12岁患者骨干中部罕见低度恶性中央骨肉瘤

骨科医师之家  2013年8月9日  阅读数:1559  字体:    

Central low-grade osteosarcoma with an unusual localization in the diaphysis of a 12-year old patient.

12岁患者骨干中部罕见低度恶性中央骨肉瘤

 

Background

Low-grade central osteosarcoma is a very rare subtype of osteosarcoma with a predilection for the metaphysis of long bones and a peak incidence in the 3(rd) decade of life. Absence of specific clinical symptoms and a good prognosis after wide resection are the characteristics of this entity. Chemotherapy is not indicated in this highly differentiated tumour.

背景

    低度恶性中央骨肉瘤是一种罕见的亚型骨肉瘤,常见于长骨干骺端,30岁左右为该病高发期。该实体瘤的特征为缺乏特异性临床症状,广泛切除后预后良好。该高分化肿瘤不宜用化学疗法进行治疗。

 

Case report

A 12-year old girl was referred to an orthopaedic tumour centre due to the increasing pain in the left diaphyseal femur when walking. The pain started 3 days prior to the presentation and did not persist at night or at rest. Additionally the patient herself detected a mass in the left femur. The clinical examination revealed a visible and palpable swelling of the femur. On exertion of pressure it was painful. The surrounding skin and the remaining examination were normal. There was no history of weight loss, fever, chills, fatigue or exhaustion. The patient had no relevant past medical history. Routine laboratory tests were within normal range.

病历报告

    12岁女孩被送往骨科肿瘤中心进行治疗,她走路时左股骨骨干疼痛不断加剧。送往医院3天前开始疼痛,但夜间或休息时无痛感。另外患者自己在其左股骨处发现一处肿块。临床检查发现左股骨处有一明显可触及股骨肿块。按压肿块时有疼痛感。周围皮肤及其它检查均正常。该患者无体重减轻、发烧、发冷、疲劳或疲惫症状,也无相关既往病史。常规实验室测试均在正常范围内。

Radiography showed an osseous expansion with lamellated periosteal reaction in the diaphysis of the left femur (Figure 1A, B). The MR scan showed in T1-weighed imaging in the mid of the left femur with contrast medium enhancement, lesion in the mid of the left femur. Cortical destruction was present. The size of the lesion was 12 cm (cranio-caudal) × 3,5 cm (sagittal) × 4 cm (transversal) (Figure 2A). Whole body bone scan showed the increased uptake of the tracer in the diaphysis of the left femur, but no evidence of further lesions (Figure 2B). Staging was negative. Radiological differential diagnoses included fibrous dysplasia, Ewing’s sarcoma and chronic osteomyelitis. Incisional biopsy was conducted from the lateral side. The biopsy specimen showed a hypocellular spindle-cell proliferation arranged in fascicles with mild cytologic atypia and only single mitotic figures. The tumour proliferation permeated surrounding bone structures and entrapped bony trabeculae (Figure 3A, B) Diagnosis of a low-grade central osteosarcoma of the diaphysis was made.

 

放射显影显示左股骨骨干处有骨质扩张并层状骨膜反应(图1 A, B)。造影增强并T1加权成像磁共振扫描左股骨中部,显示左股骨中部病变,皮质损坏。皮损大小为12 cm(轴向)×3.5 cm (纵向)× 4 cm (横向)(图2A)。全身骨骼扫描显示股骨骨干处示踪剂摄取增加,但未见进一步病变(图2 B)。分期显示阴性。放射性鉴别诊断包括骨纤维异常增殖症,尤因氏肉瘤和慢性骨髓炎。从横侧面进行切取活检。活检标本显示簇生梭形细胞低增生,并伴有轻微细胞学变异,细胞只进行单核分裂。肿瘤增殖扩散到周边骨结构及其包埋的骨小梁(图3A, B)。该患者因此被确诊为骨干处低度恶性中央骨肉瘤。
          


FINGURE 1A,B X-ray shows a 12 cm intraosseous expansion and lamellated periosteal reaction in the diaphysis of the left femur (anterio-posterior, lateral).

1A, B)X线显示左股骨骨干中12厘米长髓内扩张和层状骨膜反应(轴向侧面)。

FIGURE 2A T1 weighed MR imaging detects a hyperintense, contrast medium enhanced, lesion in the mid of the left femur. Cortical destruction can be seen. The biopsy tract can be seen on the lateral side.

2 (A) T1加权磁共振扫描成像,显示高信号、造影剂增强、左股骨中部病变;可见皮质损坏;侧面可见活检束。

 

 

FIGURE 2B Whole body bone scan showed increased uptake of the tracer in the diaphysis of the left femur (Tc-99m-3 phases bone scintigraphy).

2 (B) 全身骨骼扫描,显示股骨骨干示踪剂摄取增加(Tc-99m-3相骨闪烁显像)。

 

FIGURE 3A The tumour is composed of a well differentiated fibroblastic component entrapping bony trabeculae. The spindle cells are set in a collagenous matrix (H&E stain).

3A)该肿瘤由高度分化的成纤维细胞组成,该细胞包埋骨小梁。梭形细胞处于胶原基质中(H&E染色)。

FIGURE 3B On low power magnification long parallel seams of bone are surrounded by a hypocellular spindle cell stroma (H&E stain).

3B)低倍镜下伸长平行骨缝周边低增生梭形细胞基质(H&E染色)。

FIGURE 4 The X- ray shows the reconstruction of the femur with a vascularized fibula, a homologous allograft and a plate (11 months postoperatively).

4 X片显示股骨血供腓骨、同源异体及其骨板的重建(术后11个月)。

 

Conclusions

Considering the patient’s age and the diaphyseal location in the presented case, these features are very uncommon within an already extremely rare tumour entity. A constellation like this makes it difficult to obtain a correct diagnosis, which can only be achieved by a multidisciplinary approach, including radiology, pathology and orthopaedics. When misdiagnosed or mistreated the patient is set at risk to develop high-grade osteosarcoma and metastasis. Unnecessary exposition to chemo- or radiotherapy has to be avoided. Therefore, one should be aware of this rare subtype of osteosarcoma and LGCOS should be considered as a differential diagnosis.

结论

   考虑到患者年龄及骨干位置, 以上特点在极罕见的肿瘤实体中更为少见。这些特征使得该病很难正确诊断,必须通过放射学、病理学和整形外科等多学科结合。若误诊或处理不当,患者可能发展为高级骨肉瘤,并有转移的危险。应避免不必要的化疗或放疗。因此,人们应对这种罕见的亚型骨肉瘤充分认识,并用LGCOS作为一种鉴别诊断方法。

责任编辑:马黎,王影影