黑色素恶性周围神经鞘膜瘤一例

患者男,48岁。1年前发现下腹部有一约鸡蛋大小的肿块,无疼痛、腹泻及便秘。于半年前,患者自觉肿块逐渐增大伴有间歇性腹痛及体重下降。近日来双下肢浮肿,面色苍白,间歇性隐痛较前频繁,体重下降6kg。查体:下腹部可触及20cm×20cm球形肿块,质软,界清,可移动,局部无红、肿、热、痛。 B超检查:腹部可探及实质性团块,呈假肾状,周边回声     

盆腔内恶性周围神经鞘膜瘤一例

患者　男 ,35岁。入院 6周前无明显诱因出现尿频、尿急 ,4周前出现发热 ,肛周及下腹部间断隐痛。曾被诊为盆腔脓肿 ,给予抗生素治疗 2周 ,发热症状缓解 ,但尿频、尿急症状仍在 ,且肛周及下腹部疼痛加剧。肛门指检 :前列腺左叶正常 ,右叶旁触及一个质韧、表面光滑的肿物 ,下界清楚 ,上界未触及 ,无触痛。超声检查 :前列腺及双侧精囊区可见一个类圆形混杂团块 ,以实性回声为主 ,间有近液性回声区。盆腔CT检查 :平扫于盆腔内膀胱后下方、直肠前方可见不规则分叶状肿块。肿块密度低于肌肉而且密度混杂 ,可见片状稍高密度区和较低密度区。病变与…     

恶性周围神经鞘膜瘤的临床研究(36例报告)

为探讨恶性周围神经鞘膜瘤 (MNST)的临床及肿瘤生物学特征。对 36例MNST患者的临床病理资料及随访资料进行统计分析 ,并与国外文献结果作对照。结果显示 ,MNST以 4 0～ 6 0岁年龄组患病比例最高 ,男∶女为 2 6∶1,发病部位依次为躯干 >四肢 >头颈部 ;术后复发率为 4 2 10 % ,5年存活率为 8 33% ;未发现由神经鞘瘤或神经纤维瘤病恶变所致病例 ,也未发现与放疗后恶变有关的病例。提示MNST是一种原发的软组织恶性肿瘤 ,早期手术彻底切除是治疗的关键 ,其肿瘤生物学特征与国外资料报道有所差异     

肩胛骨恶性周围神经鞘瘤一例

患者男,47岁。2年多前因扭伤左臂,感觉左肩背部疼痛,给予对症治疗后好转,时有反复,未行正规检查及处理。近1个月,左肩臂胀痛不适,对症处理无缓解。体检:左肩胛骨下份可扪及一约12cm×10cm大小的包块,边界较清,质硬,不活动,压痛(+),局部皮肤色泽、温度正常。左肩关节活动度正常。X线显示:左肩胛骨下部呈膨胀性骨质破坏,似肥皂泡样改变,边界较清晰,可见硬化缘,范围约7cm×6cm,邻近骨膜未见增生反应,亦未见明显的软组织块影(图1,2)。X线诊断:考虑骨肿瘤,以骨巨细胞瘤可能性大。手术所见:左肩胛骨下角骨质完全破坏,范围约8cm×6cm,部分边界不清…     

胸部恶性神经鞘膜瘤一例

患者　男 ,34岁。因受凉咳嗽 1月余 ,胸背痛 1周 ,自觉近日消瘦就诊。Ｘ线正位胸片示右胸中外带一约 10ｃｍ×17ｃｍ大小、边界光滑、密度均匀、类圆形致密阴影。ＣＴ平扫示右胸腔一约 10ｃｍ× 15ｃｍ大小软组织肿块 ,紧贴外侧胸壁 ,ＣＴ值 10～ 32ＨＵ ,右肺组织明显受压 (图 1、2 )。ＣＴ诊断 :右胸腔内巨大占位性病变 ,考虑胸膜恶性肿瘤可能性大。术后病理结果为恶性神经鞘膜瘤。讨论　恶性神经鞘膜瘤占软组织肉瘤的 10 % ,好发于四肢及躯干等体表部位 ,发生在胸部少见。胸部神经鞘膜瘤多起源于脊神经 ,病灶多见于后纵隔脊椎旁区 ,少数…     

