Primary epithelioid sarcoma of bone: report of a unique case, with immunohistochemical and fluorescent in situ hybridization confirmation of INI1 deletion.
Primary epithelioid sarcoma of bone: report of a unique case, with immunohistochemical and fluorescent in situ hybridization confirmation of INI1 deletion.
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原发性骨上皮样肉瘤：一个独特病例的报告，免疫组织化学和荧光原位杂交证实了 INI1 缺失。
DOI：
10.1097/pas.0b013e31819b92d5
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发表时间：
2009
期刊：
影响因子：
--
通讯作者：
A. Folpe
中科院分区：
文献类型：
--
作者：
D. Raoux;M. Péoc'h;F. Pedeutour;B. Vaunois;A. Decouvelaere;A. Folpe
我们报告的临床和病理特点，这是我们所知的，第一例上皮样肉瘤的骨。1例31岁女性，既往病史不明显，表现为骨盆疼痛，计算机断层扫描发现髂骨有5 cm破坏性、部分钙化的骨内病变。组织学上，肿瘤由相对均匀但明显呈恶性的上皮样细胞组成，并伴有散在的横纹肌样细胞。肿瘤细胞间可见玻璃样变至部分钙化的基质，呈“铁丝网”样钙化。免疫组化显示肿瘤细胞表达细胞角蛋白、波形蛋白、上皮膜抗原和CD34，而INI1蛋白表达完全缺失。荧光原位杂交显示INI1基因纯合性缺失。广泛的临床和放射学检查没有显示软组织肿瘤的证据，诊断为原发性骨上皮样肉瘤。在此之后，患者接受了肿瘤的完全切除术，目前在手术后6个月无疾病。这些极其罕见的肿瘤必须严格区分其他更常见的骨肿瘤，特别是成软骨细胞瘤和骨肉瘤。认识到上皮样肉瘤可能发生在骨，仔细的组织学评价和辅助免疫组化上皮标志物，CD34和INI1蛋白应允许识别这样的肿瘤。研究额外的情况下，原发性上皮样肉瘤的骨将是必要的，以更好地了解其临床行为。
We report the clinical and pathologic features of, what is to the best of our knowledge, the first case of epithelioid sarcoma of bone. A 31-year-old woman with an unremarkable past medical history presented with pelvic pain and was found by computed tomography scan to have a destructive 5 cm, partially calcified intraosseous lesion of the iliac bone. Histologically, the tumor consisted of relatively uniform but clearly malignant-appearing epithelioid cells, with scattered rhabdoid-appearing cells. A hyalinized to partially calcified matrix was present between the tumor cells, with a "chickenwire" pattern of calcification. By immunohistochemistry, the neoplastic cells expressed cytokeratins, vimentin, epithelial membrane antigen and CD34, and showed complete loss of INI1 protein expression. Fluorescence in situ hybridization showed homozygous deletion of the INI1 gene. An extensive clinical and radiographic workup did not show evidence of a soft tissue tumor, and the diagnosis of a primary epithelioid sarcoma of bone was made. After this, the patient underwent a complete resection of her tumor, and is currently disease free, 6 months after surgery. These extremely rare tumors must be rigorously distinguished from other more common tumors of bone, in particular, chondroblastoma and osteosarcoma. Awareness that epithelioid sarcoma may occur in bone, careful histologic evaluation and ancillary immunohistochemistry for epithelial markers, CD34 and INI1 protein should allow for recognition of such tumors. Study of additional cases of primary epithelioid sarcoma of bone will be necessary to better understand its clinical behavior.
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影响因子：
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作者：
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DOI：
--
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Kohashi K;Oda Y;et. al.
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DOI：
--
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期刊：
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影响因子：
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