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腹膜后肺泡状横纹肌肉瘤侵入椎管:一例报告并文献复习

Retroperitoneal alveolar rhabdomyosarcoma intruding into spinal canal: A case report and literature review.

作者信息

Zhang Yongbai, Huang Wenpeng, Li Liming, Qiu Yongkang, Jiao Hao, Chen Zhao, Yang Qi, Song Lele, Kang Lei

机构信息

Department of Nuclear Medicine, Peking University First Hospital, Beijing, China.

Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.

出版信息

Front Med (Lausanne). 2022 Nov 3;9:1019964. doi: 10.3389/fmed.2022.1019964. eCollection 2022.

Abstract

BACKGROUND

Rhabdomyosarcoma (RMS) is the most frequent soft sarcoma in children and adolescents. Alveolar rhabdomyosarcoma (ARMS) is a relatively rare subtype that is characterized by aggressive behavior and an unsatisfactory prognosis. An ARMS can arise anywhere but most commonly occurs at extremity sites with a very small fraction in the retroperitoneum. The utility of 2-Deoxy-2-[fluorine-18]-fluoro-D-glucose (F-FDG) positron emission tomography combined with computed tomography (PET/CT) remains to be established in ARMS.

CASE REPORT

A 3-year-old female child was accidentally found with a large left upper abdominal mass for a day. CT examination indicated a huge soft tissue mass in the left retroperitoneum extending superiorly to the level of the left hilus renalis and inferiorly to the left acetabulum in the pelvic cavity, with intrusion into the lumbar foramens. F-FDG PET/CT found a mass in the left retroperitoneum from the level of T12 to the left acetabulum, with the maximum standardized uptake value (SUV) of about 7.0, and a CT value of about 39 HU, invading the left L3-5 intervertebral foramina and protruding into the spinal canal, with unclear boundary with the spinal cord. Retroperitoneal tumor resection and the repair operation of vascular exploration were performed. An ARMS was confirmed by postoperative biopsy, immunohistochemical staining, and genetic detection with the rupture of the fork head in rhabdomyosarcoma (FKHR). The patient received chemotherapy and was in a good condition with no recurrence and obvious complications.

CONCLUSION

Retroperitoneal ARMS is rare and indicates a poor outcome with the potential to involve vital organs and intrude into the spinal canal. Accurate diagnosis and staging using PET/CT would contribute to better risk stratifications and appropriate treatment individually.

摘要

背景

横纹肌肉瘤(RMS)是儿童和青少年中最常见的软组织肉瘤。肺泡状横纹肌肉瘤(ARMS)是一种相对罕见的亚型,其特点是侵袭性强且预后不佳。ARMS可发生于任何部位,但最常见于四肢,仅有极小一部分发生于腹膜后。2-脱氧-2-[氟-18]-氟-D-葡萄糖(F-FDG)正电子发射断层扫描联合计算机断层扫描(PET/CT)在ARMS中的应用价值仍有待确定。

病例报告

一名3岁女童偶然发现左上腹有一巨大肿块1天。CT检查显示左腹膜后有一巨大软组织肿块,向上延伸至左肾门水平,向下延伸至盆腔内左髋臼,侵入腰椎间孔。F-FDG PET/CT发现左腹膜后从T12水平至左髋臼有一肿块,最大标准化摄取值(SUV)约为7.0,CT值约为39 HU,侵犯左L3-5椎间孔并突入椎管,与脊髓边界不清。行腹膜后肿瘤切除术及血管探查修复手术。术后活检、免疫组化染色及基因检测证实为ARMS,伴有横纹肌肉瘤叉头蛋白(FKHR)断裂。患者接受化疗,目前情况良好,无复发及明显并发症。

结论

腹膜后ARMS罕见,预后不良,有可能累及重要器官并侵入椎管。使用PET/CT进行准确诊断和分期有助于更好地进行风险分层并实施个体化的适当治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ec1/9670135/87a1e490c69c/fmed-09-1019964-g0001.jpg

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