【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

9 7月
HistoryA teenager presents with arm pain.患者为青少年,手臂疼痛。

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

1.There is ground-glass bone density(骨骼磨玻璃密度).

 骨骼磨玻璃密度

True

False

2.There is bony expansion(骨质膨胀).

True

False

3.Chondroid matrix(软骨基质) is present.

True

False

4.There is periosteal reaction(骨膜反应).

True

False

5.Which choice is a likely diagnosis for the humerus?

Juvenile Paget\’s disease 青少年佩吉特氏病

Fibrous dysplasia 骨纤维结构不良

Osteomyelitis 骨髓炎

Ewing\’s sarcoma 尤文氏肉瘤

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

Additional Images

An additional image of the elbow-forearm is shown below along with the humerus image.

下图示:肱骨摄片时可见前臂异常密度。

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

6.What is the diagnosis for the finding(s) involving the proximal forearm?

 前臂近段的异常密度该如何诊断?

Fibrous dysplasia with secondary aneurysmal bone cyst

骨纤维结构不良继发动脉瘤样骨囊肿

Fibrous dysplasia with secondary giant cell tumor

骨纤维结构不良继发骨巨细胞瘤

Unicameral bone cyst

单房性骨囊肿

Fibrous dysplasia with chondroblastoma

骨纤维结构不良合并成软骨细胞瘤

Secondary osteosarcoma

继发性骨肉瘤

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

Follow-up Radiograph

A follow-up radiograph of the elbow is shown below.

复查X线片。

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

7.Which choice most likely characterizes the findings?

 图中所示应考虑下列哪项?

Interval healing 愈合期

Malignant transformation 恶变

Secondary osteomyelitis 继发骨髓炎

Iatrogenic change 医源性改变

None of the above

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

选择题答案:

  1. True
  2. True
  3. False
  4. False
  5. Fibrous dysplasia
  6. Fibrous dysplasia with secondary aneurysmal bone cyst
  7. Iatrogenic changeSlight interval enlargement of lesion consistent with curettage. Increased density consistent with packing

    随年龄增长,病灶体积略增大。病灶内高密度为填充物。

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

Findings and Diagnosis

FindingsThere is mild expansion and ground-glass opacity of the humerus and radius metadiaphysis consistent with fibrous dyaplasia. There is a roughly 1-cm, round lytic lesion in the proximal radius.肱骨轻度膨胀,呈磨玻璃密度改变,桡骨干骺端及骨干也可见异常密度,符合骨纤维结构不良。桡骨近端可见类圆形溶骨性结节,直径约1cm。

Differential Diagnosis

  • Secondary aneurysmal bone cyst 继发动脉瘤样骨囊肿
  • Giant cell tumor 骨巨细胞瘤
  • Osteoblastoma 成骨细胞瘤
  • Unicameral bone cyst 单房性骨囊肿
  • Osteosarcoma 骨肉瘤
  • Metastasis 转移瘤
DiagnosisPolyostotic fibrous dysplasia with secondary aneurysmal bone cyst多骨性骨纤维结构不良继发动脉瘤样骨囊肿

【双语病例】骨纤维结构不良继发动脉瘤样骨囊肿1例

Key Points

  • Aneurysmal bone cyst (ABC) is an expansile, blood-filled, cystic lesion.动脉瘤样骨囊肿(ABC)为膨胀性、囊性病变,内可充满血液。
  • Although benign, it can be locally aggressive and cause bone weakening or destruction.ABC为良性为良性病变,但可具有局部侵袭性,引起骨质吸收或破坏。
  • Symptoms: Pain increasing for weeks/months, enlarging mass, pathologic fracture. ABCs in the spine can cause neurologic symptoms.主要症状:数周或数月内疼痛加剧,肿块体积增大,病理性骨折。脊柱ABC可引起神经系统症状。
  • Disease of the young; 70% occur in people younger than 20 years old.发病年龄较小,约70%的患者不到20岁。
  • Location: Long bones 80% > pelvis > posterior elements of spine > hands 10% metaphysis 80%, diaphysis 20%.位置:四肢长骨(80%)>脊柱后部>手(10%)。其中80%发生于干骺端,20%发生于骨干。
  • Etiology of ABCs is unknown. Is felt to be a vascular malformation that forms within bone either 1) de novo 2) in an area of prior trauma 3) as a reaction to another bony lesion (MC = giant cell tumor; however, various other benign and malignant tumors are also found — i.e., fibrous dysplasia, nonossifying fibroma, sarcoma, etc.).动脉瘤样骨囊肿的病因尚不完全明确,可能与血管畸形有关,可能为自发性,也可能继发于外伤、或其他病变(最常见的是巨细胞瘤,也有可能是骨纤维结构不良、非骨化性纤维瘤、肉瘤等多种良恶性病变)。
  • Imaging findings: Multicystic eccentric lesion with expansion of surrounding bone (“soap-bubble” appearance) ± septations.影像学表现:偏心性多囊性病变,周围骨质膨胀(皂泡样),内可伴有分隔。
  • MRI may show blood in the lesion and expansion of soft tissues.MRI可见病灶内血性信号,软组织膨胀。
  • Bone scan: “Donut sign” — increased peripheral uptake, photopenic center.骨扫描:“甜甜圈征”——周边摄取增高,中央摄取稍低。
  • Phases of progression: Initial (small, no expansion, little erosion), rapid growth (destructive, massive bone lysis, periosteal growth cannot keep up so no reaction), stable (classic appearance above), healing (progressive ossification of septae, forms an irregular trabeculated mass).病变的进展过程:初始期(病灶小,无明显膨胀、侵蚀),迅速增强期(骨质破坏、溶骨性肿块、无明显骨膜反应),稳定期(可见上述的典型表现),愈合期(骨性分隔逐渐进展,形成含有小梁结构的不规则肿块)。
  • Recurrence in 10% to 20% of cases. No malignant transformation.约10%-20%的患者可复发,但不会恶变。
  • Biopsy must be performed if there is any doubt of the diagnosis because of the strong association with giant cell tumor.由于该病与巨细胞瘤高度相关,所以如果诊断有疑问,需进行活检。
  • Treatment: Arterial embolization versus surgical curettage/excision; wide resection for lesions with extensive bony destruction治疗:动脉栓塞或手术刮除/切除;有广泛骨质破坏的病变需广泛切除。

发表评论

您的电子邮箱地址不会被公开。 必填项已用*标注