DSRCT

DSRCT
  • 文章类型: Case Reports
    一名13岁男孩入院,有1个月的颈部疼痛史和2周的双侧髋关节疼痛史,伴有低热。正电子发射断层扫描-计算机断层扫描(PET-CT)显示左肾存在恶性肿瘤,并转移到左肾门,腹膜后,主动脉旁淋巴结,以及遍布全身的多个骨骼部位。鉴于患者的左肾囊完整且与周围组织的边界清晰,进行左肾切除术。术后病理诊断为左肾促纤维增生性小圆细胞瘤(DSRCT)。第一阶段手术后20天给予CAV-VIP交替化疗。在第6个周期结束后,病人再次接受了手术。主动脉和后静脉前方的肿瘤,大网膜,腹膜后淋巴结和肝门淋巴结,腹部可见的肿瘤被切除.第二阶段手术后继续进行CAV-VIP交替化疗。在第4周期术后化疗结束时,开始放疗。第二阶段手术后11个月进行的腹部CT扫描未发现腹部肿瘤有任何复发;但是骨转移仍然存在。患者目前正在接受安洛替尼的口服靶向治疗,同时持续随访。
    A 13-year-old boy was admitted to the hospital with 1-month history of neck pain and a 2-week history of bilateral hip joint pain accompanied by low fever. Positron emission tomography-computed tomography (PET-CT) revealed the presence of a malignant tumor in the left kidney with metastases to the left renal hilum, retroperitoneum, para-aortic lymph nodes, and multiple bone sites throughout the body. Given that the patient\'s left kidney capsule was intact and the boundary with surrounding tissues was clear, left nephrectomy was performed. Postoperative pathological diagnosis showed desmoplastic small round cell tumor (DSRCT) of the left kidney. CAV-VIP alternating chemotherapy was given 20 days after the first stage surgery. After the end of the 6th cycle, the patient underwent surgery again. The tumor in front of the aorta and postcava, the greater omentum, the retroperitoneal lymph nodes and the hepatic hilum lymph nodes, and the visible tumors in the abdomen were removed. CAV-VIP alternating chemotherapy was continued after the second stage surgery. At the end of the 4th cycle of post operation chemotherapy, radiotherapy was started. An abdominal CT scan conducted 11 months after second-stage surgery did not reveal any recurrence of abdominal tumors; however bone metastases persisted. The patient is currently receiving oral targeted therapy with anlotinib while ongoing follow-up continues.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    未经证实:促纤维化小圆细胞瘤(DSRCT)是一种罕见的,侵略性,一个单独的临床病理实体的间充质恶性肿瘤。它对年轻人有偏爱,没有任何种族偏好的证据.目前DSRCT的诊断金标准包括组织病理学,免疫组织化学,和细胞遗传学研究,以确认可变的表型表达和特征性染色体易位。
    未经证实:一名65岁男子出现腹部肿块感和不完全性肠梗阻。除碳水化合物抗原外,初始实验室测试均在正常范围内。对比增强CT显示,在中腹壁和下腹壁的大网膜区域占据了质量混杂密度。对图像进行3D重建以阐明肿瘤与结肠之间的关系,并通过结肠镜检查进行确认。手术后,免疫组织化学和荧光原位杂交(FISH)显示EWSR1-WT1基因在22q12重排,证实了纤维增生性小圆细胞肿瘤的诊断。
    未经批准:与DSRCT对年轻人的偏爱不同,在我们的病例中,患者是一名65岁的男性,其巨大的肿块累及横结肠和膀胱。
    UNASSIGNED: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, mesenchymal malignancy of a separate clinicopathological entity. It has a predilection for young men, with no evidence of any ethnic predilection. The current diagnostic gold standard for DSRCT includes histopathologic, immunohistochemical, and cytogenetic studies in order to confirm the variable phenotypic expression and characteristic chromosomal translocation.
    UNASSIGNED: A 65-year-old man presented with a sensation of an abdominal mass and a presentation of an incomplete bowel obstruction. Initial lab tests were in the normal range except for carbohydrate antigen. Contrast-enhanced CT showed that a large, mass-confounding density was occupied in the omentum majus area of the middle and lower abdominal wall. A 3D reconstruction of the images was performed to clarify the relationship between the tumor and the colon and was confirmed by a colonoscopy. After surgery, immunohistochemistry and fluorescence in situ hybridization (FISH) revealed EWSR1-WT1 gene rearrangement at 22q12, confirming the diagnosis of desmoplastic small round cell tumor.
    UNASSIGNED: Being different from the predilection of DSRCT for young men, the patient in our case is a 65-year-old man with a huge mass involving the transverse colon and the bladder.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

公众号