恶性外周神经鞘膜瘤7例临床病理分析

目的 探讨恶性外周神经鞘膜瘤的临床病理特点.方法 对7例恶性外周神经鞘膜瘤经显微检查及免疫组化证实,观察其镜下形态改变.结果 恶性外周神经鞘膜瘤形态多样,免疫组化S-100,Leu7,MBP是敏感、可靠的标记物,其联合使用有助于明确诊断.结论 恶性外周神经鞘膜瘤组织镜下形态多变,差异很大,常易误诊其它肿瘤,免疫标记有助于该肿瘤的诊断.     

非典型脊膜瘤和神经纤维瘤的碰撞瘤1 例

Hokari等［1］首次报道椎管内可同时存在两种不同的病变,然而在排除神经纤维瘤病的患者的椎管内碰撞瘤仅报道有2例［2］。现对1例排除神经纤维瘤病的患者被诊断为非典型脊膜瘤和神经纤维瘤碰撞瘤进行报道,旨在提高神经肿瘤外科医生对此病的认识并进行相应的精准治疗。     

神经纤维瘤病Ⅰ型伴下肢恶性神经鞘瘤1例

1病例摘要 患者男，36岁，蒙古族，全身体表多发结节20年，左大腿肿物8个月并切除术后4个月，肿物复发伴疼痛1个月人院。患者20年前无诱因出现躯干及四肢体表多发结节，生长缓慢，未予处理。8个月前左大腿后外侧出现一肿物，生长迅速，起始直径约3．0cm，短期内达14cm，于4个月前行手术切除，术后未做病理检查。1个月前原部位肿物复发并迅速增大，伴局部及左下肢疼痛。家族中其母亲有类似体表结节症状，未治疗，仍健在。查体：头面部、前胸后背及四肢皮肤可见大量结节性肿物，直径0．5cm～2．5cm，大者呈皮赘状下垂，质软，无压痛。后背部分皮肤呈片状咖啡色素斑。左大腿中段后外侧可见长15cm纵形手术切口瘢痕，局部膨隆，可触及直径约13cm肿物，质硬，活动度小，压痛明显，左下肢肌力Ⅲ级，直腿抬高试验阳性。彩超示：左大腿后外侧肌层内实质性不规则肿物。CT示：左大腿后外侧实质性肿物，股骨局部骨质破坏。临床诊断：神经纤维瘤病Ⅰ型，左下肢神经纤维瘤术后复发，恶变可能。硬膜外麻醉下行肿物切除，术中见肿物呈灰黄色，包膜较完整，包绕坐骨神经干，与神经干分界不清，分块全部切除肿物，保留坐骨神经。免疫组化：S-100（＋），Vimentin（＋），actin（-），SMA（-），EMA（-），病理诊断：恶性神经鞘瘤。切口Ⅰ期愈合，回当地放疗，目前随访中。     

枕骨恶性外周神经鞘膜瘤1例报道

恶性外周神经鞘膜瘤（malignant peripheral nerve sheath tumors,MPNST）又称为恶性神经鞘瘤、恶性施万细胞瘤（ma-lignant schwannoma）、神经纤维肉瘤（neurofibrosarcoma）或神经源性肉瘤（neurogenic sarcoma）,是一种较少见的来源于神经软组的织恶性肿瘤。恶性外周神经鞘瘤50％发生在躯干,30％发生在四肢,头颈部大约占20％,颅部发生率较低,多起源于三叉神经或听神经［1］。本文报告我科近期收治的1例发生在枕骨的恶性外周神经鞘膜瘤,现报道如下。     

椎管内非典型神经鞘瘤和非典型脊膜瘤的MRI鉴别诊断

目的:分析椎管内非典型神经鞘瘤（intraspinal atypical schwannoma,IAS）和非典型脊膜瘤（atypical spinal meningioma,ASM）的MRI征象和临床特点,讨论两者的MRI鉴别诊断。方法:回顾性分析80例我院2017年01月至2020年07月经病理证实的IAS和ASM的术前MRI图像,提取其影像特征,运用SPSS分析软件对它们的MRI特征及临床特点进行统计分析。结果:IAS组平均年龄(51.5±10.1)岁,ASM组平均年龄(61.1±12.9)岁;两组在性别、年龄及发病部位上具有非常显著的统计学差异（P均＜0.01）。在形态学上IAS组和ASM组的纵径、横径及纵横径比之间的差异均无统计学意义（P均＞0.05）。在MRI信号特点（T1WI、T2WI信号强度、T2WI信号混杂性、是否囊变）及强化特征（强化程度、强化均匀性、是否环形强化）方面两组之间的差异均具有非常显著的统计学意义（P均＜0.01）。结论:区分IAS和ASM的MRI征象和临床特点,对其鉴别具有参考价值。     

