首页> 外文期刊>The Internet Journal of Pathology >A Retroperitoneal/omental tumor with characteristics of Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and desmoplastic small round cell tumor (DSRCT) with unique pathologic and immunohistochemical features, presented as a renal tumor
【24h】

A Retroperitoneal/omental tumor with characteristics of Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and desmoplastic small round cell tumor (DSRCT) with unique pathologic and immunohistochemical features, presented as a renal tumor

机译:腹膜后/大网膜肿瘤,具有尤因氏肉瘤/周围原始神经外胚层肿瘤(EWS / pPNET)和增生性小圆形细胞瘤(DSRCT),具有独特的病理学和免疫组化特征,表现为肾脏肿瘤

获取原文
获取外文期刊封面目录资料

摘要

We present a unique case of retroperitoneal/omental tumor with characteristics of Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and desmoplastic small round cell tumor (DSRCT) in a young adult. This highly aggressive neoplasm must be distinguished from other primitive tumors that require different therapy. The tumor presented as a large mass originating from the right kidney, an evolving as a perirenal mass invading the kidney. Multiple recurrences within the following four post-operative years demonstrated a tumor with a unique location and the coexpression of FL-1 and WT1. Immunohistochemistry of the tumor shows the known immunophenotypic overlap between EWS/pPNET and DSRCT. The capacity of simultaneous multidirectional phenotypical expression of the tumor may serve a support to the concept that EWS/pPNET and DSRCT form single group tumors. The patient died of disease four years post-operatively. The accurate distinction between these entities has clear prognostic and therapeutic implications. Introduction Ewing’s sarcoma/peripheral primitive neuroectodermal tumor constitutes a group of malignant small round cell tumors (SRCTs) of presumed neuroectodermal origin that occur most commonly in bone and soft tissues of children and young adults1. Accurate diagnosis of SRCTs is hindered by their significant morphologic and genetic overlap and complicated by their rarity. EWS/PNET is characterized by a balanced chromosomal translocation which generates a fusion transcript of the EWS gene and the Friend leukemia virus integration 1 (FLI-1). Cases of primary EWS/pPNET of the kidney2, retroperitoneum3, and omentum4have been sporadically reported in the literature. To our knowledge, the present case is the second case to be reported of EWS/pPNET observed in retroperitoneum/omentum as a multilocular cyst with intracystic hemorrhage and necrosis. The tumor was unique with its location, and the coexpression of FLI-1 and Wilms tumor–associated (WT1) tumor suppressor gene, which are diagnostic of EWS/pPNET5 and DSRCT6, respectively. Case report A 28 years-old man presented with a recent vague right lomber pain had a right nephrectomy in a community hospital of a small city with a final diagnosis of high grade, sarcomatoid renal cell carcinoma, with disseminated perirenal infiltration. Two years later, he was referred to admit to the urology department for further evaluation. The laboratory studies was within normal limits. MRI and CT scan demonstrated multiple mass located on the left lower quadrant of the abdomen; one was on the anterolateral aspect of the descending colon extending into the inferior aspect of the liver; the second mass was located retrocecally and anterior to the right iliac muscle, lateral to the the right psoas muscl, and the third one was anterior to iliac artery bifurcation, and next to the right psoas muscle. They showed internal cystic degenerations, were lobulated, necrotic and highly hemorrhagic. Excision of the tumor was followed by four cycles of polychemotherapy (gemcitabine and cysplatin) which was switched to phosphamide and doxorubicine following a follow-up CT scan. Six months later CT scan was demonstrated disease progression. Abdominal ultrasonography (USG) and the CT scan was demonstrated a huge cystic mass with lobulated contours, measuring 24 cm in cranio-caudal dimension. There was a large, hemorrhagic and cystic tumor inferior to the liver, involving omental tissue and the right psoas muscle. Six months later he was readmitted and the omental mass was excised, a left double–j catheter was inserted. Three months later, subhepatic and mesenteric masses disappeared but the right pelvic hipodense residual lesion expanded. The double-j catheter was removed and two cycles of chemotherapy (vincristine, endoxan) was administered. Four months later he was readmitted, and referred to the radiation oncology for radiotherapy. Abdominal USG and CT scan was revealed some benefit of the for
机译:我们介绍了一个年轻人的腹膜后/大网膜肿瘤的独特病例,其具有尤因氏肉瘤/周围原始神经外胚层肿瘤(EWS / pPNET)和增生性小圆形细胞瘤(DSRCT)的特征。这种高度侵袭性的肿瘤必须与需要不同疗法的其他原始肿瘤区分开来。肿瘤表现为起源于右肾的巨大肿块,并演变为侵犯肾脏的肾周肿块。术后四年内多次复发表明,肿瘤具有独特的位置,并与FL-1和WT1共表达。肿瘤的免疫组织化学显示EWS / pPNET和DSRCT之间存在已知的免疫表型重叠。肿瘤同时多方向表型表达的能力可以为EWS / pPNET和DSRCT形成单组肿瘤的概念提供支持。病人死于手术后四年。这些实体之间的准确区分具有明确的预后和治疗意义。引言尤因肉瘤/周围原始神经外胚层肿瘤由一组推测为神经外胚层起源的恶性小圆形细胞瘤(SRCT)组成,最常见于儿童和年轻人的骨骼和软组织中1。 SRCTs的严重形态和基因重叠以及稀有性使其难以准确诊断。 EWS / PNET的特征是平衡的染色体易位,可产生EWS基因和Friend白血病病毒整合1(FLI-1)的融合转录本。在文献中偶发地报道了肾脏2,腹膜后3和网膜4的原发性EWS / pPNET病例。据我们所知,本病例是第二例在腹膜后/大网膜中观察到的EWS / pPNET多囊性囊肿出血和坏死的多囊肿。该肿瘤在其位置以及与FLI-1和Wilms肿瘤相关(WT1)抑癌基因的共表达中具有独特性,分别可诊断EWS / pPNET5和DSRCT6。病例报告一名28岁的男性患者最近伴有右腰部隐痛,在一个小城市的社区医院进行了右肾切除术,最终诊断为高度肉瘤样肾细胞癌,并弥漫性肾周浸润。两年后,他被转诊到泌尿科接受进一步评估。实验室研究在正常范围内。 MRI和CT扫描显示位于腹部左下象限的多个肿块。一个是在降结肠的前外侧延伸到肝脏的下侧面。第二个肿块位于ce骨后方,在右侧肌的前方,在右侧腰肌的侧面,第三个肿块在动脉分叉的前方,在右侧腰肌的旁边。他们表现出内部的囊性变性,叶状,坏死和高度出血。切除肿瘤后,进行四个周期的多化学疗法(吉西他滨和顺铂),在后续的CT扫描后切换为磷酰胺和阿霉素。六个月后,CT扫描显示疾病进展。腹部超声检查(USG)和CT扫描显示巨大的囊性肿块,轮廓呈小叶状,颅尾尺寸为24 cm。在肝脏下方有一个大的出血性和囊性肿瘤,累及网膜组织和右腰大肌。六个月后,他再次入院并切除了网膜肿块,插入了一个左双j导管。三个月后,肝下和肠系膜肿块消失了,但右盆腔hiphodense残留病变扩大了。移除双j导管,并进行两个周期的化学疗法(长春新碱,内毒素)。四个月后,他再次入院,并转至放射肿瘤学进行放射治疗。腹部USG和CT扫描显示了

著录项

相似文献

  • 外文文献
  • 专利
获取原文

客服邮箱:kefu@zhangqiaokeyan.com

京公网安备:11010802029741号 ICP备案号:京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有
  • 客服微信

  • 服务号