fistula.…”

“…2 The second arises from a subperiosteal hemorrhage and periosteal elevation and reaction is observed. 7 The third type arises within the bone itself, causing expansion of the bone and thinning of the cortex and is the least common. 5,7,23 As in our case, pseudotumor of the cranium is an example of this third type.…”

“…7 The third type arises within the bone itself, causing expansion of the bone and thinning of the cortex and is the least common. 5,7,23 As in our case, pseudotumor of the cranium is an example of this third type. 5 Histologically, hemophilic pseudotumors are composed of coagulum at various stages of evolution 23 with a fibrous capsule containing hemosiderin-laden macrophages, 10,18 granulation tissue, fibrinoleukocyte material, and giant cells, which are often seen with foreign body reactions.…”

“…10 As a general principle, mild-to-moderate lesions of recent origin, not causing pain or deformity can be managed with factor VIII infusions and immobilization. 3,5,7,23 Immobilization and replacement therapy for at least 8 weeks leads to healing in most patients. 7 We treated this child with factor VIII 50 U/kg every 12 hours for 2 weeks to achieve a factor level of 100% at all times.…”

“…[2][3][4][5] Pseudotumors are seen in 1% to 2% of patients with severe factor VIII or IX deficiency. 2,[5][6][7] The term "pseudotumor" derives from the roentgenographic skeletal changes consisting of areas of bone destruction and new bone formation as well as calcification and/or ossification of surrounding soft tissues, resembling a malignant skeletal tumor, particularly osteosarcoma. 8 There is no universally agreed treatment protocol for hemophilic pseudotumor.…”