恶性外周神经鞘膜瘤的MRI诊断

目的 总结恶性外周神经鞘膜瘤(MPNST) MRI表现,提高对本病的认识. 方法 回顾性分析12例经手术、病理证实的MPNST患者的临床和MRI表现.结果 12例中9例合并神经纤维瘤病Ⅰ型.7例发生于脊神经根,1例位于大腿根部,骨盆骶髂骨1例,肘部1例、椎管内髓外硬膜下1例及大腿下段1例.9例(9/12,75％)皮肤、皮下有多发小结节.2例有溶骨性骨质破坏.7例T2WI上肿块沿神经生长,浸润包绕神经,肿块呈纺锤形、多结节融合型,信号混杂,有出血、坏死表现.T2WI上周围多发神经纤维瘤呈蠕虫样、小靶环样.其中恶变的肿块内仍可见“靶征”,但结节增大,实性成分增加,周围高信号环缩小或消失,“靶征”变得不典型甚至消失,肿块实性部分强化明显.结论 大部分MPNST的临床及MRI具有一定的特点,术前可以诊断.     

恶性周围神经鞘膜瘤摄取骨显像剂一例

患者男，62岁，右侧腹痛2个月，腹膜后神经鞘膜瘤化疗栓塞术后1个月。2个月前患者出现右侧腹痛，外院CT检查见右腹膜后肿块，于本院泌尿外科行手术治疗。术中肿块无法切除，术后病理检查诊断为外周神经鞘膜瘤（低度恶性，图1），后转入介入病房行腹膜后神经鞘膜瘤化疗栓塞术，体格检查：右上腹见一长约30cm手术瘢痕，     

乳腺恶性神经鞘瘤一例

病例资料患者,女,60岁,发现左乳肿块伴疼痛2个月。体检:左乳晕后区扪及4.5cm×5.0cm大小的肿块,质硬,边界尚清,活动度可,稍有压痛。实验室检查未见异常。高频钼靶X线表现:左乳晕后区偏内侧见一4.5cm×4.2cm大小的类圆形致密影,密度尚均匀,边界清晰,其内可见粗颗粒状钙化灶,周围血运增加,皮下脂肪层模糊,腋下未见肿大淋巴结影。结合病史诊断:左乳腺占位,肉瘤可能性大。手术病理大体标本所见:肿瘤呈灰红色的软组织肿块,大小为5.7cm×4.7cm×2.5cm,切面见一灰白结节,大小为3.8cm×3.3cm,切面灰白小区有出血,质中偏嫩,似有包膜。病理组织学检查…     

肋骨巨大恶性神经鞘膜瘤1例

恶性神经鞘膜瘤是由神经鞘细胞、神经束衣和神经内衣细胞组成的恶性肿瘤。本病少见 ,巨大者更罕见。现就我院经手术和病理证实的 1例恶性神经鞘膜瘤报道如下。1　病历简介女 ,8岁 ,偶感右背部疼痛 3年 ,晚上略明显 ,未在意。半月前右背部疼痛加剧 ,晚上更明显 ,即入院诊治。体格检查 :除背部压痛、以肩胛间 4— 6肋脊柱旁最明显外 ,余未发现阳性体征。Ｘ线胸片 :右后上胸壁可见软组织包块影 ,大小约 4 5ｃｍ×4 5ｃｍ× 6 0ｃｍ ,向前突向肺野 ,密度均匀 ,界限清晰 ,第 4后肋向上推压移位 ,并且可见硬化边 ,第 5后肋可见溶骨性破坏 ,骨皮质…     

心包恶性神经鞘膜瘤一例

患者　男 ,33岁。外伤后左胸疼痛、胸闷不适 1月余 ,曾昏厥 1次。胸部平片显示心影增大 ,心缘不规则 ,左肋膈角填塞 (图 1)。CT检查 :心包内见巨大混杂密度肿块 ,大小15cm× 13cm左右 ,边缘结节状 ,实质部分明显强化 ,病灶与大血管根部、左心房、左心室分界不清 ;双侧胸腔积液     

小腿恶性神经鞘膜瘤一例

患者 男,87岁.1年来左小腿胫前包块逐渐增大,发热1周入院.体检:体温39.2℃,左小腿中段前部可见大小约5 cm×5 cm的包块,呈浅褐色,质中,皮温高,活动差,无压痛.实验室检查:C反应蛋白108 mg/L,血沉28 mm/1 h,梅毒螺旋体(TP)抗体阳性,血清梅毒实验阴性,结核抗体阳性.既往有肺结核病史50年.     