恶性外周神经鞘膜瘤的预后相关因素分析

目的 探讨影响恶性外周神经鞘膜瘤(MPNST)患者预后的相关因素.方法 回顾性分析2010年9月至2019年12月33例MPNST患者的临床病理资料,其中男性15例,女性18例;中位年龄42岁(范围:3～69岁).神经纤维瘤病1(NF-1)型11例,散发型22例;四肢部位20例,躯干部位13例;手术切缘阴性28例,手术...     

多发性恶性神经鞘瘤1例

临床资料患者女性 ,38岁 ,因扭伤后肩痛 65天 ,右手麻木 4 5天 ,加重伴无力 32天入院。查体 :右上肢肌力Ⅳ～Ⅴ级 ,伴Ｃ7 Ｔ1痛觉减退。肌电图示 :右侧大小鱼际肌失神经损害 ,右侧尺神经运动传导速度减慢。入院诊断 :右臂丛神经损伤原因待查。入院后给予Ｂ族维生素、激素、能量合剂、镇痛等治疗 ,效果不佳 ,右上肢无力进行性加重。 35天后发现右胸部包块 ,约 2ｃｍ× 2ｃｍ。针吸细胞学检查示 :恶性肿瘤 ,多系神经鞘瘤 ,不排除转移性肿瘤。 71天后患者出现下肢无力 ,右下肢麻木。ＭＲＩ检查提示Ｔ7平面椎管内肿瘤 ,行椎管内肿瘤部分切除术 ,…     

多发性恶性神经鞘瘤1例

临床资料 患者女性，38岁，因扭伤后肩痛65天，右手麻木45天，加重伴无力32天入院。查体：右上肢肌力Ⅳ～Ⅴ级，伴C7-T1痛觉减退。肌电图示：右侧大小鱼际肌失神经损害，右侧尺神经运动传导速度减慢。入院诊断：右臂丛……     

The atypical Spitz tumor of uncertain biologic potential

BACKGROUND:

Atypical Spitz tumors (AST) are rare spitzoid melanocytic proliferations with an uncertain malignant potential. ASTs have overlapping features of both Spitz nevi and spitzoid melanoma, and consequently generate controversy with diagnosis and management. Sentinel lymph node biopsy (SLNB) has been proposed as a possible means to gain additional insight into the true biologic potential of these tumors; however, previous reports on the use of SLNB in ASTs have been limited by small numbers of patients and short durations of follow‐up.

METHODS:

The authors extracted data from their institution's prospective melanoma database, collected between 1994 and 2007, for all patients with ASTs of uncertain biologic potential. They reviewed the clinical features of these patients, including the sentinel lymph node status, and the histological features of the tumors.

RESULTS:

A total of 67 patients with ASTs were identified, with a median age of 23.7 years. The mean depth was 2.4 mm. Of these, 57 had a SLNB performed, with 27 (47%) having a positive sentinel lymph node. SLNB‐positive cases had a significantly lower mean age than SLNB‐negative cases (17.9 vs 28.7 years; P = .013); however, no other significant differences were observed. All 27 patients with a positive SLNB were alive and disease free with median follow‐up of 43.8 months. One patient who did not receive a SLNB developed recurrent disease with regional and distant metastases.

CONCLUSIONS:

ASTs do not appear to behave like conventional melanoma. There is a high incidence of microscopic lymph node deposits in SLNBs, but despite this finding, patients have a favorable prognosis. Our findings raise several questions regarding the malignant potential of ASTs, and the role of SLNB in their management. Cancer 2009. © 2009 American Cancer Society.     

