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上颌骨间叶性软骨肉瘤误诊为良性纤维病变:叙利亚的一例罕见病例报告

Mesenchymal chondrosarcoma of maxilla misdiagnosed as a benign fibrous lesion: A rare case report from Syria.

作者信息

Abdo Shouman Abdulrahman, Mohammed Kamilla A, Kaadeh Hadeel M, Haphian Yehia M, Hamzeh Mohamad M

机构信息

Faculty of Medicine, University of Aleppo, Aleppo, Syrian Arab Republic.

Faculty of Medicine, University of Aleppo, Aleppo, Syrian Arab Republic.

出版信息

Int J Surg Case Rep. 2025 Feb;127:110874. doi: 10.1016/j.ijscr.2025.110874. Epub 2025 Jan 12.

Abstract

INTRODUCTION

Mesenchymal chondrosarcoma (MC) is a high-grade variant of chondrosarcoma, essentially composed of poorly differentiated spindle cells interspersed with areas of cartilage or chondroid matrix. MC is extremely rare; it only accounts for 0.1 % of head and neck tumors and for only 1 % of all chondrosarcomas (CSs).

CASE PRESENTATION

A 21-year-old man presented with a medical history of a painful irritation at the dextral maxillary region, presented as a mass at the vestibule of the oral cavity near the upper molars, and had been misdiagnosed as a benign fibrous lesion and excised without performing a biopsy. Magnetic resonance imaging (MRI) revealed an invasive lesion filling the right maxillary sinus and penetrating the orbital floor. A biopsy was then performed and revealed an MC.

CLINICAL DISCUSSION

The patient underwent a wide surgical resection, except for the infraorbital region, in which the tumor was surrounded by a fibrous capsule separating it from the anatomical structures of the eye. Due to the lack of wide resection in the orbital floor area (to preserve the eyeball), we applied the chemotherapy that was done with cisplatin and doxorubicin.

CONCLUSION

Confirmed diagnosis by biopsy and treatment, both surgical and chemical, with frequent follow-up are decisive factors in progressing MC.

摘要

引言

间叶性软骨肉瘤(MC)是软骨肉瘤的一种高级别变异型,主要由分化差的梭形细胞组成,其间散在分布着软骨或软骨样基质区域。MC极为罕见;仅占头颈部肿瘤的0.1%,占所有软骨肉瘤(CS)的1%。

病例介绍

一名21岁男性,有右侧上颌区域疼痛性刺激的病史,表现为上颌磨牙附近口腔前庭的肿物,曾被误诊为良性纤维病变并在未进行活检的情况下切除。磁共振成像(MRI)显示一个侵袭性病变,充满右侧上颌窦并穿透眶底。随后进行活检,结果显示为MC。

临床讨论

患者接受了广泛的手术切除,但眶下区域除外,该区域肿瘤被纤维包膜包裹,与眼部解剖结构分隔。由于眶底区域缺乏广泛切除(以保留眼球),我们应用了顺铂和阿霉素进行化疗。

结论

通过活检确诊以及手术和化疗治疗,并频繁随访,是MC治疗进展的决定性因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ca1/11788735/a243e7b11519/gr1.jpg

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