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Abstract：
BACKGROUND: Hemophilic pseudotumor is a rare but well documented complication seen in approximately 1-2% of patients with hemophilia. The incidence continues to decrease, likely because of increasingly sophisticated techniques in managing factor deficiency. We present a case of hemophilic pseudotumor in a patient without hemophilia, an exceptionally rare entity, and outline a hybrid approach to treatment.
METHODS: The patient presented with a left sided iliopsoas mass and associated radiculopathy, with a history of a poorly characterized bleeding diathesis and Noonan\'s syndrome. He had no history of trauma and was not being treated with anti-coagulation. Of note, factors VIII, IX and XI were normal. An open biopsy was consistent with hemophilic pseudotumor. The patient underwent a hybrid procedure of preoperative embolization of the left internal iliac and left deep circumflex arteries followed by surgical debridement and resection, with an excellent outcome.
CONCLUSIONS: Hemophilic pseudotumor is rarely seen in patients with hemophilia, and even less frequently in patients without. Trauma is often the inciting event. A high index of clinical suspicion is required in order to secure the diagnosis, as the radiographic appearance is non-specific. Our patient had no history of trauma, although we question whether his underlying bleeding diathesis may have predisposed him to developing the pseudotumor. Surgery remains the cornerstone of management in these cases.
CONCLUSIONS: Within the literature, there are only two other cases of hemophilic pseudotumor occurring in a non-hemophiliac patient, highlighting the rarity of this case and the associated diagnostic dilemma.
METHODS: The patient presented with a left sided iliopsoas mass and associated radiculopathy, with a history of a poorly characterized bleeding diathesis and Noonan\'s syndrome. He had no history of trauma and was not being treated with anti-coagulation. Of note, factors VIII, IX and XI were normal. An open biopsy was consistent with hemophilic pseudotumor. The patient underwent a hybrid procedure of preoperative embolization of the left internal iliac and left deep circumflex arteries followed by surgical debridement and resection, with an excellent outcome.
CONCLUSIONS: Hemophilic pseudotumor is rarely seen in patients with hemophilia, and even less frequently in patients without. Trauma is often the inciting event. A high index of clinical suspicion is required in order to secure the diagnosis, as the radiographic appearance is non-specific. Our patient had no history of trauma, although we question whether his underlying bleeding diathesis may have predisposed him to developing the pseudotumor. Surgery remains the cornerstone of management in these cases.
CONCLUSIONS: Within the literature, there are only two other cases of hemophilic pseudotumor occurring in a non-hemophiliac patient, highlighting the rarity of this case and the associated diagnostic dilemma.
摘要：
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