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长骨中的巨细胞丰富性骨肉瘤:临床、放射学和病理学特征。

Giant cell-rich osteosarcoma in long bones: clinical, radiological and pathological features.

机构信息

Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, No.197, Ruijin 2nd Road, Shanghai, 200025, China.

出版信息

Radiol Med. 2013 Dec;118(8):1324-34. doi: 10.1007/s11547-013-0936-9. Epub 2013 May 27.

DOI:10.1007/s11547-013-0936-9
PMID:23716292
Abstract

PURPOSE

The purpose of this study was to review the clinical presentation, imaging, pathology and outcome of patients with giant cell-rich osteosarcoma (GCRO) of long bones.

MATERIALS AND METHODS

Radiography (n=9), magnetic resonance imaging (MRI) (n=6), computed tomography (CT) (n=3) and clinical course of nine patients (five males and four females; mean age, 26 years) with pathologically confirmed GCRO were retrospectively reviewed. Specific imaging findings, including size, eccentricity, ossification, lysis, cystic change, expansile growth, periosteal reaction, cortical destruction, soft tissue extension and joint involvement were documented.

RESULTS

Presenting symptoms were pain in six patients and pain and palpable mass in three. An ill-defined margin surrounding a predominantly osteolytic lesion was detected at the proximal tibia (n=7) or femur (n=2) on imaging studies. Seven cases showed limited ossification. Three cases had tumours in the metaphysis and six in the metaepiphysis. The average maximum tumour dimension was 4.7 cm×5.2 cm×7.8 cm. Microscopically, tumours were composed of atypical cells with scanty osteoid formation and multinucleated giant cells. All patients received chemotherapy, and surgery was performed in eight patients. Three patients were dead and six were alive at the last follow-up.

CONCLUSIONS

GCRO is a rarer variant that has very close resemblance to giant cell tumour. Patients usually present nonspecific symptoms of pain and palpable mass. It usually shows an osteolytic lesion with locally spared new bone formation in the metaphysis and/or metaepiphysis on imaging. Histologically, the atypical tumour cells with osteoid formation and multinucleated giant cells are the key factor in the diagnosis and differential diagnosis.

摘要

目的

本研究旨在回顾长骨巨细胞丰富型骨肉瘤(GCRO)患者的临床表现、影像学表现、病理学和转归。

材料和方法

回顾性分析 9 例经病理证实为 GCRO 患者的影像学(n=9)、磁共振成像(MRI)(n=6)、计算机断层扫描(CT)(n=3)和临床资料(5 例男性,4 例女性;平均年龄 26 岁)。记录特定的影像学表现,包括大小、偏心性、骨化、溶骨性、囊性变、膨胀性生长、骨膜反应、皮质破坏、软组织延伸和关节受累。

结果

6 例患者表现为疼痛,3 例患者表现为疼痛和可触及的肿块。影像学检查显示,近端胫骨(n=7)或股骨(n=2)上的主要溶骨性病变周围有一个边界不清的区域。7 例病例显示有限的骨化。3 例病例肿瘤位于干骺端,6 例病例位于骺端。平均最大肿瘤尺寸为 4.7cm×5.2cm×7.8cm。显微镜下,肿瘤由具有少量骨样形成和多核巨细胞的非典型细胞组成。所有患者均接受化疗,8 例患者接受手术治疗。3 例患者死亡,6 例患者在最后一次随访时仍存活。

结论

GCRO 是一种罕见的变体,与巨细胞瘤非常相似。患者通常表现为疼痛和可触及肿块等非特异性症状。它通常在影像学上显示出溶骨性病变,在干骺端和/或骺端有局部保留的新骨形成。组织学上,具有骨样形成和多核巨细胞的非典型肿瘤细胞是诊断和鉴别诊断的关键因素。

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