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一名青少年男性患者的肺泡横纹肌肉瘤——病例报告及当前观点

Alveolar rhabdomyosarcoma in an adolescent male patient - case report and current perspectives.

作者信息

Pop Daniel Laurenţiu, Nodiţi Gheorghe, Abu-Awwad Ahmed, Maliţa Daniel Claudiu, Zamfir Carmen Lăcrămioara, Grigoraş Mirela Loredana, Vermeşan Dinu, Prejbeanu Radu, Hărăguş Horia George, Boşcu Anca Laura, Ciupe Bogdan Cătălin, Deleanu Bogdan Nicolae, Faur Cosmin Ioan, Folescu Roxana

机构信息

Department of Orthopedics and Traumatology, "Dr. Pius Brînzeu" Clinical Hospital, "Victor Babeş" University of Medicine and Pharmacy, Timişoara, Romania;

出版信息

Rom J Morphol Embryol. 2018;59(4):1247-1252.

Abstract

Alveolar rhabdomyosarcoma (RMS) is a common pediatric malignant mesenchymal tumor, representing half of soft tissue sarcomas and approximately 5% of all cancers. We present the case of an adolescent male patient treated in our Department for a tumoral mass located in the middle third of the forearm. Magnetic resonance imaging (MRI) and angiography-computed tomography (angio-CT) showed a large mass located in the muscles of the anterior compartment of the forearm. Surgical treatment consisted of tumor ablation including segmental resection of the radial and ulnar arteries and of the median nerve, followed by saphenous autograft vascular bypass. The treatment plan was based on tumor type, histological grading (high), age, tumor size greater than 5 cm, unfavorable location, postoperative tumor, node, metastasis (TNM) stage II, presence of microscopic tumoral tissue in the margins of the resected piece, lymph node metastases (N1) and bone metastases (M1) found on positron-emission tomography (PET)-CT according to the German soft tissue sarcoma study (CWS)-IV 2002 protocol. The chemotherapy used Carboplatin and Topotecan. Survival was less than two years after the initial presentation. Adolescent extremity masses should raise suspicion to exclude serious malignancy. Despite early diagnosis and use of multimodal therapies, alveolar RMS prognostic remains unpredictable.

摘要

肺泡状横纹肌肉瘤(RMS)是一种常见的儿童恶性间叶性肿瘤,占软组织肉瘤的一半,约占所有癌症的5%。我们报告了一例青少年男性患者,在我院接受治疗,其肿瘤位于前臂中三分之一处。磁共振成像(MRI)和血管造影计算机断层扫描(血管造影CT)显示在前臂前室肌肉中有一个大肿块。手术治疗包括肿瘤切除,包括桡动脉、尺动脉和正中神经的节段性切除,随后进行自体大隐静脉血管搭桥。治疗方案基于肿瘤类型、组织学分级(高)、年龄、肿瘤大小大于5 cm、位置不佳、术后肿瘤、淋巴结、转移(TNM)II期、切除标本边缘存在微小肿瘤组织、根据2002年德国软组织肉瘤研究(CWS)-IV方案在正电子发射断层扫描(PET)-CT上发现的淋巴结转移(N1)和骨转移(M1)。化疗使用卡铂和拓扑替康。初次就诊后生存期不到两年。青少年肢体肿块应引起怀疑,以排除严重恶性肿瘤。尽管早期诊断并采用了多模式治疗,但肺泡状RMS的预后仍然难以预测。

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