Multicentric malignant peripheral nerve sheath tumor

We present a case of malignant peripheral nerve sheath tumor of multicentric origin, an extremely rare condition. A 25-year-old man was admitted to hospital with presenting symptoms of cough, dyspnea and left lateral back pain. Computed tomography and magnetic resonance imaging revealed extrapleural masses in the left hemithorax in addition to synchronous left inguinal mass. After surgical resection of the masses from the thoracic and inguinal regions, histological examination confirmed the preoperative diagnosis of malignant peripheral nerve sheath tumor.     

CT of malignant peripheral nerve sheath tumor

Malignant peripheral nerve sheath tumors are soft tissue sarcomas that typically arise from a neurofibroma. Patients with neurofibromatosis type 1 represent approximately half of the population diagnosed with these tumors. This autosomal-dominant genetic disorder is distinguished by loss-of-function mutations in the neurofibromin 1 gene, which ultimately promotes atypical cellular proliferation. These biologically aggressive tumors are associated with a poor prognosis as they are resistant to available therapies and have high rates of recurrence, progression, and mortality. In this article, we report the case of a 45-year-old male with a history of neurofibromatosis type 1 who was diagnosed with a malignant peripheral nerve sheath tumor. We focus on optimizing diagnosis and treatment through the application of radiological imaging modalities, including cinematic rendering.     

食管恶性外周神经鞘膜瘤1例

患者男,71岁。因无明显诱因出现进行性吞咽困难1月就诊。体检和实验室检查未发现异常。上消化道钡剂双对比造影显示食管下段偏心性不规则狭窄,壁僵硬破坏,病变长约9.0cm,其内多发不规则结节状充盈缺损,黏膜不规则中断,见多发小毛刺样腔内龛影,病变累及贲门,贲门处见圆形充盈缺损,余各段食管未见明确异常,钡剂通过未见明确受阻(图1,2)。     

外周恶性神经鞘膜瘤的影像学表现

目的:总结分析外周恶性神经鞘膜瘤的影像学表现,提高鉴别诊断能力。方法:回顾性分析11例经手术或病理证实的恶性神经鞘膜瘤的影像学表现,并与手术病理对照分析,11例中行CT检查3例,行MRI检查4例,CT和MRI均检查4例。结果:CT平扫表现为等、低混合密度软组织肿块影,形态不规则,病灶边缘较光整2例,边缘毛糙5例。CT增强扫描呈不均匀强化,坏死区不强化,网格样强化2例,不均匀强化5例。T1WI呈等、略低信号,内见局灶性长T1信号影;T2WI序列以略高信号为主,内见局灶性长T2信号,周围软组织见片状浸润高信号;MRI增强扫描呈不均匀强化。结论:恶性外周神经鞘膜瘤的CT和MRI表现具有一定的特征性。     

Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that almost always arise in soft tissue. They can develop in pre-existing neurofibromas or schwannomas, de novo from peripheral nerves, or following radiation therapy. Primary intraosseous MPNST is rare and has been reported most frequently in the mandible. Of the reported cases involving the long bones, none has been associated with neurofibromatosis type 1 (NF-1). We report a case of MPNST arising in the femur in a patient with NF-1.     

Retroperitoneal malignant peripheral nerve sheath tumor: evaluation with serial FDG-PET

Retroperitoneal malignant peripheral nerve sheath tumor (MPNST), a rare type of neurogenic tumor, was diagnosed in a 14-year-old girl with a history of neurofibromatosis type 1 (NF1). Immunochemistry demonstrated spindle cells positive for S-100 protein. The patient had multiple tumor recurrences and she was evaluated with serial F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET). A tumor in the right iliac wing showed increased FDG uptake on PET. FDG-PET played an important role in therapy planning and subsequent follow up. This case emphasizes the important role FDG-PET could play in the staging, restaging, and posttherapy follow up of MPNST.     

Perineural invasion through the maxillary division of the right trigeminal nerve in a rare case of nasolabial malignant peripheral nerve sheath tumor

SUMMARY: We report the CT and MR imaging findings in a patient with malignant peripheral nerve sheath tumor in the right nasolabial area, which exhibited typical imaging features of cystic adenoid carcinoma.     

A giant malignant peripheral nerve sheath tumour of the breast: CT and pathological findings

Malignant peripheral nerve sheath tumour (MPNST) is a rare soft tissue sarcoma. In particular, primary MPNST of the breast is extremely rare. We report a case of a giant malignant peripheral nerve sheath tumour involving the entire right breast, which was not associated with neurofibromatosis type 1 (NF-1) or previous radiation therapy. A CT scan showed a huge heterogeneous soft tissue mass with well-enhanced nodules at its periphery and low-density internal necrosis, which was confirmed by modified radical mastectomy.