Adamantinoma of the Femur With High-Grade Epithelial and Sarcomatoid Components: Case Report and Review of the Literature

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AJSP-Reviews and Reports Pub Date : 2020-01-01 DOI:10.1097/PCR.0000000000000359

Nima Sharifai, R. Runyon, M. Friedman, C. Cipriano, J. Chrisinger

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引用次数: 1

Abstract

Adamantinomas are rare low-grade malignant bone tumors that are composed of epithelial cells set in an osteofibrous stroma. Osteofibrous dysplasia-like and classic forms are well known; however, sarcomatous transformation (dedifferentiation) is very rarely observed. We present the case of a 48-year-old woman who presented with a 5-cm proximal femoral lytic lesion at risk of pathologic fracture. Frozen-section evaluation demonstrated an intimate admixture of atypical squamous nests and spindle cells, consistent with metastatic sarcomatoid carcinoma, so prophylactic intramedullary nailing was completed. Postoperative clinical and radiologic evaluation failed to identify a primary carcinoma, and short-term follow-up demonstrated rapid tumor spread throughout the femur and thigh. The patient underwent hip disarticulation, and microscopic examination demonstrated areas of classic adamantinoma associated with both high-grade epithelial and sarcomatoid components. Diffuse metastatic spread was evident within seven months. Along with a description of this case, we review the clinical, radiographic, histologic, and immunophenotypic characteristics of the eight adamantinomas with high-grade sarcomatous transformation that have been previously reported in detail in the English literature, and discuss important considerations for differential diagnosis.

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具有高级别上皮和肉瘤样成分的股骨金刚烷瘤:病例报告和文献回顾

金刚素瘤是一种罕见的低级别恶性骨肿瘤，由骨纤维间质中的上皮细胞组成。骨纤维性发育不良样和典型形式是众所周知的;然而，很少观察到肉瘤转化(去分化)。我们提出的情况下，一个48岁的妇女谁提出了5厘米股骨近端溶解病变的病理性骨折的风险。冷冻切片显示非典型鳞状巢和梭形细胞的密切混合，与转移性肉瘤样癌一致，因此完成了预防性髓内钉治疗。术后临床和放射学评估未能确定原发癌，短期随访显示肿瘤迅速扩散至股骨和大腿。患者接受髋关节脱臼，显微镜检查显示典型的金刚素瘤区域与高级别上皮和肉瘤样成分相关。7个月内弥漫性转移扩散明显。随着本病例的描述，我们回顾了8例具有高级别肉瘤转化的金刚烷瘤的临床、影像学、组织学和免疫表型特征，这些特征在英国文献中已经有详细的报道，并讨论了鉴别诊断的重要注意事项。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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AJSP-Reviews and Reports PATHOLOGY-

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期刊介绍： Each issue of Pathology Case Reviews examines one vital theme in the field with peer-reviewed, clinically oriented case reports that focus on diagnosis, specimen handling and reports generation. Each theme-oriented issue covers both histopathologic and cytopathologic cases, offering a comprehensive perspective that includes editorials and review articles of the newest developments in the field, differential diagnosis hints, applications of new technologies, reviews of current issues and techniques and an emphasis on new approaches.