关键词: image-guided radiation therapy malignant peripheral nerve sheet tumor radiation oncology radiotherapy sarcoma

来  源:   DOI:10.3389/fonc.2023.1129537   PDF(Pubmed)

Abstract:
UNASSIGNED: Malignant peripheral nerve sheath tumors (MPNSTs) are a group of rare soft tissue sarcomas of mesenchymal origin. These tumors generally require extensive local excision owing to their aggressive potential. Though the role of radiotherapy is controversial, in this report, we present the case of an MPNST in the forearm that was treated with microsurgery followed by image-guided radiation therapy to achieve complete tumor disappearance at the 18-month follow-up.
UNASSIGNED: A 69-year-old woman with underlying paranoid schizophrenia was referred to our department with pain, severe swelling, and ecchymosis of her right forearm. Physical examination showed hypoesthesia in the segments innervated by the median nerve and reduced motor strength of her right hand. A gadolinium-enhanced MRI showed a large malignant peripheral nerve sheath tumor (13 x 8 x 7 cm) of the median nerve in the forearm. She underwent microsurgical en-bloc tumor resection with sparing of the median nerve. Thirty-five days postoperatively, she underwent image-guided radiotherapy (IGRT) using volumetric modulated arc therapy (VMAT). Serial MRI scans of the forearm with Gadolinium and whole-body CT scan with contrast enhancement at 30 days, 6 months, 1 year, and 18 months postoperatively documented no tumor recurrence, remnants, or metastases.
UNASSIGNED: In this report, we demonstrate the successful use of advanced radiotherapy techniques such as IGRT while avoiding demolitive surgery for MPNST. Though a longer follow-up is necessary, at the 18-month follow-up, the patient demonstrated good outcomes from surgical resection followed by adjuvant RT for MPNST in the forearm.
摘要:
恶性外周神经鞘瘤(MPNSTs)是一组罕见的间充质起源的软组织肉瘤。这些肿瘤通常需要广泛的局部切除,因为它们具有侵袭性。虽然放射治疗的作用是有争议的,在这份报告中,我们介绍了前臂MPNST的病例,在18个月的随访中,我们采用显微外科手术,然后进行影像引导放射治疗,以实现肿瘤完全消失.
一名69岁女性患有潜在的偏执型精神分裂症,因疼痛被转诊到我们部门,严重肿胀,和她的右前臂瘀斑.体格检查显示,正中神经支配的节段感觉减退,右手的运动强度降低。钆增强MRI显示前臂正中神经有一个大的恶性周围神经鞘瘤(13x8x7cm)。她接受了显微外科整块肿瘤切除术,保留了正中神经。术后35天,她接受了使用体积调制电弧治疗(VMAT)的图像引导放射治疗(IGRT).连续MRI扫描前臂与钆和全身CT扫描与对比增强在30天,6个月,1年,术后18个月没有肿瘤复发,残余物,或转移。
在本报告中,我们证明了IGRT等先进放疗技术的成功应用,同时避免了MPNST的脱模性手术.虽然需要更长时间的随访,在18个月的随访中,该患者在前臂手术切除后接受MPNST辅助放疗后表现良好.
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