Gao Zhen-Hua, Yin Jun-Qiang, Liu Da-Wei, Meng Quan-Fei, Li Jia-Ping
Department of Radiology, First Affiliated Hospital of Sun Yat-sen University, 510080 Guangzhou, China.
Department of Orthopedics, First Affiliated Hospital of Sun Yat-sen University, 510080 Guangzhou, China.
Cancer Imaging. 2013 Dec 11;13(4):520-6. doi: 10.1102/1470-7330.2013.0042.
To describe the clinical, imaging, and pathologic characteristics and diagnostic methods of telangiectatic osteosarcoma (TOS) for improving the diagnostic level.
The authors retrospectively reviewed patient demographics, serum alkaline phosphatase (AKP) levels, preoperative biopsy pathologic reports, pathologic materials, imaging findings, and treatment outcomes from 26 patients with TOS. Patient images from radiography (26 cases) and magnetic resonance (MR) imaging (22 cases) were evaluated by 3 authors in consensus for intrinsic characteristics. There were 15 male and 11 female patients in the study, with an age of 9-32 years (mean age 15.9 years).
Eighteen of 26 patients died of lung metastases within 5 years of follow-up. The distal femur was affected more commonly (14 cases, 53.8%). Regarding serum AKP, normal (8 cases) or mildly elevated (18 cases) levels were found before preoperative chemotherapy. Radiographs showed geographic bone lysis without sclerotic margin (26 cases), cortical destruction (26 cases), periosteal new bone formation (24 cases), soft-tissue mass (23 cases), and matrix mineralization (4 cases). The aggressive radiographic features of TOS simulated the appearance of conventional high-grade intramedullary osteosarcoma, though different from aneurysmal bone cyst. MR images demonstrated multiple big (16 cases) or small (6 cases) cystic spaces, fluid-fluid levels (14 cases), soft-tissue mass (22 cases), and thick peripheral and septal enhancement (22 cases). Nine of 26 cases were misdiagnosed as aneurysmal bone cysts by preoperative core-needle biopsy, owing to the absence of viable high-grade sarcomatous cells in the small tissue samples.
The aggressive growth pattern with occasional matrix mineralization, and multiple big or small fluid-filled cavities with thick peripheral, septal, and nodular tissue surrounding the fluid-filled cavities are characteristic imaging features of TOS, and these features are helpful in making the correct preoperative diagnosis of TOS.
描述毛细血管扩张性骨肉瘤(TOS)的临床、影像学和病理特征及诊断方法,以提高诊断水平。
作者回顾性分析了26例TOS患者的人口统计学资料、血清碱性磷酸酶(AKP)水平、术前活检病理报告、病理材料、影像学表现及治疗结果。由3位作者共同评估26例X线平片及22例磁共振(MR)成像的患者图像的内在特征。研究中男性15例,女性11例,年龄9 - 32岁(平均年龄15.9岁)。
26例患者中有18例在随访5年内死于肺转移。股骨远端受累更为常见(14例,53.8%)。术前化疗前血清AKP水平正常(8例)或轻度升高(18例)。X线平片显示地图样骨质溶解,无硬化边缘(26例)、皮质破坏(26例)、骨膜新生骨形成(24例)、软组织肿块(23例)及基质矿化(4例)。TOS的侵袭性X线表现类似传统的高级别髓内骨肉瘤,但不同于动脉瘤样骨囊肿。MR图像显示多个大的(16例)或小的(6例)囊性间隙、液 - 液平面(14例)、软组织肿块(22例)及周边和分隔增厚强化(22例)。26例中有9例术前粗针穿刺活检误诊为动脉瘤样骨囊肿,原因是小组织样本中缺乏存活的高级别肉瘤细胞。
具有偶尔基质矿化的侵袭性生长方式,以及多个大小不一的充满液体的腔隙,周围有增厚的周边、分隔及结节状组织,是TOS的特征性影像学表现,这些特征有助于TOS的术前正确诊断。