Department of Neurosurgery, Kanazawa Medical University, Ishikawa, Japan.
Neurosurgery. 2011 Oct;69(4):E1010-5; discussion E1015-6. doi: 10.1227/NEU.0b013e318223b651.
The authors report a rare case of multiple intraosseous inflammatory myofibroblastic tumors presenting with an aggressive clinical course.
A 60-year-old man presented with a 3-month history of headache and 2 weeks of jaw pain. Magnetic resonance imaging showed a homogeneously enhancing mass in the right parietal bone with subcutaneous and intracranial invasion. Bone scintigraphy revealed 4 intraosseous lesions involving the cranium, mandible, ischium, and calcaneum. After admission, the patient showed left hemiparesis and seizures caused by rapid intracranial tumor extension. The cranial and mandible tumors were resected. Histopathological examinations of both specimens revealed myofibroblastic spindle cell proliferation with inflammatory cell infiltration, and a diagnosis of inflammatory myofibroblastic tumor was made. Two days postoperatively, the patient presented with a high fever and disturbance of consciousness with swelling of the subcutaneous tissues of the head and mandibular lesions. Magnetic resonance imaging revealed a massive intracranial extension of the tumor. Corticosteroid therapy induced remarkable shrinkage of all lesions, and relief from symptoms was obtained. Radiotherapy was then performed for residual tumors.
Multiple intraosseous inflammatory myofibroblastic tumors of the bone are very uncommon and may mimic malignant tumors. It is important to recognize that this entity can occur in the cranium and as multiple bony lesions. The recommended treatment is complete surgical resection with adjuvant steroid treatment. Considering the aggressive nature of this entity, additional chemo- and/or radiotherapy may be warranted.
作者报告了一例罕见的多发性骨内炎症性肌纤维母细胞瘤病例，其临床表现呈侵袭性。
一名 60 岁男性，因头痛病史 3 个月，颌痛 2 周就诊。磁共振成像显示右顶骨内均质强化肿块，伴皮下及颅内侵犯。骨扫描显示颅、下颌骨、坐骨和跟骨 4 处骨内病变。入院后，患者因颅内肿瘤迅速扩展而出现左侧偏瘫和癫痫。切除颅顶和下颌骨肿瘤。两份标本的组织病理学检查均显示肌纤维母细胞梭形细胞增生伴炎症细胞浸润，诊断为炎症性肌纤维母细胞瘤。术后 2 天，患者出现高热和意识障碍，伴有头部和下颌病变的皮下组织肿胀。磁共振成像显示肿瘤广泛颅内扩展。皮质类固醇治疗使所有病变显著缩小，症状缓解。然后对残留肿瘤进行放疗。
骨内多发性炎症性肌纤维母细胞瘤非常罕见，可能类似于恶性肿瘤。重要的是要认识到这种病变可发生在颅骨和多处骨病变。推荐的治疗方法是完整的手术切除加辅助类固醇治疗。鉴于该病变具有侵袭性，可能需要额外的化疗和/或放疗。
