喉部原始神经外胚叶肿瘤1例

原始神经外胚叶肿瘤（primitive neuroectodemlal tunaor．PNET）是神经上皮组织的胚胎性肿瘤，来源于原始神经外胚层细胞，组织学形态分类归属于恶性小圆细胞肿瘤．由幼稚的神经外胚层细胞组成。根据肿瘤发生部位的不同，PNET分为中枢性（cPNET）和外周性（pPNET）两类。由于pPNET和Ewing肉瘤、Askin瘤具有相同的临床表现、形态学特征、免疫组化表型、遗传学特点及临床预后，     

胸部原始神经外胚层肿瘤的诊断与治疗(附四例报告)

目的：探讨累及胸部的外周原始神经外胚层肿瘤（primitive neuroectodermal tumor,PNET）的临床特点、病理学特点、诊断标准、治疗与预后,进而提高胸部PNET的诊疗水平。方法：回顾性分析4例胸部PNET的临床资料。本组纵隔PNET3例,其中2例肿瘤侵犯上腔静脉,行肿瘤大部切除,1例在胸腔镜下行纵隔包块探查活检术;右肺PNET1例,行锁骨上包块活检术。结果：4例术后病理和免疫组化检查均诊断为PNET。2例未行任何治疗,1例行全身化疗,1例行局部放疗。未接受治疗2例生存期分别为6、7个月;接受全身化疗者生存期为12个月;接受局部放疗者截止2006年8月已生存4个月,结论：胸部PNET可发生于任何年龄,原发病灶体积较小时即可发生远处淋巴结转移;临床误诊率高,需依靠病理及免疫组化确诊。由于胸部PNET恶性程度较高,局部浸润与远处转移迅速,因此手术切除率较低,预后较差,需寻找更为有效的治疗方法。     

原始神经外胚层瘤一例并文献复习

目的 探讨原始神经外胚层瘤(PNET)的临床特点及诊治方法.方法 回顾分析1例病例,结合文献资料,总结原始神经外胚层瘤的临床特点、免疫组织化学、鉴别诊断和治疗方法.结果 患者出现DIC,多种治疗方法无效,凝血功能异常,自动出院.结论 原始神经外胚层瘤主要依靠病理检查联合免疫组织化学确诊,因其恶性程度高,又无完善治疗方法.目前只采用外科手术、化疗和放疗的治疗方法,预后不良.     

血尿表现的前列腺原始神经外胚层瘤1例并文献复习

目的:探讨前列腺原始神经外胚层瘤临床表现、病理学特点、诊断标准及治疗与预后,以提高对本病的诊疗水平。方法:回顾性分析我院前列腺原始神经外胚层瘤1例及总结文献检索所得前列腺PNET病例12例的临床表现、病理结果及治疗与预后。结果:病例中最短生存期为术后一个月死于肺转移。最长生存期为随访至12个月。我院病例术后4月复查盆腔肿瘤复发。结论:外周PNET十分罕见,前列腺PNET更罕见。对于排尿困难和血尿的青年患者如诊断不明确应考虑本病可能。超声、CT、MRI可发现该疾病但无特异性,病理可诊断。早期诊断、积极治疗对预后影响大,对于晚期患者化疗效果明显。     

中枢性原始神经外胚层肿瘤7例及文献复习

目的探讨中枢性原始神经外胚层肿瘤的诊断和治疗方法以指导临床。方法回顾分析7例中枢性原始神经外胚层肿瘤患者的临床特征、诊断和治疗。结果7例均行手术治疗、术后病理证实为中枢性原始神经外胚层肿瘤。肿瘤全切除4例，次全切除3例。术后近期患者症状均好转。6例随访2个月～3．5年，1例失访。有3例复发，其中1例死亡。结论中枢性原始神经外胚层肿瘤属于高度侵袭性肿瘤．此病预后极差。     

婴儿色素性神经外胚瘤的临床病理研究

目的 探讨婴儿色素性神经外胚瘤的临床病理特点。方法 对2例婴儿色素性神经外胚瘤进行临床资料分析和组织病理学、免疫组织化学及电镜观察。结果 该肿瘤多发生于1岁以内的婴幼儿。主要发生在上、下颌骨，呈浸润性生长、溶量性破坏。组织学特征为：肿瘤由含色素的上皮样细胞和小的神经母细胞样细胞构成。免疫组化结果为：上皮样瘤细胞表达CK、EMA、HMB45、S－100蛋白及Vim，小的瘤细胞则表达NSE，Syn及Vim。超微结构观察：大的瘤细胞质内含黑色素小体，小的瘤细胞质内见神经内分泌颗粒。结论 婴儿色素性神经外胚瘤起源于原始的神经外胚层细胞，为低度恶性肿瘤。     