182例胰腺神经内分泌肿瘤的临床病理特征和预后分析

  目的  探讨胰腺神经内分泌肿瘤（pancreatic neuroendocrine neoplasm,PanNEN）的临床病理特征和预后因素。  方法  收集2011年1月至2018年12月天津医科大学肿瘤医院收治的胰腺NEN,依据世界卫生组织（WHO）消化系统肿瘤2019版分类进行复核,分析比较不同分类胰腺NEN临床病理特征的异同,并分别进行生存分析。  结果  在最终确诊的182例PanNEN中,神经内分泌瘤（neuroendocrine tumor,NET）G1、NET G2、NET G3、神经内分泌癌（neuroendocrine carcinoma,NEC）和混合性神经内分泌-非神经内分泌肿瘤（mixed neuroendocrine-non-neuroendocrine neoplasm,MiNEN）分别为78例（42.9%）、82例（45.1%）、5例（2.7%）、15例（8.2%）和2例（1.1%）。临床病理特征方面,胰腺NEN分级越高,神经/脉管侵犯、淋巴结/远处转移等侵袭性行为越多见,诊断时进展期患者的比例越高（均P < 0.05）。NEC的Ki-67指数均值显著高于NET G3（P < 0.001）,但二者在30%~60%区间有重叠。WHO2019版分类与总体生存（overall survival,OS）和无进展生存（progression-free survival,PFS）显著相关（均P < 0.05）。对于NET G1患者,诊断时为进展期是OS和PFS较差的独立预后因素（分别HR=12.472,P=0.002;HR=10.56,P < 0.0012）。对于NET G2患者,手术切除是OS较好的独立预后因素（HR=8.217,P=0.001）,诊断时即有远处转移是PFS较差的独立预后因素（HR=26.137,P < 0.001）。NEN G3的预后主要与Ki-67指数有关,但NET G3和NEC的截断值不同（NET G3:45%,NEC:70%）。  结论  胰腺NEN是一组异质性肿瘤,不同WHO分类的胰腺NEN的临床病理特征及预后均不同。胰腺NEN以分化好的NET为主,但是部分NET在诊断时即见转移,术后可以复发/转移。NET G3与NEC的鉴别要点主要是肿瘤分化、细胞增殖活性、p53免疫组织化学染色等分子检测。治疗方面仍无统一标准,尤其对于未明确NET G3或NEC的NEN G3患者,需综合评估分级、分期,并监测疾病进展情况。未来仍需多中心大样本的研究来制定更加全面细致的诊疗标准。      

Clinicopathological study of seven cases of symptomatic supratentorial subependymoma

Subependymomas are rare, slow-growing tumors, the majority of which are found incidentally at postmortem examination. The authors retrospectively analyzed seven cases of symptomatic supratentorial subependymomas.Five were females and two were males, ranging in age at operation of 6–50 years (median 45). The follow-up period ranged from 1.5 to 8.3 years. Tumors were intraventricularly located as a lobulated mass with cystic changes: four in the frontal horn, two in the trigone, and one in the third ventricle. Moderate to marked enhancement was noted in two tumors of the trigone and in one tumor of the frontal horn on both CT scan and MR imaging. MR spectroscopy of a recurrent subependymoma demonstrated a higher Cho/Cr ratio of 2.66, compared with a Cho/Cr ratio (0.48) of a non-recurrent subependymoma. Angiography, which was performed in four patients, revealed no staining in two and delayed modest staining in two. Radiosurgery was performed in two patients but was ineffective. Five patients with gross total tumor resection showed no evidence of tumor recurrence to the last follow-up. The two subtotally resected trigonal tumors progressed two years after operation. No histological difference except MIB-1 index was noted between recurrent and non-recurrent cases.In conclusion, we suggest that subependymoma could show progressive biological behavior, especially in cases of markedly enhancing, irregularly contoured, large tumors located in the trigone. For symptomatic supratentorial subependymomas, gross total resection is the treatment of choice and radiation has little effect on tumor control.     

10例宫颈原位腺癌临床病理特征分析

目的:探讨宫颈原位腺癌（adenocarcinoma in situ,AIS）的临床病理特征和诊断要点。方法:回顾性分析某医院病理科存档的10例宫颈原位腺癌患者的临床及病理资料,并复习相关文献。结果:年龄31~51岁,平均年龄44岁。7例表现为腰痛、异常的阴道流血及排液,3例无明显的临床症状,因HPV检测阳性而行宫颈活检确诊。9例为宫颈内膜型,1例为复层产黏液性上皮内病变（SMILE）;5例为单纯宫颈原位腺癌,5例伴有高级别鳞状上皮内病变（HSIL）。镜下观察,单纯性AIS腺体仍保持原有的结构特征,但细胞异型性明显,细胞核拥挤、深染、重叠,核分裂象多见,可见凋亡小体。SMILE肿瘤细胞由复层上皮细胞构成,可见富含黏液细胞或具有产黏液趋势,巢团状排列。10例细胞不同程度阳性表达p16、CEA,Ki67增殖指数均>20%;术后1例进展为浸润性腺癌,2例失访,余均无病生存。结论:宫颈原位腺癌缺乏典型的临床表现且病变位置较深,具有相对特征的形态学改变和免疫表型,需要充分的病理取材和临床密切随访。     

