椎旁原始神经外胚层瘤CT和MRI诊断

目的:研究椎旁外周性原始神经外胚层瘤(pPNET)的CT及MR表现,提高对该病的认识。方法:报道5例经手术病理证实的pPNET的临床、CT、MRI及组织学所见,并复习相关文献。结果:5例均见单侧椎旁肿块,累及椎管内硬膜外,肋骨骨质侵蚀,胸髓受压变形;CT显示肿块形态不规则、密度均匀;MRI示T1WI肿块信号均匀、略高于邻近骨骼肌信号,T2WI呈不均匀高信号,增强后肿块明显均匀强化。术后MRI显示局部肿块复发和胸腔积液,CT发现肺部、纵隔多发转移结节。结论:pPNET为少见的起源于胸壁、椎旁的恶性肿瘤,其CT、MRI表现无特征性,但可明确肿瘤的范围、进行手术切除可能性评估及治疗后随访。CT易显示肋骨、椎体骨质破坏、肺部小转移灶,MRI则可显示胸壁受累、胸髓受压变形的程度和范围等。     

小儿外周原始神经外胚层肿瘤的MSCT诊断

目的分析小儿外周原始神经外胚层肿瘤(pPNET)的MSCT表现,提高对该病的认识。方法回顾性分析11例经病理确诊的pPNET的MSCT表现。结果 8例巨大软组织肿块伴骨质破坏,其中3例表现为骨质放射状增生伴局部骨质破坏,其中2例位于胸壁,1例位于肩胛骨;5例为溶骨性骨质破坏,其中2例位于后纵隔、1例发生于副鼻窦、1例发生在腰椎和1例发生在骶椎。3例为未伴骨破坏的软组织肿块,其中2例发生于腹膜后,1例发生于皮下脂肪层内。除发生在皮下脂肪层内病灶较小者,其余病灶都较大,平均直径为8 cm。软组织肿块以压迫推移邻近组织的方式生长。瘤体内钙化少见。结论 pPNET的MSCT典型表现为巨大软组织肿块,伴或不伴骨质破坏,增强呈均匀或非均匀的团絮状强化,大血管穿行于瘤体内较少见。有一定的特点,需与其他恶性软组织肿瘤鉴别。MSCT能较好显示病变内部结构,血供状况以及与邻近组织的关系,有助于术前分期、手术方案的确定、有无远处转移和治疗效果评价。     

骨原始神经外胚层肿瘤（2例报告并文献复习）

目的；提高对骨原始神经外胚层肿瘤的认识。材料与方法：对２例骨原始神经外胚层肿瘤的临床表现、影像特征进行描述，并结合文献分析。结果：（１）骨原始神经外胚层肿瘤来源于神经嵴的胚胎迁移细胞。（２）多见于儿童和青少年，发病部位以胸部最常见，其余部位还有骨盆，四肢等。（３）影像表现一般呈溶骨性骨折破坏伴有软组织肿块，多有囊变，少见瘤骨和钙化。（４）Ｅｗｉｎｇ‘ｓ瘤与骨原始神经外胚层肿瘤在组织学起源上是相关性     

骨原始神经外胚叶肿瘤影像学表现

目的研究骨原始神经外胚叶肿瘤的影像学特点。资料与方法搜集5例骨原始神经外胚叶肿瘤的影像及临床、手术病理资料,分析其影像学表现。结果5例中．胸椎2例,胸壁、股骨、胫骨各1例。1例胸壁病变表现为巨大软组织肿块,并推移周围组织。2例脊椎病变破坏椎管内外骨质及软组织。2例四肢长骨病变呈溶骨性骨质破坏,1例有软组织肿块。5例均未见病变内钙化及局部淋巴结肿大。MRI对显示病变组织成分及侵袭范围较X线和CT好。结论溶骨性骨破坏和巨大软组织肿块是骨原始神经外胚叶肿瘤的常见影像学表现,病灶内钙化和局部淋巴结肿大少见。MR／对显示病变组织成分及侵袭范围较好。本病最终诊断依靠病理和免疫组织化学检查。     

骨和累及骨的外周性原始神经外胚层瘤的临床病理与影像学表现

目的探讨骨和累及骨的外周性原始神经外胚层瘤(pPNETs)的临床病理与影像学表现。资料与方法7例中6例有X线检查、2例有CT检查、4例有MR检查,分析其临床病理和影像学特点。结果6例X线片上均呈溶骨性骨质破坏,其中2例病变区存在骨质硬化,5例合并软组织肿块,均未见骨膜反应。2例CT像上均为溶骨性骨质破坏合并软组织肿块形成,软组织内未见钙化或骨化。MRI检查4例中有3例病变在T1WI呈中等信号,1例在T1WI呈低信号,4例在T2WI上均呈中、高信号,信号不均匀,增强扫描呈中度不均匀强化。4例均合并软组织肿块,其中3例有囊变坏死区。结论影像学上骨内病变呈溶骨性破坏,可伴有病变区的骨质硬化而一般无骨膜反应,并伴有较大软组织肿块者应考虑到pPNETs的可能。     