信息动态

原始神经外胚层瘤(primitive neuroectodermal tumor,PNET)是一种起源于神经嵴胚胎残留组织、有多种分化潜能的小圆细胞恶性肿瘤,是与Ewing肉瘤同一谱系的一类高侵袭性肿瘤.近年来随着免疫组化的广泛应用及分子生物学的飞速发展,PNET的报道逐渐增多,但原发于泌尿系统的PNET临床少见,发生于膀胱的PNET则更罕见.结合相关文献,本研究探讨PNET的临床和病理特征及诊断和鉴别诊断要点.     

肾脏原始神经外胚叶肿瘤1例报告及文献复习

目的：探讨肾脏外周原始神经外胚叶肿瘤（pPNET）的临床和病理特征。方法：对1例肾脏原始神经外胚叶肿瘤临床表现、病理特征及免疫组化表型进行观察并文献复习。结果：患者男性,35岁,以肉眼血尿为首发症状,B超示左肾上极实性肿物。镜下可见核分裂相,肿瘤坏死明显,免疫组化CD99（＋＋＋）、NSE（＋）、EMA（-）,病变符合原始神经外胚叶肿瘤。结论：原始神经外胚叶肿瘤的诊断依赖病理特征及免疫表型,并需与其他小细胞恶性肿瘤进行鉴别。     

49例原始神经外胚层肿瘤临床特征及生存分析

摘 要：［目的］ 探讨影响原始神经外胚层肿瘤预后的相关因素。［方法］ 回顾性分析了49例原始神经外胚层肿瘤患者的临床资料及预后相关因素。［结果］ 49例原始神经外胚层肿瘤的1年、2年、3年、5年生存率分别为65.3%、51.0%、26.5%、8.4%、中位生存时间为16个月;单因素分析显示年龄、肿块大小、分期、治疗方式是影响原始神经外胚层肿瘤患者预后的重要因素。Cox多因素分析后发现肿瘤分期和治疗方式是影响其预后的独立危险因素。［结论］ 原始神经外胚层肿瘤采用联合放疗的综合治疗方案有助于改善患者的预后。     

脊柱原始神经外胚层肿瘤的临床特点和影像学分析

目的:分析脊柱原始神经外胚层肿瘤的临床和影像学特点,以提高对该病的认识.方法:分析6例经手术病理或穿刺活检确诊的脊柱原始神经外胚层肿瘤的临床、病理特点及影像学表现.结果:6例脊柱原始神经外胚层肿瘤,其中发生于颈椎和腰椎各2例,胸椎和骶椎各1例,影像学表现主要为椎体的溶骨性破坏和椎管内硬膜外巨大软组织肿块,增强后明显不均匀强化,而骨膜反应、钙化或骨化不明显.HE染色肿瘤细胞镜下均均呈小圆形,且伴有Homer-Wright(H-W)"菊形团"排列;免疫组化神经染色至少有2种神经标记为阳性.结论:眷柱原始神经外胚层肿瘤临床表现和影像学表现具有一定特点,但缺乏特异性,确诊需组织病理学.影像学检查的目的在于明确肿瘤侵犯范围,确定手术的可切除性以及评价疗效.     

腹腔内促结缔组织增生性小圆细胞瘤临床病理分析

目的：探讨腹腔内促结缔组织增生性小圆细胞瘤临床病理特征。方法：对2例原发于腹腔的促结缔组织增生性小圆细胞瘤进行HE、免疫组织化学观察并复习相关文献。结果：肿瘤细胞为小圆形，呈巢团状排列，周围结缔组织及血管明显增生。免疫组织化学表达上皮性、间叶性、神经性标记物。结论：促结缔组织增生性小圆细胞瘤是一种特殊的高度恶性肿瘤；预后极差，主要应与小细胞未分化癌、尤文氏肉瘤、PNET、神经母细胞瘤等鉴别。     

Establishment of a Human Cell Line Secreting Neuron-specific Enolase from a Primitive Neuroectodermal Tumor of the Retroperitoneal Cavity

