早期肾原始神经外胚层肿瘤一例报告

原发于肾的原始神经外胚层肿瘤( primitive neuroectodermal tumor,PNET)罕见,肿瘤生长快,侵袭性强,发现时常为晚期,治疗效果很差.如何提高对肾PNET的认识,特别是早期肾PNET的认识,对此病的诊断和治疗均有重要的意义.我院收治早期肾PNET患者1例,现报告如下.患者,男,37岁.因间歇性肉眼血尿10 d于2008年12月15日入院.血尿呈无痛性全程伴小蚯蚓状血块,无腰部疼痛,无发热盗汗.体检双侧肾区无叩痛.B超检查示左肾皮髓交界处异常回声区,无明显肿块形,考虑肾柱肥大可能.IVU示左肾上组肾盏显影不良.     

肾原始神经外胚层肿瘤1例报告

肾原始神经外胚层肿瘤(renal primitive neuroectoder-mal tumor,rPNET)是一种高度恶性的肉瘤,临床罕见,预后极差,术前影像学检查通常难以确诊[1].本文报道我院2016年12月收治的1例rPNET青年女性患者.     

椎管内外周型原始神经外胚层肿瘤1例报告

原始神经外胚层肿瘤(primitive neuroectodermal tumors,PNETs)是一组临床上罕见的发生于中枢神经系统和周围肌肉、骨骼等间叶组织、处于未分化阶段的具有多潜能分化能力的小细胞肿瘤。根据生长部位不同,分为中央型原始神经外胚层肿瘤(central PNETs,c PNETs)和外周型原始神经外胚层肿瘤(peripheral PNETs,p PNETs)。p P-     

椎管内外原始神经外胚层肿瘤一例报告

患者男,17岁,因反复左下肢疼痛半年,伴腰部酸痛1个月入院。体格检查:L2,3棘间及左棘突旁压痛、叩击痛;会阴部感觉正常,左小腿内侧皮肤感觉减弱,左股四头肌肌力3级,左膝腱反射消失,跟腱反射正常;腰椎前屈活动受限,左下肢活动正常;左髌骨上10cm处周径较对侧小2cm;左下肢直腿抬高约20°,加强试验阳性。病理反射未引出。实验室检查未见明显异常。MR检查:L2,3椎体水平椎管偏左侧可见一等T1等T2信号,脊髓明显受压,向右侧移位,增强扫描后肿块明显强化;水平面、冠状面均显示肿块沿L3左侧椎间孔向椎管外生长,约3.8cm×3.6cm×4.1cm,边缘不规则,脊…     

外周性原始神经外胚层瘤一例

正患者女,20岁,因发现颈项部无痛性肿物3年入院。患者于3年前无意中发现未予重视,近半年自觉肿物迅速增大来诊。查体:心、肺、腹部未见明显异常,颈软,皮肤色泽正常,无湿疹、溃疡、瘘管,气管居中。颈项部偏右侧可见5.0 cm×6.0 cm×4.0 cm大小肿物,质韧,边界清楚,表面无红肿破溃,活动度尚可,颈部未触及肿大淋巴结。入院后完善化验及检查未见明显异常,超声检查提示:后颈部距皮下0.4 cm可见范围约4.9 cm×5.2 cm×3.3 cm低回声团,边界尚清晰,形态欠规整,内回声不均匀,CDFI示其内及周边可见血流信号。于2017年9月21日在局麻下行颈部肿物切除术,术中见肿物无明显边界,伴胶冻状液体流出,快速冰冻提示恶性肿     

阴茎原发性外周原始神经外胚层肿瘤1例

外周原始神经外胚层肿瘤 (PNET)常发生在下肢和椎旁软组织等处 ,发生在阴茎者实属罕见 ,我们在会诊中遇到 1例 ,现报告如下。1　临床资料病人 ,男 ,18岁。 2 0 0 2年 9月发现阴茎头部肿块渐大 3个月 ,大小约 5cm× 4cm× 4cm ,几乎累及阴茎头部一周 ,有疼痛感 ,排尿无障碍 ,且无包皮过长病史。全身体检未见其他部位有占位病灶。在外院活检 ,切片经苏木精 伊红染色后至我院会诊。镜下见肿瘤组织在表皮下呈浸润性生长 ,瘤组织呈分叶状、条索状、片状分布 ,并见宽窄不等的纤维间隔 ,瘤组织内见多灶性坏死 ,瘤细胞大小较一致 ,胞质稀少 ,染色…     

乳腺原始神经外胚层肿瘤一例

正患者男,21岁,未婚,因"右乳巨大肿块5月余"收入郑州大学人民医院(河南省人民医院)治疗。5月前无意中触及右乳一肿物,未予特殊治疗,肿物渐进性增大,伴胀痛,无局部红肿、发热等特殊不适。否认家族性恶性肿瘤病史及遗传病史。全身体格检查未见明显异常。乳腺专科检查:双乳头无内陷及湿疹样变,双乳皮肤无橘皮征、酒窝征;右乳可触及一巨大肿块,约15.0 cm×10.0 cm,质硬伴胀痛;左乳未触及明显肿块;     

胰腺原始神经外胚层肿瘤一例

原始神经外胚层肿瘤(primitive neuroectodermal tumour,PNET)是一种较为罕见的高度恶性神经系统肿瘤,多发生在骨和软组织等处,极少发生于实质性脏器.我院收治1例胰腺原始神经外胚层肿瘤患者,现报道如下.     

