Sim K B, Hong S K
Department of Neurosurgery, Seoul Red Cross Hospital, Korea.
Neurosurgery. 1996 Dec;39(6):1239-42. doi: 10.1097/00006123-199612000-00035.
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.
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.
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.
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.
血友病性假肿瘤是血友病的一种罕见并发症,在重度血友病患者中发生率为1%至2%。其主要发病部位是长骨和骨盆。此前仅有1例该肿瘤发生于颅骨的病例报道。
我们报告1例累及右顶骨板障的颅骨血友病性假肿瘤病例。一名29岁重度血友病(因子VIII,活性为正常的0.8%)男性患者,出现一个5×5×2 cm大小、外观难看的头皮突出物,以及左臂刺痛不适。入院前约5年,他注意到右顶区有一个核桃大小、无压痛的肿块,在轻微头部外伤后略有增大。头部外伤后2至3个月,因顶叶受压出现刺痛不适。
在适当的因子替代治疗下,进行手术切除并做组织诊断。板障内内容物的组织学检查显示为陈旧性血凝块。
术后,手臂的刺痛不适完全消失。据我们所知,这是英文文献中第二例颅骨血友病性假肿瘤病例。本文结合文献复习介绍了颅骨血友病性假肿瘤的诊断和治疗。