Primitive neuroectodermal tumor (PNET) is one of the small round cell malignancies of presumed neural crest origin for which an effective treatment has not yet been established. In the present study, a human cell line, designated KU-9, was established from a 27-year-old male patient with PNET of the retroperitoneal cavity and has been successfully maintained in nude mice and in culture. On histological examination, the primary tumor was composed of poorly differentiated small round cells arranged in clusters showing a variety of mitotic changes, and contained Homer-Wright rosettes. The histopathological appearance of the KU-9 xenografts was similar to that of the primary tumor. Electron microscopy revealed neurosecretory granules and cytoplasmic processes in the xenograft. No significant amplification of N- myc gene was observed in the KU-9 cells. The KU-9 cells showed chromosome numbers ranging from 56 to 61 with consistent structural abnormalities being add(2)(q31), +add(ll)(pll.2), +add(13)(pll.l), and + del(22)(q12). Cultured KU-9 cells grew exponentially with a doubling time of about 50 h and a time-dependent increase in medium levels of neuron-specific enolase (NSE) was noted. Serum levels of NSE in KU-9 tumor-bearing nude mice were significantly elevated and a linear relationship between the serum NSE levels and the tumor NSE content or tumor volume was observed, suggesting that serum levels of NSE may reflect the PNET tumor burden and tumor extent. These results indicate that the KU-9 cell line provides a reproducible model system which could be useful in gaining some insight into the histogenesis and oncogenesis of PNET and in establishing an effective treatment for PNET.     

宫颈原发性透明细胞癌临床病理观察

目的:探讨宫颈原发性透明细胞癌的临床病理学特征、鉴别诊断要点，提高对其认识。方法:回顾性分析1例宫颈原发性透明细胞癌，分析其病理组织学和免疫表型特征，并复习相关文献。结果:患者，女性，58岁，绝经8年。30年前因“卵巢囊肿”行手术治疗。宫颈B超未见明显新生物及溃疡。巨检示宫颈至宫颈管质硬，环绕管壁一周，质硬处切面灰白灰黄色，实性，界不清。镜下见肿瘤大部分由较小并且相对规则的乳头构成，有时可见含有纤维血管轴心的大乳头，乳头轴心可透明变性。部分区域显示扩张的腺体被覆单层扁平及立方上皮细胞。肿瘤细胞呈中重度异型增生，胞质透明，细胞呈多边形。部分区域细胞呈鞋钉样，细胞核呈染色质深染，部分有明显核仁，细胞突向腺腔内。其他部位未发现子宫内膜异位改变。免疫标记PAX8、NapsinA显示弥漫强阳性，p16显示局灶强阳性，p53显示斑驳状阳性。结论:宫颈原发性透明细胞癌是一种罕见的肿瘤，诊断依据其病理组织学形态及免疫表型，要注意与宫颈原发性腺癌及转移性癌相鉴别。     

A Case of Renal Primitive Neuroectodermal Tumor Confirmed by Fluorescence in situ Hybridization

Primitive neuroectodermal tumor (PNET) is a member of the Ewing''s sarcoma family of tumors (ESFT). We report a case of PNET in a 66-year-old male who presented with a large solid tumor within the parenchyma of the middle pole of the left kidney with metastases to the left adrenal gland and right ischium. A fine-needle biopsy was performed and showed a small round cell tumor. Results of immunohistochemical staining suggested this tumor belonged to ESFT. Preoperative VDC-IE (combined vincristine, doxorubicin and cyclophosphamide followed by another combination of ifosfamide and etoposide) chemotherapy and left radical nephrectomy and adrenalectomy were performed. The histopathological findings of the resected tumor were similar to those in the biopsy specimen, but the results of AE1/AE3 were different. For the diagnosis, fluorescence in situ hybridization was performed. Split signals of the EWSR1 gene were detected, and transmission electron microscopy showed neuroendocrine granules and microtubules. The final diagnosis of this tumor was PNET of the kidney.Key Words: Primitive neuroectodermal tumor, Kidney, Fluorescence in situ hybridization, VDC-IE chemotherapy     

