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一名患有牙列不齐的11岁男孩下颌骨尤文肉瘤的部分手术切除。

Partial surgical excision of ewing sarcoma in the lower mandible of an 11-year-old boy with dental malocclusion.

作者信息

Zamani Saba, Faraji Navid, Mokarram Mohammadreza, SadighBatha Samira

机构信息

Dental Students' Research Committee, Department of Pediatric Dentistry, School of Dentistry, Isfahan University of Medical Sciences, Isfahan, Isfahan Province, Iran.

Department of Medical-Surgical Nursing, School of Nursing and Midwifery, Urmia University of Medical Sciences, Nursing and Midwifery Faculty, Campus Nazlu, Urmia, West Azerbaijan, Iran.

出版信息

Ann Med Surg (Lond). 2025 May 30;87(7):4666-4671. doi: 10.1097/MS9.0000000000003445. eCollection 2025 Jul.

Abstract

INTRODUCTION AND IMPORTANCE

Ewing sarcoma (ES) is an uncommon and aggressive tumor that primarily affects children and adolescents, peaking in incidence between the ages of 10 and 20. Although it most frequently occurs in long bones and the pelvis, its presence in the lower mandible is extremely rare, creating notable challenges in both diagnosis and treatment.

CASE PRESENTATION

This report details an 11-year-old boy from a low-income background who was diagnosed with Ewing sarcoma localized to the lower mandible. Initially, he presented with ongoing pain and swelling, which were mistakenly attributed to dental problems, resulting in a delayed diagnosis. Imaging studies identified a permeative bone lesion, which was confirmed through biopsy to be Ewing sarcoma with the EWSR1-FLI1 fusion gene. The patient underwent partial surgical excision followed by a chemotherapy regimen. Unfortunately, he developed neutropenia and septic shock during treatment, leading to his eventual death.

CLINICAL DISCUSSION

This case highlights the diverse clinical presentations of Ewing sarcoma, stressing the necessity for heightened awareness of malignant conditions in pediatric patients. It also discusses the socioeconomic factors that can delay diagnosis, underscoring the importance of raising awareness in underserved areas. A multidisciplinary treatment approach is essential, along with careful monitoring for complications.

CONCLUSION

This case exemplifies the challenges in diagnosing and managing Ewing sarcoma in atypical sites such as the mandible. It emphasizes the need for early symptom recognition, addressing socioeconomic disparities and implementing comprehensive care strategies to enhance patient outcomes in pediatric oncology.

摘要

引言与重要性

尤因肉瘤(ES)是一种罕见且侵袭性强的肿瘤,主要影响儿童和青少年,发病高峰年龄在10至20岁之间。尽管它最常发生于长骨和骨盆,但在下颌骨中极为罕见,这给诊断和治疗带来了显著挑战。

病例介绍

本报告详细描述了一名来自低收入家庭的11岁男孩,他被诊断为局限于下颌骨的尤因肉瘤。最初,他出现持续疼痛和肿胀,被误诊为牙齿问题,导致诊断延迟。影像学检查发现一个浸润性骨病变,经活检证实为伴有EWSR1-FLI1融合基因的尤因肉瘤。患者接受了部分手术切除,随后进行化疗。不幸的是,他在治疗期间出现了中性粒细胞减少和感染性休克,最终死亡。

临床讨论

该病例突出了尤因肉瘤多样的临床表现,强调了提高对儿科患者恶性疾病认识的必要性。它还讨论了可能导致诊断延迟的社会经济因素,强调了在医疗服务不足地区提高认识的重要性。多学科治疗方法至关重要,同时要仔细监测并发症。

结论

该病例例证了在诸如下颌骨等非典型部位诊断和管理尤因肉瘤的挑战。它强调了早期症状识别、解决社会经济差异以及实施全面护理策略以改善儿科肿瘤患者预后的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0682/12369705/c2a7d7e4c33a/ms9-87-4666-g001.jpg

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