Successful combination therapy of a proximal haemophilic pseudotumour with surgery, radiation and embolization in a child with mild haemophilia A

Abstract

We describe the management of a young boy with mild haemophilia A and a massive iliac pseudotumour with a multi modality approach involving factor replacement, radiation therapy, embolization and surgery. The patient was initially treated with recombinant factor VIII and radiation therapy. Because of inadequate response and worsening of bony erosion, the patient had a preoperative embolization followed by surgical excision. The surgical procedure was associated with minimal blood loss and the patient had a relatively smooth postoperative course with no physical morbidity. This case illustrates successful aggressive management of a large, proximally located pelvic pseudotumour, which resulted in an excellent outcome despite the need for a normally morbid operation.

Transcatheter arterial embolisation for paediatric inflammatory pseudotumour of the maxillary sinus

“…However, no previous report has presented the utility of arterial embolisation as the sole treatment for inflammatory pseudotumour. 20 The precise effect of embolisation on tumour cells remains largely unknown. 21 The ischaemia and hypoxia generated by arterial embolisation are possible mechanisms for the observed reduction in inflammatory pseudotumour mass.…”

Tibial Pseudotumor in a Child With Hemophilia

“…If the tumor has disappeared, it is considered cured, but if the tumor is still present it should be surgically treated [3]. Surgical resection is curative for most pseudotumors but this modality includes risks of massive lifethreatening hemorrhage, infection, and even possible limb amputation [8]. Radiotherapy has been recommended by some authors, especially in the presence of factor VIII inhibitor [12].…”

“…In our case, plain x-ray findings (Figure 1) were not diagnostic and the possibility of a neoplasm could not be excluded from the initial differential diagnosis, but 628 E. S. Hatzipantelis et al There are no standard protocols in the literature with regards to the management of pseudotumors. Collaboration of many specialists and special centers are needed for the treatment of such patients [3,8]. The choice of therapy depends on many parameters such as the size of the tumor, the age of the patient, and the relation with underlying organs.…”

Management of haemophilic pseudotumours with special emphasis on radiotherapy and arterial embolization

“…The reverse is also true; Ahuja et al. [34] and Echternacht et al. [39] each reported one lesion of recent development in which radiotherapy failed to halt their progression.…”

“…Other authors successfully used embolization as an adjunct to surgical resection so they can reduce the pseudotumour size and vascularity and consequently the chance of massive haemorrhage during operation [34,46] (Table 3). Sevilla et al.…”