肺原发性恶性外周神经鞘瘤的临床病理分析

目的 探讨肺脏原发性恶性外周神经鞘瘤(MPNST)的临床病理学特征及诊断、鉴别诊断要点。方法 对2例肺MPNST进行临床病理学分析及免疫组织化学与超微结构研究。结果 2例光镜下均显示MPNST的形态特点，免疫组织化学显示S-100蛋白、MBP、Vim、NSE肿瘤细胞呈阳性表达，电镜观察1例可见特征性Luse小体。结论 肺原发性MPNST极为罕见，临床诊断较困难。根据其光镜病理形态特征，S-100蛋白、MBP免疫组织化学检测和(或)电镜检查可确诊。结合文献，该肿瘤早期无明显临床症状，一般发现时已属晚期，预后差。病理学上应与肺脏平滑肌肉瘤、纤维肉瘤、单相型滑膜肉瘤、肉瘤样癌鉴别。     

恶性外周神经鞘瘤临床与病理特征

目的　探讨恶性外周神经鞘瘤（MPNST）的临床与病理形态特征。 方法　收集本院收治的22例MPNST临床资料，应用光学显微镜观察病理形态特点，免疫组化分析其表型，并进行相关文献复习。 结果　22例MPNST中，男10例，女12例，年龄15~82岁，中位年龄43岁。头颈部3例，躯干及四肢近端13例，四肢远端5例，全身多发1例。临床表现主要是局部逐渐增大的无痛性肿块，症状与体征与肿块的部位及进展速度有关。19例患者行肿瘤切除手术，术后其中6例患者被嘱须行后续放疗，13例患者行化疗（以多柔比星和异环磷酰胺为主）。免疫组化检测，Vimentin（15/15）、CD99（8/8）、IMP3（10/11）、S-100蛋白（16/19）阳性，Ki-67增殖指数5%~80%。1年病死率45%，中位生存时间25个月。局部复发率55%（12例），远处转移率32%（7例）。末次随访3例无瘤生存。 结论　MPNST的临床及病理有其特点，但某些医生对其认识不足。本文总结辨析要点，以期提高该病确诊率，指导治疗和康复。     

伴菊形团样结构的恶性外周神经鞘膜瘤1例

患者男性,33岁,因枕部包块术后2年、复发1年入院.1年前在无明显诱因下,再次出现相同部位包块,质韧,压痛,较前增大,无恶心,呕吐.查体：枕部可触及一4.0 cm×3.0 cm包块.TOPO定位：左侧枕部皮下软组织内可见梭形软组织块影,密度尚均匀,测CT值约56 Hu,相邻颅骨外板骨质结构完整,未见明显受压及骨质破坏征象,两侧大脑半球密度均匀,灰白色,分界清,未见明显异常密度影.各脑室系统及大脑表面沟池显示正常.大脑中线结构居中.CT示：（1）左侧枕部皮下占位,（2）颅脑平扫颅内未见明显异常.临床诊断：左枕部纤维瘤[第一段]     

恶性外周神经鞘瘤临床与病理特征

目的　探讨恶性外周神经鞘瘤（MPNST）的临床与病理形态特征。 方法　收集本院收治的22例MPNST临床资料,应用光学显微镜观察病理形态特点,免疫组化分析其表型,并进行相关文献复习。 结果　22例MPNST中,男10例,女12例,年龄15~82岁,中位年龄43岁。头颈部3例,躯干及四肢近端13例,四肢远端5例,全身多发1例。临床表现主要是局部逐渐增大的无痛性肿块,症状与体征与肿块的部位及进展速度有关。19例患者行肿瘤切除手术,术后其中6例患者被嘱须行后续放疗,13例患者行化疗（以多柔比星和异环磷酰胺为主）。免疫组化检测,Vimentin（15/15）、CD99（8/8）、IMP3（10/11）、S-100蛋白（16/19）阳性,Ki-67增殖指数5%~80%。1年病死率45%,中位生存时间25个月。局部复发率55%（12例）,远处转移率32%（7例）。末次随访3例无瘤生存。 结论　MPNST的临床及病理有其特点,但某些医生对其认识不足。本文总结辨析要点,以期提高该病确诊率,指导治疗和康复。     

