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颅底软骨肉瘤:一例报告。

Chondrosarcoma Skull Base: A Case Report.

机构信息

Department of Pathology, All India Institute of Medical Science, Jodhpur, Rajasthan, India.

Department of Neurosurgery, All India Institute of Medical Science, Jodhpur, Rajasthan, India.

出版信息

Curr Med Imaging. 2023;19(11):1346-1350. doi: 10.2174/1573405619666230207144546.

Abstract

INTRODUCTION

Chondrosarcomas are primary malignant bone tumor that rarely occurs in the head and neck region. Squash cytology of skull base neoplasm shows atypical chondrocytes and myxoid stroma, which suggests many possibilities like chordoma, chordoid glioma, chordoid meningioma and chondrosarcoma. Isocitrate dehydrogenase gene (IDH) mutations have been reported in 50% to 60% of the head and neck region chondrosarcoma.

CASE PRESENTATION

A 37-year-old female came to the outpatient department and complaint of difficulty in walking and swaying to the right side for one year. The radiology was suggestive of right-sided trigeminal schwannoma. However, the squash cytology showed the presence of necrosis, and pink to bluish-coloured myxoid stroma. The tumor cells were pleomorphic and had a hyperchromatic nucleus, hyalinized condensed to granular cytoplasm. The histopathological examination of intraoperative soft tissue showed the presence of cellular lobules of atypical chondrocytes arranged in the myxoid background. The features were of Chondrosarcoma. No parenchymal invasion was found.

CONCLUSION

This case report aims to create awareness about a rare tumor, which rarely forms a differential diagnosis for skull base neoplasms. As chondrosarcoma are immunoreactive to IDH1 so this marker can be useful in clinching the diagnosis in conjunction with other immunohistochemical markers in a small biopsy from skull base neoplasms.

摘要

简介

软骨肉瘤是一种罕见发生于头颈部的原发性恶性骨肿瘤。颅底肿瘤的挤压细胞学显示非典型软骨细胞和黏液样基质,提示许多可能性,如脊索瘤、脊索样胶质瘤、脊索样脑膜瘤和软骨肉瘤。异柠檬酸脱氢酶基因 (IDH) 突变已在 50% 至 60%的头颈部软骨肉瘤中报道。

病例介绍

一名 37 岁女性因行走困难和向右侧摇晃一年来到门诊部。影像学提示右侧三叉神经鞘瘤。然而,挤压细胞学显示存在坏死,以及粉红色到蓝紫色的黏液样基质。肿瘤细胞多形性,核深染,透明至颗粒状细胞质浓缩。术中软组织的组织病理学检查显示存在排列在黏液样背景中的非典型软骨细胞的细胞小叶。这些特征是软骨肉瘤。未发现实质侵犯。

结论

本病例报告旨在提高对一种罕见肿瘤的认识,这种肿瘤很少对头颈部肿瘤形成鉴别诊断。由于软骨肉瘤对 IDH1 呈免疫反应性,因此该标志物结合其他免疫组织化学标志物在颅底肿瘤的小活检中可以有助于明确诊断。

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