Clinical features and surgical treatment of hemophilic pseudotumors: A single-center experience over a 20-year period.

Abstract

Background: Hemophilic pseudotumor (HPT) is an uncommon but severe complication in patients with hemophilia (PWH). Given the rarity, case series were scarce and largely confined to case report in the literature. Consequently, the aim of this study is to present surgical results of HPT over a 20-year period from a single institute.

Methods: We retrospectively reviewed 11 HPT in 10 PWH who underwent surgical treatment in our institute between 1999 and 2020. Their clinical features, surgical management, outcomes, and complications were identified.

Results: There are 10 men PWH with 11 HPT who had undergone a total of 20 surgical procedures over a 20-year period. The mean age at surgery was 41.2 years with an average follow-up of 9.7 years. Nine patients had hemophilia A (five severe, three moderate, and one mild) and one patient had severe hemophilia B. Localization of the HPT was confined to soft tissue in two patients (9.1%), to intraosseous lesion in five patients (45.5%) and to subperiosteal location in the remaining four patients (36.4%). Three patients (27.2%) had complications, including one wound infection, one chronic osteomyelitis, and one recurrence. Postoperative complete resolution was achieved in nine HPT except one recurrence and one persistent chronic osteomyelitis; the former was stable after further excision, and the latter was resolved by amputation.

Conclusion: Surgical therapy for HTP is feasible and effective when covered with adequate factor replacement. Given the variety, careful case-to-case evaluation is important to take the appropriate surgical modality and avoid potential complication.