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具有“造釉细胞瘤样”特征的去分化软骨肉瘤:一例报告并文献复习

Dedifferentiated chondrosarcoma with "adamantinoma-like" features: A case report and review of literature.

作者信息

Gambarotti M, Righi A, Frisoni T, Donati D, Vanel D, Sbaraglia M, Dei Tos A P

机构信息

Surgical Pathology Department, Orthopaedic Institute, Bologna, Italy.

Surgical Pathology Department, Orthopaedic Institute, Bologna, Italy.

出版信息

Pathol Res Pract. 2017 Jun;213(6):698-701. doi: 10.1016/j.prp.2017.04.019. Epub 2017 May 6.

Abstract

Dedifferentiated chondrosarcoma is defined by the presence of a low grade malignant cartilaginous component juxtaposed to a high grade malignant non-cartilaginous sarcomatous components. Only 4 cases in which the high grade component showed epithelial differentiation have been reported in the literature; three featured a squamous and the one a glandular epithelial component. Here we describe a case of dedifferentiated chondrosarcoma exhibiting epithelial "adamantinoma-like" basaloid features. The patient underwent wide resection of the proximal tibia and post-operative chemotherapy and died 8 months after the diagnosis due to lung and bone metastases.

摘要

去分化软骨肉瘤的定义是存在一个低级别恶性软骨成分与一个高级别恶性非软骨肉瘤成分并列。文献中仅报道了4例高级别成分显示上皮分化的病例;3例具有鳞状上皮成分,1例具有腺上皮成分。在此,我们描述1例表现出上皮“造釉细胞瘤样”基底样特征的去分化软骨肉瘤病例。该患者接受了胫骨近端广泛切除术及术后化疗,诊断后8个月因肺和骨转移死亡。

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