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[骨纤维发育不良及其鉴别诊断]

[Osteofibrous dysplasia and its differential diagnosis].

作者信息

Del Grande F, Exner G U, Hodler J

机构信息

Institut für Diagnostische Radiologie, Universitätsspital Zürich.

出版信息

Praxis (Bern 1994). 1999 Oct 21;88(43):1770-4.

Abstract

We present a case of a 13 year old girl with swelling and pain of the right lower leg persisting for four months. The radiological and histological diagnosis is osteofibrous dysplasia. Osteofibrous dysplasia is a rare bone lesion predominantly affecting the cortex of the tibia. Almost all patients are younger than ten years at presentation. Swelling is a typical clinical manifestation. Radiologically and even histologically the distinction between the osteofibrous dysplasia and the more aggressive adamantinoma can be difficult. Other bone lesions potentially mimicking osteofibrous dysplasia include: fibrous dysplasia, osteoid osteoma, osteosarcoma, osteoblastoma and intracortical Brodie's abcess. The natural course of the disease is unpredictable. Lesions with typical radiological appearance are considered to be "no touch lesions". In equivocal cases block resection (with both a diagnostic and therapeutic purpose) should be performed.

摘要

我们报告一例13岁女孩,右小腿肿胀疼痛持续4个月。放射学和组织学诊断为骨纤维发育不良。骨纤维发育不良是一种罕见的骨病变,主要累及胫骨皮质。几乎所有患者发病时年龄均小于10岁。肿胀是典型的临床表现。在放射学甚至组织学上,骨纤维发育不良与侵袭性更强的造釉细胞瘤很难区分。其他可能酷似骨纤维发育不良的骨病变包括:纤维发育不良、骨样骨瘤、骨肉瘤、骨母细胞瘤和皮质内布罗迪脓肿。该病的自然病程不可预测。具有典型放射学表现的病变被视为“无需处理的病变”。在不明确的病例中,应进行大块切除(兼具诊断和治疗目的)。

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