外周原始神经外胚叶肿瘤的临床及CT、MRI诊断

目的分析外周原始神经外胚叶肿瘤(pPNET)的临床特点和CT、MRI表现,以提高对该病的认识。资料与方法回顾分析8例经病理证实的pPNET的临床表现和CT、MRI征象。结果原发于上肢pPNET1例,头面部2例,腹膜后2例,脊柱旁3例。初诊时2例有骨转移,1例颌下淋巴结肿大。病灶体积较大,呈浸润生长,推压邻近结构,并可凸入椎管。肿瘤密度／信号不均,CT为软组织密度,轻至中度强化;MR T1WI肿块与肌肉等信号,T2WI呈不均匀高信号,增强扫描呈花环状或蜂窝状强化。1例有钙化,3例邻近骨质破坏,呈溶骨性破坏伴骨膜反应。结论pPNET是好发于青少年的少见恶性小圆形细胞肿瘤,预后差。CT、MRI征象无特异性,但对评价肿瘤的可切除性、治疗效果和制订治疗方案非常有价值。     

外周性原始神经外胚层肿瘤的CT及MRI影像学特征

目的分析外周性原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumor,pPNET)的CT和MRI影像学表现,以提高对其认识和诊断的准确性。资料与方法回顾性分析经病理证实的11例pPNET患者的CT及MRI影像学表现。11例pPNET中9例行CT检查,2例行脊柱MRI检查。结果 11例病灶中1例位于鼻腔,4例位于胸壁,2例位于腹膜后,2例位于盆腔,2例位于脊柱。9例软组织pPNET表现为较大且浸润性生长的软组织肿块,伴或不伴邻近骨质的溶骨性破坏。肿块内密度多不均匀,增强后呈中等程度以上不均匀强化。双期扫描动脉期多数肿瘤内见细小供血动脉,静脉期肿瘤持续性强化且密度趋向均匀。2例脊柱pPNET表现为椎体骨质破坏及周围软组织肿块,肿块边界不清,包绕椎体并延伸至椎管内压迫脊髓。增强后病灶明显强化且不均匀。结论 pPNET有一定的临床及影像学特点,密切结合临床及影像学表现是诊断的关键。     

Primitive neuroectodermal tumor of the leptomeninges

Summary Primitive neuroectodermal tumor (PNET) without an obvious intraparenchymal mass lesion and with diffuse spread in the subarachnoid space is rare. We report such a rare case and discuss the computed tomography (CT) and magnetic resonance (MR) features.     

外周性原始神经外胚层肿瘤的影像表现

目的分析外周原始神经外胚层肿瘤(pPNET)的CT和MR I表现,以期提高对该病的认识。方法回顾性分析6例经病理诊断的外周原始神经外胚层肿瘤的CT和MR I表现。男4例,女2例,其中起源于骨组织2例,软组织pPNET 4例。结果骨组织pPNET表现为溶骨性骨质破坏伴有软组织肿块,病灶CT呈等密度,MR I T1W I等信号,T2W I等、高信号,增强明显但不规则,无明显瘤骨和骨膜反应;软组织pPNET病灶体积比较大,直径>6 cm,CT等密度,MR I T1W I等、低信号,T2W I等、高信号,增强明显,对周围正常组织有侵袭,无钙化。2例有明显的坏死。结论pPNET的CT和MR I表现缺乏特征性,但有助于本病良恶性鉴别、判断手术可切除性、检出远处转移和评价治疗效果。     

胸部原始神经外胚层瘤1例

患者女,19岁,5 d前无明显诱因右下胸持续性疼痛,伴有咳嗽、咳痰少许,为白色泡沫痰,痰中带有鲜红色血丝.体检:贫血貌,气管左移,右侧胸廓饱满,右肺叩诊浊音,右肺呼吸音消失.     

Primary Yolk Sac Tumor Arising in the Pancreas with Hepatic Metastasis: a Case Report

Extragonadal yolk sac tumor (YST) is a relatively rare entity. We describe here the case of an extragonadal YST that occurred in the pancreas with hepatic metastasis in an adult woman. The contrast enhanced CT images of the abdomen revealed a heterogeneous, solitary mass occupying the pancreatic neck and body with slightly inhomogeneous contrast enhancement. Two low-density lesions in the liver were also displayed on the CT images. The patient underwent surgery and the diagnosis of YST was pathologically verified.     

Primitive neuroectodermal tumour of the cauda equina

Primary primitive neuroectodermal tumours of the cauda equina are rare. We report a case and review the literature.     

Primitive neuroectodermal tumor in a child with tuberous sclerosis

 The case of a 13-year-old boy with tuberous sclerosis and primitive neuroectodermal tumor of bone is presented. A pathogenetic association between these two entities is postulated, the unifying underlying mechanism being a maldevelopment of the neural crest, or neurocristopathy.     

Magnetic Resonance Imaging Characteristics of Primary Intraspinal Peripheral Primitive Neuroectodermal Tumour

Purpose

The purpose of our study was to describe the salient magnetic resonance imaging (MRI) findings in primary intraspinal peripheral primitive neuroectodermal tumour (PNET).

