K B Sim1, S K Hong. 1. Department of Neurosurgery, Seoul Red Cross Hospital, Korea.
Abstract
OBJECTIVE AND IMPORTANCE: Hemophilic pseudotumor is a rare complication of hemophilia, occurring in 1 to 2% of patients with severe hemophilia. Its principal sites of occurrence are the long bones and the pelvis. Only one case of this tumor occurring in the cranium has been previously reported. CLINICAL PRESENTATION: We report a case of cranial hemophilic pseudotumor involving the diploe of the right parietal vault. A 29-year-old man with severe hemophilia (Factor VIII, 0.8% of normal activity) presented with an unsightly scalp protrusion measuring 5 x 5 x 2 cm and tingling discomfort in the left arm. About 5 years before admission, he noted a walnut-sized, nontender mass in the right parietal area that had enlarged slightly after minor head trauma. Tingling discomfort developed as the manifestation of the compression of the parietal lobe in the 2 to 3 months after the head trauma. INTERVENTION: Under proper factor replacement therapy, surgery was undertaken for excision and tissue diagnosis. Histological examination of the content in the diploe revealed old blood coagulum. CONCLUSION: Postoperatively, the tingling discomfort in the arm resolved completely. To our knowledge, this is the second case of the cranial hemophilic pseudotumor in the English literature. Diagnosis and management of cranial hemophilic pseudotumor are presented with a review of the literature.
OBJECTIVE AND IMPORTANCE: Hemophilic pseudotumor is a rare complication of hemophilia, occurring in 1 to 2% of patients with severe hemophilia. Its principal sites of occurrence are the long bones and the pelvis. Only one case of this tumor occurring in the cranium has been previously reported. CLINICAL PRESENTATION: We report a case of cranial hemophilic pseudotumor involving the diploe of the right parietal vault. A 29-year-old man with severe hemophilia (Factor VIII, 0.8% of normal activity) presented with an unsightly scalp protrusion measuring 5 x 5 x 2 cm and tingling discomfort in the left arm. About 5 years before admission, he noted a walnut-sized, nontender mass in the right parietal area that had enlarged slightly after minor head trauma. Tingling discomfort developed as the manifestation of the compression of the parietal lobe in the 2 to 3 months after the head trauma. INTERVENTION: Under proper factor replacement therapy, surgery was undertaken for excision and tissue diagnosis. Histological examination of the content in the diploe revealed old blood coagulum. CONCLUSION: Postoperatively, the tingling discomfort in the arm resolved completely. To our knowledge, this is the second case of the cranial hemophilic pseudotumor in the English literature. Diagnosis and management of cranial hemophilic pseudotumor are presented with a review of the literature.