Primary Epithelioid Sarcoma of the Zygomatic Bone
Primary Epithelioid Sarcoma of the Zygomatic Bone
复制标题
颧骨原发性上皮样肉瘤
DOI：
10.2350/16-03-1798-cr.1
复制
发表时间：
2016
影响因子：
1.9
通讯作者：
Shengmei Zhou
中科院分区：
文献类型：
--
作者：
H. Tran;Nick Shillingford;Stefanie M Thomas;J. Hammoudeh;Shengmei Zhou
原发性骨上皮样肉瘤是一种非常罕见的肿瘤，在英国文献中仅有2例报告。一名先前健康的18岁男子提出了6个月的历史，右侧面部麻木和刺痛和右眼复视。计算机断层扫描显示一个边界不清的肿块，致密的骨基质集中在右侧颧骨。外部活检被解读为骨肉瘤。切除标本显示大上皮样细胞和梭形细胞嵌入一个突出的透明化基质与局灶性化生骨形成。肿瘤细胞AE 1/AE 3和上皮膜抗原呈强阳性和弥漫阳性，但由于肿瘤细胞存在致密的透明化基质和SAT 2B弱表达，不能立即明确诊断ES。免疫组化显示INI 1蛋白表达不足，染色体微阵列分析显示SMARCB 1基因纯合缺失，最终证明该肿瘤为ES。
Primary epithelioid sarcoma (ES) of bone is extremely rare with only 2 reported cases in the English literature. A previously healthy 18-year-old man presented with a 6-month history of right facial numbness and tingling and right eye diplopia. A computerized tomography scan revealed an ill-defined mass with dense osseous matrix centered in the right zygomatic bone. An outside biopsy was read as osteosarcoma. The resection specimen revealed large epithelioid and spindle cells embedded in a prominent hyalinized matrix with focal metaplastic bone formation. The tumor cells were strongly and diffusely positive for AE1/AE3 and epithelial membrane antigen, but a definitive diagnosis of ES was not immediately reached due to the presence of dense hyalinized matrix and weak expression of SAT2B by tumor cells. Deficient INI1 protein expression by immunohistochemistry and homozygous loss of the SMARCB1 gene by chromosomal microarray analysis ultimately justified this tumor’s designation as ES.
影响因子：
5.8
作者：
Sakharpe, Aniket;Lahat, Guy;Lev, Dina
通讯作者：
Lev, Dina