直肠伴有骨形成的恶性外周神经鞘瘤一例

患者男 ,6 1岁。数月前解大便时有里急后重感并有少许黏液血便 ,大便每天 2～ 3次 ,成型 ,于 2 0 0 1年 11月 2 2日入院。体检 :全身皮肤无色素沉着 ,躯干及四肢无瘤结 ,浅表淋巴结未扪及。胸部X光片、腹部B超及全身骨扫描未见异常。肛门指检 :距肛门 3～ 4cm处扪及 3cm× 3cm肿物 ,质软 ,前列腺不大 ,无结节 ,指套上染有血迹。直肠镜检 :直肠下段距齿状缘 3cm处有一结节状肿物突向肠腔 ,取少许组织送检 ,病理诊断为“直肠恶性肿瘤 ,具体组织学类型待手术切除后定”。临床疑“直肠癌”行根治切除手术。病理检查 :带有肛门组织的肠一段 ,长 …     

良、恶性外周神经鞘膜肿瘤新类型和少见亚型的病理诊断

外周神经肿瘤的类型和亚型繁多 ,组织形态多变 ,造成病理诊断、鉴别诊断的困难 ,尤其是恶性外周神经鞘膜瘤的诊断更为困难。本文就近年来有关良、恶性外周神经鞘膜肿瘤的新类型和少见亚型进行了文献复习 ,并简介如下 ,仅供同仁们参考。1　神经鞘瘤及亚型 (ｎｅｕｒｉｌｅｍｏｍａｏｒｓｃｈｗａｎｎｏｍａａｎｄｔｈｅｉｒｖａｒｉａｎｔｓ)神经鞘瘤是最常见的一种良性外周神经鞘膜肿瘤。一般不伴有神经纤维瘤病。罕见恶变 ,有报道发生血管肉瘤者。神经鞘瘤的亚型颇多〔1〕,下面仅就其中的几种重点介绍。1.1　细胞性神经鞘瘤 (ｃｅｌｌｕｌ…     

背部巨大恶性外周神经鞘瘤1例

患者女性,57岁,因"背部巨大肿物"入院.患者3年前无意中发现背部"枣子"大小肿物,不伴疼痛,无发热.曾在外院行3次手术切除,具体情况不详,但均术后复发.体检:患者背部可见1约45 cm×30 cm×30 cm大小肿物,质较硬,界限清,活动度可,表面部分破溃,有淡黄色液体溢出.另于全身皮肤散在多个"疣状"突起,手术切除巨大肿块.临床诊断:神经纤维瘤伴感染(背部).     

富于细胞性神经鞘瘤30例临床病理分析

目的 探讨富于细胞性神经鞘瘤(cellular schwannoma,CS)的临床病理学特点、免疫表型及鉴别诊断.方法 回顾性分析2014～2020年北京积水潭医院诊治的30例CS的临床及影像学特点、病理学特征及免疫表型等,并复习相关文献.结果 30例CS中,女性22例,男性8例,年龄12～65岁,平均44.6岁.发生...     

胃神经鞘瘤临床病理分析

目的：探讨胃神经鞘瘤（gastric schwannoma）的临床病理学特点、免疫表型、诊断及鉴别诊断。方法：对8例胃神经鞘瘤的临床特点、组织形态学和免疫组织化学结果进行分析,并复习相关文献。结果：患者女性5例,男性3例,平均年龄61岁（30~81岁）。8例胃神经鞘瘤直径1.0~7.0 cm（平均3.0 cm）,无包膜,切面灰白色,镜下,瘤细胞短梭形,束状、栅栏状排列,肿瘤的边缘见淋巴细胞套。免疫组织化学染色：S-100全阳性,胶质纤维酸性蛋白（glial fibrillary acidic protein,GFAP）7例阳性,CD117、CD34、Dog1、a-平滑肌肌动蛋白（a-Smooth muscle actin,a-SMA）、结蛋白（Desmin）阴性。3例发现血小板衍化生长因子受体（platelet-derived growth factor receptor,PDGFR）阳性,但未发现c-kit和PDGFRα的基因突变。结论：胃神经鞘瘤是一种良性肿瘤,需与胃肠道间质瘤鉴别。     

