一名患有李-弗劳梅尼综合征儿童的眼眶横纹肌肉瘤的治疗

Management of orbital rhabdomyosarcoma in a child with Li-Fraumeni syndrome.

作者信息

Jivraj Imran, Somers Gino R, Belliveau Michel J, Malkin David, DeAngelis Dan D

机构信息

Department of Ophthalmology, The Hospital for Sick Children, Toronto, Ontario.

Department of Pathology, The Hospital for Sick Children, Toronto, Ontario.

出版信息

J AAPOS. 2019 Jun;23(3):182-185. doi: 10.1016/j.jaapos.2019.01.013. Epub 2019 Apr 8.

DOI:10.1016/j.jaapos.2019.01.013

PMID:30974170

Abstract

This case highlights the management of orbital rhabdomyosarcoma in a child with Li Fraumeni syndrome (LFS). Treatment with chemotherapy and eventual orbital exenteration enabled margin-free control of the tumor. Radiation therapy was avoided to reduce the risk of inducing additional malignancy. Reactive orbital hyperostosis was observed postoperatively and was confirmed with surgical biopsy of the orbital roof. In this case, systemic surveillance imaging, which is necessary in patients with LFS, revealed an adrenal cortical carcinoma.

摘要

该病例突出了李-佛美尼综合征（LFS）患儿眼眶横纹肌肉瘤的治疗。化疗及最终的眼眶内容剜除术实现了肿瘤的无边缘控制。避免放疗以降低诱发其他恶性肿瘤的风险。术后观察到反应性眼眶骨质增生，并经眶顶手术活检证实。在该病例中，LFS患者必需的全身监测成像显示肾上腺皮质癌。