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Undifferentiated embryonal liver sarcoma in childhood: A case report

机译:儿童未分化胚胎肝肉瘤1例

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In order to improve the diagnosis and therapy of undifferentiated embryonal liver sarcoma (UELS), the present study presents the case of a 9-year-old female with UELS and discusses UELS in childhood. The patient presented with abdominal pain and fever. The laboratory tests, radiographic examination and pathological features presented by the female were similar to those of typical cases of UELS reported in childhood. The patient initially received surgical treatment and the immunohistochemical findings suggested that the patient had UELS. The patient's parents refused adjuvant chemotherapy and demonstrated a right prerenal mass 6 months post-surgery. Microscopic examination revealed that the tumor was evidence of undifferentiated embryonal sarcoma recurrence. However, the patient was comfortable and physical examination revealed no abnormal conditions. In addition, the laboratory results were normal. Abdominal computed tomography scan and ultrasound were performed every 3 months to monitor the tumor recurrence. At the time of writing, it has been 6 months after the second surgical procedure and there has been no appearence of abnormalities. Previous studies have shown that patients who receive combined therapy with complete tumor resection and adjuvant chemotherapy have a longer survival time than those who undergo surgical therapy alone. Complete tumor resection combined with adjuvant chemotherapy may reduce the risk of recurrence and enhance the survival time in patients with UELS. as in young adults (1-3). Patients with UELS usually present with a painful right upper quadrant mass, fever and other symptoms, including weight loss, vomiting, nausea, anorexia and jaundice (4-6). Ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI) and biopsy are used as diagnostic tools to identify other liver tumors. However, the typical radiological findings of liver tumors are also observed in other liver diseases, including cystic hydatid disease (5). Previous studies have shown that complete tumor resection followed by adjuvant chemotherapy and/or radiation improves survival and reduces recurrence in patients with UELS, and this has now become a current standard therapy for UELS (7-9). The present study presents a childhood case of recurrent UELS that was treated with surgical resection without chemotherapy, and discusses the clinical characteristics, laboratory test results, immunohistochemical findings and treatment for this rare disease. Written informed consent was obtained from the parents of the patient.
机译:为了改善未分化的胚胎肝肉瘤(UELS)的诊断和治疗,本研究介绍了一名9岁女性患有UELS的病例,并讨论了儿童期UELS。该患者出现腹痛和发烧。由女性提供的实验室检查,射线照相检查和病理学特征与儿童期报告的UELS典型病例相似。该患者最初接受外科手术治疗,免疫组织化学结果表明该患者患有UELS。患者的父母拒绝接受辅助化疗,并在术后6个月表现出正确的肾前肿块。显微镜检查显示该肿瘤是未分化的胚胎肉瘤复发的证据。但是,患者感到舒适,体格检查未发现异常情况。另外,化验结果正常。每3个月进行一次腹部计算机断层扫描和超声检查,以监测肿瘤的复发情况。在撰写本文时,距第二次外科手术已经6个月了,并且没有出现异常。先前的研究表明,与单独接受手术治疗的患者相比,接受完全肿瘤切除和辅助化疗的联合治疗的患者生存时间更长。完全肿瘤切除结合辅助化疗可以降低UELS患者的复发风险并延长生存时间。和年轻人(1-3)一样。 UELS患者通常会出现疼痛的右上腹肿块,发烧和其他症状,包括体重减轻,呕吐,恶心,厌食和黄疸(4-6)。超声检查(US),计算机断层扫描(CT),磁共振成像(MRI)和活检被用作诊断工具,以识别其他肝肿瘤。但是,在其他肝病,包括囊状包虫病中也观察到了肝肿瘤的典型放射影像学发现(5)。先前的研究表明,完整的肿瘤切除术后再进行辅助化疗和/或放疗可提高UELS患者的生存率并降低其复发率,这已成为UELS的当前标准疗法(7-9)。本研究介绍了儿童期复发UELS的病例,该病例经手术切除而未进行化疗,并讨论了这种罕见疾病的临床特征,实验室检查结果,免疫组化结果和治疗方法。从患者的父母那里获得了书面知情同意书。

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