Giant hemophilic pseudotumor in the left thigh: A case report

Abstract

Introduction: Hemophilic pseudotumor (HPT) is a rare complication affecting patients with hemophilia. It is characterized by a painless mass that, due to multiple bleeding episodes, slowly but progressively increases in size, leading to serious complications such as fractures of adjacent bones.

Case presentation: A 54-year-old man with severe hemophilia A attended the emergency department of a tertiary care hospital in Bucaramanga (Colombia) due to the presence of a large mass on his left thigh, which had been growing progressively over the last 10 years, as well as pain and difficulty walking. A magnetic resonance imaging (MRI) scan performed upon admission revealed an intramuscular hematoma measuring 38.2 x 19.2 x 15.2 cm in the posterior compartment of the thigh. A diagnosis of HPT was made, and conservative treatment was initiated with the administration of human coagulation factor VIII (4000 IU). However, since an increase in the lesion was evident on the follow-up MRI one month later, percutaneous drainage of the HPT was performed. Two months after percutaneous drainage, endovascular embolization with microparticles was performed, achieving partial devascularization of the pseudotumor. One week later, a new collection was observed on ultrasound, resulting in drainage of serosanguineous fluid (3000 cc). The final treatment was complete resection of the lesion, which was performed 28 days later. However, since two seromas were identified on the medial side of the left thigh on follow-up MRI (20 days after surgery), the patient underwent further surgical drainage five days later. This multidisciplinary approach allowed obtaining good clinical and functional outcomes, since the patient was able to walk without assistive devices, his knee and hip mobility improved, and there was no evidence of HPT recurrence at the 18-month follow-up.

Conclusion: This case demonstrates that an interdisciplinary approach is essential to achieve satisfactory long-term clinical and functional outcomes in patients with large HPT. Furthermore, endovascular embolization is a therapeutic alternative to be considered in cases in which HPT poses surgical challenges.