Methods

A retrospective review of the clinical and MRI images of 7 pathologically proven cases of intraspinal peripheral PNETs was performed. The various parameters, such as vertebral level of involvement; tumour location, size, focality, and margin; signal intensity of the lesion; the presence of hemorrhage or calcification; any signal voids; assessment of the adjacent cord for cord compression; cord dilatation; the presence of paraspinal tissue mass; or vertebral or other bony changes, were analysed.

Results

All 7 patients had lesions in the thoracolumbar region. Three patients had extradural lesions, 4 had intradural extramedullary lesions, and none had intramedullary lesions. Six lesions were well circumscribed. Only 1 patient had multifocal involvement. All lesions were of hypointense or isointense signal on T1-weighted imaging, whereas all but one were hyperintense on T2-weighted imaging. Lesions enhanced heterogeneously except 1 intradural extramedullary lesion, which enhanced homogeneously. A paraspinal mass was noticed in 2 patients. Vertebral collapse was present in 1 patients.

Conclusion

Intraspinal peripheral PNETs are rare spinal tumours. Although imaging characteristics are not specific, a focal circumscribed lesion in a young individual at the intramedullary, extramedullary intradural, or extradural spinal location that shows hypointense and hyperintense signal on T1- and T2-weighted images, respectively, requires PNET to be considered in the differentials.     

小儿腹膜后原始神经外胚层肿瘤1例

患儿 男,3岁6个月,以纳差10 d,腹痛4 d求诊.查体:精神反应差,腹部膨隆,左中上腹部触及一约7 cm×6 cm×4 cm大小的类圆形肿块,质地中等,表面光滑,边界清楚,活动度差,无压痛.无移动性浊音.呼吸、循环及神经系统未发现异常.血、尿、便常规及血液生化检查无明显异常改变.B超扫查提示左中上腹混合性包块影.     

成人幕上原始神经外胚层肿瘤的MRI表现及病理特点分析

目的 探讨成人幕上原始神经外胚层肿瘤(SPNET)的MRI表现与病理联系,提高诊断水平.方法 搜集10例经手术病理证实的成人SPNET的临床病理及MRI资料,回顾性分析其影像学特点,并结合病理归纳其联系.男女比例为7∶3,中位年龄45.0岁,7例以头痛、呕吐首发,2例头痛伴晕厥,1例面部及舌根部麻木.均采用GE 1.5 T MRI扫描仪行MRI平扫+增强检查.结果 病灶均单发,其中额叶5例、颞叶3例、顶叶2例,1例累及胼胝体膝部,无一例术前发生转移.病灶体积大,边界清,占位效应显著,3例无水肿,5例伴轻度水肿,2例伴明显水肿,2例伴出血,未见钙化.囊实性为主6例,表现为大囊小结节,壁结节多位于大脑凸面,囊变常靠近中线,实性为主4例,可伴灶性坏死,MRI上呈等或低T1WI、高或稍高T2WI信号,增强后呈“印戒”样或“蜂窝”样强化.结论 成人SPNET的MRI表现与组织学特点密切相关,MRI表现具有特征性,仍依靠病理学确诊.     

骶部原始神经外胚层肿瘤CT和MRI影像特征

目的探讨骶部外周原始神经外胚层肿瘤(pPNETs)的CT和MRI影像特征。资料与方法回顾性分析3例骶部pPNETs患者的临床影像资料。结果骶部不同部位的pPNETs表现不同。结论骶部pPNETs虽然缺乏特征性,但一些征象可以提示pPNETs的诊断。     

Brown Tumor of the Patella Caused by Primary Hyperparathyroidism: A Case Report

It has been reported that the common sites of brown tumors are the jaw, pelvis, ribs, femurs and clavicles. We report our experience in a case of brown tumor of the patella caused by primary hyperparathyroidism. An initial radiograph and CT showed an osteolytic lesion and MR images showed a mixed solid and multiloculated cystic tumor in the right patella. One month after the parathyroidectomy, rapid bone formation was observed on both radiographs and CT images.     

Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

Peripheral primitive neuroectodermal tumor (PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior vena cava, pulmonary tumor embolism at the initial presentation is not common. We report a case of 22-year-old female with PNET of the kidney who presented with tumor embolism in the inferior vena cava (IVC) and bilateral pulmonary artery. The patient underwent surgical resection and histopathological analysis confirmed the presence of tumor within the IVC and pulmonary arteries. The patient received adjuvant chemotherapy and is currently doing well on follow-up.     

Inflammatory Pseudotumor of the Breast: a Case Report with Imaging Findings

Inflammatory pseudotumor, also known as inflammatory myofibroblastic tumor and plasma cell granuloma, is an uncommon low-grade lesion composed of spindle cells admixed with mature plasma cells and other inflammatory cells, such as histiocytes, lymphocytes, and eosinophils. Here, we describe the mammographic and ultrasonographic findings of a case of an inflammatory pseudotumor of the breast in a 60-year-old woman. With the suspicion of malignancy, core needle biopsy and surgical excision confirmed the mass as being an inflammatory pseudotumor of the breast.