外周型原始神经外胚层肿瘤的CT和MRI影像特征

背景与目的:外周型原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)用组织学方法检查难以与其它的小圆形细胞肿瘤(如尤文氏肉瘤)区别.本研究目的在于分析和探讨外周型PNET的CT和MRI影像特征.方法:回顾性分析7例经病理证实的外周型PNET的CT和MRI表现.结果:7例PNET发病部位分别为左鼻翼、右下颌骨、左/右胸壁、左精索、胸椎旁及腰段椎管内.发生于软组织的PNET CT表现主要为边界不清的软组织肿块,密度不均匀伴坏死、囊变,无钙化,增强后明显不均匀强化.发生于骨的PNET CT表现为溶骨性骨质破坏并伴巨大软组织肿块形成.发生于椎旁或椎管内的PNET MRI表现为与脊髓和马尾分界清的软组织肿块,在T1WI上呈稍低或等信号改变,在T2WI上呈稍高或等信号改变.结论:外周型PNET的CT和MRI表现缺乏特异性影像特征;CT和MRI检查可显示肿瘤的范围和发现远处转移,对确定手术的可切除性及评价治疗效果有重要参考价值.     

胰腺实性假乳头状瘤的临床病理特征

目的 探讨胰腺实性假乳头状瘤的临床病理特征.方法 回顾性分析3例胰腺实性假乳头状瘤的临床资料、组织病理和免疫表型,并复习相关文献.结果 胰腺实性假乳头状瘤多见于女性,可发生于胰腺的任何部位,肿瘤呈囊实性,细胞形态一致,核分裂相罕见,瘤细胞形成特征性假乳头结构.免疫组化结果显示:Vim(+)、CD56(+)、CD10(+...     

Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin

A primitive neuroectodermal tumor (PNET) is a small round cell tumor that arises from the nerve crest. This tumor usually occurs in the central nervous system or soft tissue, but it can occur in the kidney in rare cases. Herein we report a case with severe multiple liver metastases after surgery for right renal PNET. The patient was a 21-year-old man with a chief complaint of right abdominal pain. Hemorrhage in a right renal malignant tumor was diagnosed, and radical nephrectomy was performed. Histopathology showed bare nuclear round atypical cells with a scarce cytoplasm proliferating like a seat and nest. Some of the cells formed a rosette structure and the tumor cells were positive for CD99, leading to diagnosis of PNET. Severe multiple liver metastases occurred 6 months after surgery, and six courses of chemotherapy with ifosfamide, etoposide and doxorubicin were performed. After this treatment, residual tumor was removed, but the tumor cells were absent histologically.     

胸腺非典型性类癌的临床病理及免疫组化表型

目的:了解胸腺非典型性类癌的临床病理特征。方法:收集2例胸腺非典型性类癌的临床资料,手术切除组织光镜切片观察,另作10项免疫组织化学标记:CKp,EMA,Ki-67,CD3,CD20,CD5,TdT,NSE,CgA,Syn。结果:2例胸腺非典型性类癌均为男性,纵膈肿瘤,由小园形细胞构成。瘤细胞CKp、EMA、NSE、CgA、Syn阳性,CD3、CD5、CD20、TdT阴性,Ki-67核阳性细胞指数>20%,肿瘤有显著坏死。结论:非典型性类癌是一种罕见的胸腺肿瘤,诊断靠病理组织学和免疫组织化学标记,手术切除为主要治疗方法。     

Characterization of a human endocrine tissue and tumor-associated Ewing's sarcoma antigen

The histogenesis of Ewing's sarcoma (ES), the second most frequent primary bone tumor in humans, remains controversial. A new cell line (SIM-1) was derived from a peripheral neuroectodermal tumor (PNET) and used for the production of a monoclonal antibody (HBA-71), which recognizes a novel cell surface antigen of ES- and PNET-derived cells and paraffin-embedded tumor sections. The HBA-71 antigen expression is restricted to PNET/ES and the antigen was not detected on cell lines or tissue sections of any other tumor tested, with the exception of ependymoma. Three proteins with molecular weights of 300,000, 185,000, and 90,000 were isolated from SIM-1 membrane extracts by HBA-71 affinity chromatography. Trypsin treatment of intact SIM-1 cells destroys the HBA-71 epitope and cleaves off two proteins with molecular weights of 210,000 and 95,000. HBA-71 antigen expression is not influenced by treatment of ES cell lines with differentiation inducers. Within normal tissues reactivity was observed with the adenohypophysis, ependymal cells, endocrine pancreas, Sertoli, and ovary granulosa cells. The reagent links ES with PNET and provides a highly valuable probe for (a) the immunohistological differential diagnosis of ES/PNET using fresh tissue or paraffin sections from other small round cell tumors, (b) the histogenetic studies of ES/PNET, and (c) the in vivo diagnostic and therapeutic procedures in patients with ES and PNET.