会阴部丛状神经鞘瘤临床病理分析

目的 探讨会阴部丛状神经鞘瘤(plexiform neurilem-moma,PN)临床病理特点和鉴别诊断.方法 对1例发生在会阴部的PN进行组织形态学观察、免疫组化标记并复习文献.结果 患者女,31岁,会阴部肿块渐增大4年,无疼痛.边界清楚,切面灰白,实性,呈多结节状,质地中等.镜检:肿物位于皮下,约有30个大小不等的结节组成,直径0.5～2.0cm不等,每个结节均见纤维包膜包绕,大多数结节以细胞致密区(Antoni A)为主,无或少有细胞疏松区(Antoni B).结节内瘤细胞呈长梭形,核呈锥形、仿锤形或波浪状,呈栅栏状和螺旋状排列,可见verocay小体.免疫组化标记显示:瘤细胞vimentin和S-100蛋白弥漫阳性,GFAP与GPG 9.5部分呈阳性.结节外包膜EMA、CD34阳性,瘤细胞阴性.NSE、CD57、ER、PR、CD68、α-SMA、desmin和H-Caldesmon均阴性.结论 丛状神经鞘瘤是一种具有特殊形态学特点的外周神经肿瘤,应与丛状神经纤维瘤、丛状纤维组织细胞和婴儿和儿童丛状恶性外周神经鞘膜瘤等相鉴别.     

Malignant peripheral nerve sheath tumor with perineurial cell differentiation (malignant perineurioma)

A unique case of malignant peripheral nerve sheath tumor (MPNST) with perineurial cell differentiation occurring in a 63-year-old woman in a subcutis of the forearm is described. The tumor contained cellular and myxoid areas. The neoplastic cells were fusiform with distinct cell borders. They were arranged in storiform pattern and in wavy parallel cell cords in the cellular areas. Focally, a pleomorphism and mitotic activity (including atypical mitoses) similar to those of malignant fibrous histiocytoma were seen. The myxoid parts contained haphazardly oriented cells and scarce lipoblast-like multivacuolated cells mimicking a liposarcoma. In the differential diagnosis, myxoid liposarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma and low-grade fibromyxoid sarcoma were considered. Immunohistochemically, perineurial differentiation was indicated by the diffuse expression of epithelial membrane antigen and focal reactivity for CD34. The tumor was negative with antibodies to S-100 protein, Leu-7, CD68 (KP1), vimentin and cytokeratin AE1/AE3. Ultrastructure of tumor cells revealed features of MPNST. No recurrence occurred in the patient during 2 years follow up.     

Malignant peripheral nerve sheath tumor of the thyroid: A clinicopathological and ultrastructural study of one case

Thyroid malignant peripheral nerve sheath tumors (TMPNST) are very uncommon neoplasms that can be confused with anaplastic carcinoma, Riedel’s thyroiditis, or other soft tissue tumors that may occur in the thyroid region. An example of TMPNST is presented in this report. The tumor occurred in a 56-yr-old woman. Fine needle aspiration did not provide adequate material. After thyroidectomy, the lesion posed important problems in differential diagnosis. Immunohistochemical, molecular, and electron microscopic features were taken into consideration to arrive at the correct diagnosis. Tumor cells were focally positive for keratins, a feature that has not been described in peripheral nerve sheath tumors of the thyroid, but that has been occasionally seen in tumors from other locations. After thyroidectomy, the patient received radiotherapy. She is well without evidence of recurrence 10 mo after surgery.     

Malignant transformation of neurofibromatosis-1 into low-grade malignant peripheral nerve sheath tumor: a case report

Malignant Peripheral Nerve Sheath Tumor (MPNST) is a malignant mesenchymal tumor. The majority of MPNSTs are found in patients with neurofibromatosis type 1 (NF-1) who have a high-grade sarcoma. At the moment, there are just a few instances of low-grade MPNST caused by NF-1. We present a case of malignant transformation of NF-1 into low-grade MPNST in a patient with a long history of the disease. Multiple protruding masses with ulceration on the right shoulder and chest wall were discovered during physical examination. Complete tumor excision was done, followed by hematoxylin-eosin and immunohistochemical staining. A portion of the tumor had higher cellularity, hyperchromatic cell nuclei, and mitoses were seen in only five out of ten high-power fields. S-100 and vimentin were positive, whereas cytokeratin, desmin, SMA, and CD34 were negative. Ki-67 (MIB1) labeling index hot-spotting was around 25%. This was thought to be NF-1 malignant transformation into low-grade MPNST. Overall, knowing the clinical and pathologic characteristics of the disease, plus growing knowledge or experience with the condition, may improve preoperative diagnostic accuracy and extending survival time.     

