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纤维发育不良中的恶性变(6例报告及文献复习)

Malignant degeneration in fibrous dysplasia (presentation of 6 cases and review of the literature).

作者信息

Campanacci M, Bertoni F, Capanna R

出版信息

Ital J Orthop Traumatol. 1979 Dec;5(3):373-81.

PMID:298846
Abstract

Malignant degeneration of fibrous dysplasia is rare. It occurs with similar frequency at all ages and in both sexes. It is more frequent in cases of polyostotic than in monostotic fibrous dysplasia. In cases of fibrous dysplasia that do show malignant degeneration it is common to find that a high level of alkaline phosphatase persists in the serum, even in adults. Previous radiotherapeutic treatment appears to me a predisposing factor. Osteosarcoma is the most frequent neoplasm, followed at some distance by fibrosarcoma and chondrosarcoma. The tumour is most often localised in the femur; it is not unusual to find it in the tibia, maxilla and mandible. The treatment and prognosis are the same as those of the involved malignant neoplasm.

摘要

骨纤维异常增殖症的恶性变很少见。其在各年龄段和男女中的发生率相似。多骨型骨纤维异常增殖症比单骨型更易发生恶性变。在确实发生恶性变的骨纤维异常增殖症病例中,即使在成年人中,血清碱性磷酸酶水平持续升高也很常见。在我看来,既往的放射治疗似乎是一个诱发因素。骨肉瘤是最常见的肿瘤,其次是纤维肉瘤和软骨肉瘤,二者发生率相对较低。肿瘤最常发生于股骨;也常见于胫骨、上颌骨和下颌骨。其治疗和预后与相关恶性肿瘤相同。

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On malignant transformation in fibrous dysplasia of bone.
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