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颈部巨大黏液性纤维肉瘤:1例病例报告并文献复习

Giant myxofibrosarcoma of neck: A case report and review of the literature.

作者信息

Zhong Linkun, Zhang Ying, Kang Hongjian, Wang Zhaohua

机构信息

Department of General Surgery, Zhongshan City People's Hospital, Zhongshan, Guangdong Province, China.

Department of Otorhinolaryngology & Head and Neck Surgery, Dalian Municipal Friendship Hospital, Dalian, Liaoning Province, China.

出版信息

Medicine (Baltimore). 2025 Apr 18;104(16):e42150. doi: 10.1097/MD.0000000000042150.

DOI:10.1097/MD.0000000000042150
PMID:40258773
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12014053/
Abstract

RATIONALE

Myxofibrosarcoma (MFS) is a subtype of soft tissue sarcoma that commonly occurs in the extremities but is rare in the neck. It is characterized by a high risk of local recurrence due to its specific infiltrative growth pattern.

PATIENT CONCERNS

A 56-year-old male presented with a large mass in the right neck with disability of the right upper limb.

DIAGNOSES

Magnetic resonance imaging revealed a 12.48 × 9.01 × 14.16 cm mass with a "tail sign." Resection with wide margins was performed to remove the tumor and the invaded surrounding tissues. Histopathological examination confirmed the diagnosis of high-grade MFS.

INTERVENTIONS

The patient developed local recurrence 24 months after surgery and died of the disease 26 months after the recurrence.

OUTCOMES

This case presents the largest MFS located in the neck reported to date. The treatment of giant MFS in the neck is challenging due to the proximity of vital structures including vessels, nerves, and other tissues. The invasive pattern of MFS facilitates extensive spread into surrounding tissues, complicating complete surgical removal. And insufficient margins frequently result in disease recurrence.

LESSONS

This case highlights the significance of wide surgical margins in instances of extensive neck infiltration by MFS. However, further studies are required to confirm the specific margin values, and the advancement of effective adjuvant therapy is essential for improving patient survival.

摘要

理论依据

黏液纤维肉瘤(MFS)是软组织肉瘤的一种亚型,常见于四肢,但在颈部罕见。因其特定的浸润性生长模式,具有较高的局部复发风险。

患者情况

一名56岁男性,右颈部出现巨大肿块,并伴有右上肢功能障碍。

诊断

磁共振成像显示一个12.48×9.01×14.16厘米的肿块,伴有“尾征”。进行了广泛切缘的切除手术,以切除肿瘤及周围受侵组织。组织病理学检查确诊为高级别MFS。

干预措施

患者术后24个月出现局部复发,复发后26个月死于该疾病。

结果

该病例是迄今为止报道的位于颈部的最大MFS。由于颈部存在包括血管、神经和其他组织在内的重要结构,颈部巨大MFS的治疗具有挑战性。MFS的浸润模式使其易于广泛扩散至周围组织,导致完整手术切除复杂化。切缘不足常导致疾病复发。

经验教训

该病例凸显了在MFS广泛浸润颈部的情况下进行广泛手术切缘的重要性。然而,需要进一步研究以确定具体的切缘值,并且有效的辅助治疗进展对于提高患者生存率至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/71aeeec57a4f/medi-104-e42150-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/27b7c51b7ed5/medi-104-e42150-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/7abb5a3f4fb6/medi-104-e42150-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/7980abd72574/medi-104-e42150-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/71aeeec57a4f/medi-104-e42150-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/27b7c51b7ed5/medi-104-e42150-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/7abb5a3f4fb6/medi-104-e42150-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/7980abd72574/medi-104-e42150-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be6f/12014053/71aeeec57a4f/medi-104-e42150-g004.jpg

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本文引用的文献

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J Pathol. 2024 Nov;264(3):293-304. doi: 10.1002/path.6347. Epub 2024 Sep 11.
2
Clear surgical margins as a prognostic indicator for disease recurrence, with no impact on survival rates in patients with myxofibrosarcoma.切缘无肿瘤残留是黏液纤维肉瘤患者疾病复发的预后指标,对生存率无影响。
Sci Rep. 2024 May 28;14(1):12232. doi: 10.1038/s41598-024-63035-6.
3
Analysis of clinical factors impacting recurrence in myxofibrosarcoma.
分析影响黏液纤维肉瘤复发的临床因素。
Sci Rep. 2024 Feb 16;14(1):3903. doi: 10.1038/s41598-024-53606-y.
4
A novel patient-derived immortalised cell line of myxofibrosarcoma: a tool for preclinical drugs testing and the generation of near-patient models.一种新型的黏液纤维肉瘤患者源性永生化细胞系:一种用于临床前药物测试和近患者模型生成的工具。
BMC Cancer. 2023 Dec 6;23(1):1194. doi: 10.1186/s12885-023-11658-9.
5
The Biological Assessment of Shikonin and β,β-dimethylacrylshikonin Using a Cellular Myxofibrosarcoma Tumor Heterogeneity Model.紫草素和 β,β-二甲基丙烯酰紫草素的细胞粘液纤维肉瘤肿瘤异质性模型的生物学评估。
Int J Mol Sci. 2023 Nov 2;24(21):15910. doi: 10.3390/ijms242115910.
6
CORR Insights®: Is the Thickness of the Margin Associated With Local Recurrence and Survival in Patients With Myxofibrosarcoma?CORR见解®:黏液纤维肉瘤患者的切缘厚度与局部复发及生存相关吗?
Clin Orthop Relat Res. 2023 Nov 1;481(11):2137-2139. doi: 10.1097/CORR.0000000000002778. Epub 2023 Jul 14.
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The Prognostic Relevance of MRI Characteristics in Myxofibrosarcoma Patients Treated with Neoadjuvant Radiotherapy.新辅助放疗治疗黏液纤维肉瘤患者时MRI特征的预后相关性
Cancers (Basel). 2023 May 19;15(10):2843. doi: 10.3390/cancers15102843.
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Is the Thickness of the Margin Associated With Local Recurrence and Survival in Patients With Myxofibrosarcoma?黏液纤维肉瘤患者的边缘厚度与局部复发和生存有关吗?
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