Fine‐needle aspiration cytology of epithelioid malignant peripheral nerve sheath tumor: A case report and review of the literature

The epithelioid variant of malignant peripheral nerve sheath tumor (eMPNST) is an extremely rare soft tissue neoplasm comprising less than 5% of all MPNSTs. It is distinguished cytomorphologically from a conventional MPNST by the presence of polymorphous round epithelioid cells arranged in loose clusters with or without spindled tumor cells. These features pose a diagnostic challenge because the differential diagnosis involves a variety of mesenchymal and non‐mesenchymal tumors including epithelioid sarcoma, sclerosing epithelioid fibrosarcoma, malignant rhabdoid tumor, chordoma, metastatic carcinomas, and melanoma. Thus, it may become imperative to perform immunochemical stains on cell blocks of FNA aspirates to arrive at definitive diagnosis. Reports describing the cytologic features of eMPNST are rare. Herein, we report a case of eMPNST with focus on cytomorphologic and cytoimmunochemical features and differential diagnosis. Diagn. Cytopathol. 2016;44:226–231. © 2015 Wiley Periodicals, Inc.     

Malignant peripheral nerve sheath tumor with prominent intracytoplasmic vacuolation: Report of a case

A case of malignant peripheral nerve sheath tumor with uncommon features is reported. A mass was noted in the left thigh of a 16 year old man. Histologically, most areas of the tumor exhibited the typical appearance of malignant peripheral nerve sheath tumor, but some tumor cells had rounded nuclei and cytoplasm, resembling an epithelioid pattern. It was noted that some rounded tumor cells showed prominent intracytoplasmic vacuolation. Immunohistochemically, almost all of the tumor cells, including the rounded and vacuolated ones, were positive for S-100 protein and vimentin. Electron microscopic study revealed well-developed cytoplasmic processes, intracytoplasmic intermediate-sized filaments, basement lamina formation and extracellular long-spacing collagens. These findings were compatible with those of Schwann cell differentiation. Moreover, ultrastructurally, the vacuolated spaces contained a few granular materials and were derived from the dilatation of the rough endoplasmic reticulum. It is speculated that intracytoplasmic vacuolation in malignant peripheral nerve sheath tumor would be caused by degeneration of the tumor cells.     

上皮样恶性周围性神经鞘瘤临床病理分析

目的 探讨上皮样恶性周围性神经鞘瘤(epithelioid malignant peripheral nerye sheath tumor,EMPNST)的临床病理特征及鉴别诊断.方法 收集9例EMPNST的临床病理资料,行光镜和EnVision法免疫组化观察,并复习文献.结果 9例EMPNST,女性4例;年龄20～67岁,中位年龄37.5岁;病变主要位于四肢,上肢3例,下肢4例,右季肋部和咽隐窝各1例;>5 cm 7例,其中1例>10 cm;<5 cm 2例,平均6.2 cm,无包膜.深在型8例,浅在型1例,组织学,纯上皮样型5例,其中2例见节细胞样或横纹肌样瘤样区域,4例混合型伴有梭形细胞区.免疫表型S-100蛋白及NSE 9例均呈阳性反应,纯上皮样型5例S-100蛋白呈强阳性,4例混合型呈灶性阳性,8例PGP 9.5阳性,7例MBP阳性,5例EMA灶性或弱阳性,4例vimentin阳性,3例CD57灶性阳性,而HMB-45、desmin、CD34、CK阴性.结论 EMPNST是恶性周围性神经鞘瘤的一种少见亚型,形态学上缺乏特征性,易与其他软组织上皮样肿瘤混淆.S-100蛋白及PGP9.5阳性是诊断EMPNST有价值的指标,但缺乏特异性,因此诊断时必须结合临床、组织形态和免疫表型的结果,综合判断以免引起误诊.