累及泌尿系统的外周原始神经外胚层肿瘤

目的探讨累及泌尿系统的外周原始神经外胚层肿瘤(pPNET)的临床特点、免疫组化特点和诊断标准，提高pPNET的诊治水平。方法回顾分析7例累及泌尿系统的pPNET患者临床资料，其中6例行肿瘤根治性切除术，1例前列腺pPNET术中发现肿瘤已侵犯膀胱颈部和直肠前壁，仅行活检术。结果7例术后病理和免疫组化检查均诊断为pPNET。围手术期无患者死亡，7例患者随访4～26个月，平均12个月，1例术后5个月死于肿瘤远处转移和肾功能衰竭。结论泌尿系统pPNET是一种好发于年轻男性的恶性肿瘤，病变进展迅速，确诊依靠病理，预后较差，但发病早期根治性手术仍有较大的治疗意义。     

肾上腺原始神经外胚层肿瘤1例报告

<正>原始神经外胚层肿瘤(primitive neuroectodermal tumors,PNET)是起源于原始神经上皮的恶性小圆细胞肿瘤;发生于肾上腺者罕见且恶性程度高、病程短、易复发转移,预后差。2018年徐州医科大学附属医院诊治1例,现报告如下。1病例报告患者女性,15岁,因"右腰部间断性疼痛1周"入     

A primitive neuroectodermal tumor on the face: case report

In 1918, Stout defined the lesion in which small round cells originating from the ulnar nerve formed a rosette as neuroepithelioma. It was claimed that this tumor originated from neuroectodermis and was different from the classical neuroblastoma. The term primitive neuroectodermal tumor (PNET) involves a group of tumors of the soft tissue originating from neural crest and resulting from the brain, spinal cord and branches of the sympathetic nervous system. Extracranial primitive neuroectodermal tumors originate from neural crest cells outside the sympathetic and central nervous system. PNET also has some distinctive histological, immunohistochemical and ultrastructural features. It is usually encountered in children and young adults; most frequently located in thoracopulmonary region (Askin's tumor). The second most commonly involved body part is the extremities. It is very rarely located on the face. PNET is an aggressive tumor. In fact, the disease has a rapid progression, causes local or distant metastases and 50% of the patients die within two years of the presentation. It is treated with aggressive surgery as well as chemotherapy and radiotherapy. In this report, we presented a case of PNET located on the right cheek with multiple distant metastases. Clinicians should be on alert when treating facial tumors, not to skip PNET, which is a very aggressive one.     

外周性原始神经外胚层肿瘤超声表现1例

患者男,60岁,主因"肩背部肿物伴疼痛1个月"就诊。查体:左肩背部可触及一肿物,约6.5cm×6.5cm,突出体表,质韧,无波动感,边界较清,活动度差;表面皮肤无红肿。超声:左肩背部肌层内见低回声,约6.5cm×6.5cm×3.6cm,边界清,有包膜,内部回声不均匀,可见散在条状高回声及不规则形液     

Primary primitive neuroectodermal tumor of the kidney: A case report

There are nearly 50 PNET cases in the literature with primary renal localization. The mean age for renal PNET is 28 but it can be seen in a wide range between 4 and 69 years. In this atypical localization PNET may be mistaken with a variety of small round cell tumors, particularly with blastema predominant Wilms’ tumor and due to its distinctive prognostic and therapeutic features differential diagnosis is very important. Immunohistochemical studies are useful in discriminating PNET from other renal small round cell tumors. Renal PNETs tend to metastasize at early stages and they have a high rate of recurrence. Our case is a 45-year-old male presented with left flank pain and hematuria. In computerized tomography a mass in left kidney was observed and nephrectomy was performed. Histopathologic examination revealed a small round cell tumor with rosettes and pseudorosettes. Immunohistochemically the lesion was diffuse strong positive with CD99 and NSE, negative with LCA, pancytokeratin, vimentin, desmin, smooth muscle actin, chromogranine A and S-100. At the time of diagnosis the pathologic stage of the tumor was T1aN0Mx. The patient did not take any further therapy and in 1-year follow-up no local recurrence or distant metastasize occurred.     

