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Ewing sarcoma of the thoracic epidural space in a young child

机译:幼儿胸腔硬膜外腔的尤文肉瘤

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PurposeTo report on the clinical course and treatment of Ewing sarcoma of the thoracic epidural space in a 5-year-old girl.MethodsWe present the case of a 5-year-old girl who experienced back pain (day 1); on day 10, the pain had exacerbated and involuntary movements in the lower limbs occurred, and an MRI performed in her local hospital revealed a tumor lesion at the upper thoracic level.ResultsOn day 13, emergency surgery was performed for partial resection of the tumor. Pathological examination of the resected tumor by immunostaining and gene testing revealed that it was MIC2 positive and an EWS-FLI 1 chimera, respectively, and Ewing sarcoma was diagnosed. The involuntary movements resolved immediately after the surgery. Three weeks after the operation, chemotherapy and radiation therapy were commenced. After 5?months, deep tendon reflexes recovered to normal. MRI showed that the tumor has not recurred at 29?months after surgery.ConclusionsThe majority of epidural patients undergo emergency surgery only after symptom exacerbation, which includes the development of neurological deficits. Thus, preoperative diagnosis of Ewing sarcoma of the epidural space is difficult and diagnosis is frequently made by a post-operative gene test. The resection area is limited to the intralesional margin area because a larger resection is difficult due to the characteristics of the affected region; thus, there is a higher possibility of recurrence and careful follow-up of the case is necessary...
机译:目的报道一名5岁女孩胸膜硬膜外腔尤因肉瘤的临床病程和治疗方法。方法:本病例为一名5岁女孩发生背痛的病例(第一天)。第10天,疼痛加剧,下肢出现不自主运动,在当地医院进行的MRI检查显示胸部上段有肿瘤病变。结果,第13天,进行了紧急手术以部分切除肿瘤。通过免疫染色和基因测试对切除的肿瘤进行病理检查,结果显示分别为MIC2阳性和EWS-FLI 1嵌合体,并诊断出尤因肉瘤。手术后,非自愿运动立即消失。术后三周,开始化学疗法和放射疗法。 5个月后,深层肌腱反射恢复正常。 MRI显示肿瘤在术后29个月没有复发。结论大多数硬膜外患者仅在症状加重后才进行急诊手术,其中包括神经功能缺损的发展。因此,难以对硬膜外腔的尤因肉瘤进行术前诊断,并且经常通过术后基因测试来进行诊断。切除区域被限制在病灶内边缘区域,因为由于受影响区域的特性,很难进行较大的切除;因此,复发的可能性更高,需要对病例进行认真的随访。

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