Mathkour Mansour, Garces Juanita, Beard Bryce, Bartholomew Angela, Sulaiman Olawale A R, Ware Marcus L
Department of Neurosurgery, Ochsner Health System, New Orleans, Louisiana, USA; Department of Neurosurgery, Tulane Medical Center, New Orleans, Louisiana, USA.
Department of Neurosurgery, Ochsner Health System, New Orleans, Louisiana, USA; Department of Neurosurgery, Tulane Medical Center, New Orleans, Louisiana, USA.
World Neurosurg. 2016 May;89:730.e9-730.e13. doi: 10.1016/j.wneu.2016.01.054. Epub 2016 Feb 1.
Osteosarcoma is the second most common primary tumor of the skeletal system and the most common primary bone tumor. Usually occurring at the metaphysis of long bones, osteosarcomas are highly aggressive lesions that comprise osteoid-producing spindle cells. Craniofacial osteosarcomas comprise <8% and are believed to be less aggressive and lower grade. Primary osteosarcomas of the skull and skull base comprise <2% of all skull tumors. Osteosarcomas originating from the clivus are rare. We present a case of a primar, high-grade clival osteosarcoma.
A 29-year-old man presented to our institution with a progressively worsening right frontal headache for 3 weeks. There were no sensory or cranial nerve deficits. Computed tomography revealed a destructive mass involving the clivus with extension into the left sphenoid sinus. Magnetic resonance imaging revealed a homogenously enhancing lesion measuring 2.7 × 2.5 × 3.2 cm. The patient underwent endonasal transphenoidal surgery for gross total resection. The histopathologic analysis revealed proliferation of malignant-appearing spindled and epithelioid cells with associated osteoclast-like giant cells and a small area of osteoid production. The analysis was consistent with high-grade osteosarcoma. The patient did well and was discharged on postoperative day 2. He was referred for adjuvant radiation therapy and chemotherapy. Two-year follow-up showed postoperative changes and clival expansion caused by packing material.
Osteosarcoma is a highly malignant neoplasm. These lesions are usually found in the extremities; however, they may rarely present in the craniofacial region. Clival osteosarcomas are relatively infrequent. We present a case of a primary clival osteosarcoma with high-grade pathology.
骨肉瘤是骨骼系统中第二常见的原发性肿瘤，也是最常见的原发性骨肿瘤。骨肉瘤通常发生在长骨的干骺端，是由产生骨样组织的梭形细胞构成的高度侵袭性病变。颅面部骨肉瘤占比不到8%，一般认为其侵袭性较低、分级也较低。颅骨和颅底的原发性骨肉瘤占所有颅骨肿瘤的比例不到2%。起源于斜坡的骨肉瘤很罕见。我们报告一例原发性、高级别斜坡骨肉瘤病例。
一名29岁男性因进行性加重的右侧额部头痛3周就诊于我院。无感觉或颅神经功能缺损。计算机断层扫描显示一个破坏性肿块累及斜坡并延伸至左侧蝶窦。磁共振成像显示一个均匀强化的病变，大小为2.7×2.5×3.2厘米。患者接受了经鼻蝶窦手术以实现肿瘤全切。组织病理学分析显示出现恶性梭形和上皮样细胞增殖，伴有破骨细胞样巨细胞和小面积骨样组织形成。分析结果符合高级别骨肉瘤。患者恢复良好，术后第2天出院。他被转诊接受辅助放疗和化疗。两年随访显示有术后改变以及由填充材料导致的斜坡扩大。
骨肉瘤是一种高度恶性肿瘤。这些病变通常见于四肢；然而，它们很少出现在颅面部区域。斜坡骨肉瘤相对少见。我们报告一例具有高级别病理特征的原发性斜坡骨肉瘤病例。