Supratentorial primitive neuroectodermal tumor: report of a surgical case

We reported a rare case of supratentorial primitive neuroectodermal tumors (sPNET) and reviewed the literature. A 15-year-old girl presented with a one-month history of headache and vomiting. CT scans showed a huge, solid and cystic tumor with calcification, occupying the left anterior cranial fossa. The solid portion of the tumor was hypointense on T1-weighted images, slightly hyperintense on T2-weighted images, hyperintense on diffusion- weighted images, isointense on fluid-attenuated inversion recovery (FLAIR) images, and strongly enhanced after administration of contrast medium. The expansile tumor had a broad attachment to the dura matter of the anterior cranial fossa. The patient underwent an uneventful extirpation of the tumor. Microscopically, the solid tumor contained small, round poorly-differentiated cells with pleomorphic nuclei and brisk mitotic activity. The tumor cells were immunoreactive for synaptophysin and GFAP, whereas lack of MIC2 gene product expression was confirmed using the monoclonal antibody 12E7. The MIB-1-positive rate was 43%. The histological diagnosis was sPNET. Postoperatively, a dose of 30.6Gy was administrated to the whole brain and a boost of 19.6Gy to the T2-hyperintensity lesion. The patient successively received a dose of 30.4Gy to the whole spine and Linac stereotactic radiotherapy with a marginal dose of 16.8Gy at the tumor bed. Three months after radiotherapy the patient received chemotherapy using carboplatin and etoposide. Follow-up MR images showed no evidence of recurrent tumor 5 months after the radiochemotherapy. On the basis of MR findings on both diffusion-weighted and FLAIR images, preoperative diagnosis of sPNET may be important for choosing appropriate therapeutic strategies for this tumor.     

Peripheral primitive neuroectodermal tumor of the jugular foramen: case report

OBJECTIVE AND IMPORTANCE: Peripheral primitive neuroectodermal tumor (pPNET) is a rare type of tumor, most commonly found in the limbs of children and young adults. The authors report an extremely rare case of pPNET located at the jugular foramen whose clinical course demonstrated rapid progression. CLINICAL PRESENTATION: A 23-year-old man presented with a 2-month history of progressive hoarseness and dysphagia. The neuroradiological appearance of the lesion was a jugular foramen tumor. INTERVENTION: The patient underwent a partial resection of the tumor through a far lateral suboccipital craniotomy. After surgery, the patient experienced an unexpected deterioration in consciousness. Magnetic resonance images on postoperative Day 18 revealed rapid and large expansion of the residual tumor into the posterior fossa. No adjuvant therapy was administered, and the patient died 6 weeks after diagnosis. CONCLUSION: The pathological diagnosis of the surgical specimen was pPNET, according to the findings of hematoxylin and eosin and immunohistochemical stainings. To the best of our knowledge, this is the first reported case of pPNET at the jugular foramen.     

Intracranial primitive neuroectodermal tumor in an infant: a case report

A 2-month-old girl with a supratentorial primitive neuroectodermal tumor (PNET), which extended into the skull, is herein presented. The patient underwent total removal of the tumor and also received a course of postoperative chemotherapy. After a follow-up period of 12 months, the infant is alive without recurrence. Histologically, the tumor was composed of poorly differentiated neuroectodermal cells, and these neoplastic cells showed a mild immunohistochemical reaction for GFAP and synaptophysin, and a moderate reactivity for neuron specific enolase and vimentin. In addition, a moderate level of immunoreactivity for HBA71 antigen (p30/32M1C2), which is the product of the M1C2 gene and is found in peripheral PNETs but not in central PNETs, was noted in many neoplastic cells. Although this tumor was located intracranially, it may be classified as a peripheral PNET.     

A supratentorial primitive neuroectodermal tumor exhibiting pathological characteristics of medulloepithelioma: a case report

A 50-year-old female presented with headache symptoms and left hemifacial paresis. Brain computed tomography and magnetic resonance images demonstrated a right frontal lobe tumor with hemorrhage and calcification. A gross total resection was carried out. Pathologically, the tumor exhibited three kinds of structures. The first was an astrocytic area which occupied the large part of this tumor. Small, round cells, which were positive for glial fibrillary acidic protein, were randomly distributed. The second was an epithelioid area with the highest MIB-1 labeling index of the three structures. Undifferentiated tumor cells demonstrated a dense proliferation, and short spindle-shaped cells exhibited an epithelial and a partly luminal alignment that resembled the embryonal neural tube. The third was a sarcomatoid area. Long spindle-shaped cells, which were positive for vimentin, were in a bundle-like form. We diagnosed this tumor as a primitive neuroectodermal tumor (PNET) with epithelial differentiation. PNETs are rare brain tumors with a predominance in children. Histologically, undifferentiated tumor cells demonstrate dense proliferations. The tumor arises from germinal matrix cells that differentiate into neuronal, glial, and ependymal cells. Medulloepithelioma, which is classified in the PNET category, is a very rare brain tumor that usually develops in childhood. Pathological studies have demonstrated that papillary, tubular, or trabecular arrangements of neoplastic neuroepithelium mimic the embryonic neural tube predominantly. In our case, the tumor partly demonstrated epithelial differentiation, which is a feature of medulloepithelioma, not only glial and mesenchymal differentiation. This is a rare case of PNET with uncommon histological features.