Clinicopathological and immunohistochemical study of 30 cases of post-radiation atypical vascular lesion of the breast

Atypical vascular lesions (AVL) that occur in the field of prior radiation therapy for breast carcinoma are placed within the differential diagnosis with low grade angiosarcoma and other benign vascular lesions. Although considered a benign entity, the exact biological behavior of AVLs is not fully established because of the small number of cases reported in the literature. We aim to further characterize these lesions clinically and histopathologically, and to study their behavior. We report a series of 30 patients with AVL of the breast occurring after radiation exposure, diagnosed and treated at the European Institute of Oncology, Italy. Immunohistochemical study was performed in all cases, using CD31, D2-40, CD105, and Ki-67 antibodies. Twenty-seven patients were treated with standard doses of conventional adjuvant radiation therapy for the prior breast carcinoma. Three patients were treated with intraoperative radiotherapy with electrons. The post-radiation latency interval from breast carcinoma to AVL was 48.5 months (ranged from 1 to 146 months). Most of the lesions were classified as lymphatic type (78.6 %) based on D2-40 positivity. No extension into subcutaneous tissue or significant atypia was noted in all cases. Despite the fact that the AVL of our series have shown benign behavior in 93.3 %, one patient developed local recurrence of AVL, and two cases progressed to angiosarcoma at the previous AVL site. Further studies should be conducted to better understand the clinical behavior and to propose additional histopathologic diagnostic criteria to distinguish AVL from low grade angiosarcoma and those AVL with increased risk for malignant progression. Concerning current treatments of AVL, we recommend complete excision with free surgical margins and close follow up.     

食管肿瘤5406例临床病理特点对比分析

目的:探讨不同组织学类型食管肿瘤的临床病理特点.方法:回顾性分析5 406例食管肿瘤术后的病理诊断结果,观察各种肿瘤的早诊率、淋巴结转移率及年龄分布情况,对比分析各种食管肿瘤的特点.结果:5 406例患者中,29例双原癌(同时合并贲门腺癌),5 377例食管单器官肿瘤.鳞癌占95.5%(5 164/5 406),腺鳞癌占1.8%(99/5 406),腺癌占0.5%(29/5 406),其他类型4%(213/5 406).全部病例中早诊率为9.1%(490/5 406),淋巴结转移率为39.6%(2 142/5 406).各型早诊率和淋巴结转移率之间差异无统计学意义,χ2值分别为7.384和12.677,P值分别为0.597和0.178.小细胞癌与鳞癌的淋巴结转移率之间差异有统计学意义,χ2=4.704,P=0.030.结论:食管胸中段是各型肿瘤的好发部位,鳞癌最多见,腺鳞癌次之,其次为小细胞癌、腺癌及癌肉瘤等.各型肿瘤早诊率及淋巴结转移率均相似,仅小细胞癌淋巴结转移率明显高于鳞癌.提高食管肿瘤的早诊率是改善预后的关键.     

残胃癌的临床病理特征及预后分析

目的探讨残胃癌的临床病理特征及预后相关因素。方法回顾性分析45例残胃癌患者的临床病理资料,并进行随访。结果45例残胃癌患者的男女比例为44：1。初次手术距残胃癌的诊断时间为5～42年,平均23年。残胃病变位于吻合口28例,位于贲门9例,其余部位8例。未分化癌1例,低分化腺癌36例,中分化腺癌7例,高分化腺癌1例。根治性切除患者的1、3、5年生存率分别为100．0％、78．8％和47．2％,非根治性切除患者的1、3、5年生存率分别为62．5％、25．0％和0,两组患者生存率差异有统计学意义（P〈0．05）。10例病变未切除患者均于2年内死亡,平均生存时间为12个月。各病理分期患者间生存率差异有统计学意义（P〈0．05）。结论残胃癌多于Billroth Ⅱ式胃大部切除术后10年以上发生,男性多于女性,病变主要位于吻合口附近。进展期残胃癌病理类型以低分化腺癌常见。残胃癌的预后与病理分期、能否行根治性切除密切相关。     

24例与癌并发的胃肠间质瘤临床病理分析

  目的  研究与消化道癌并发的胃肠间质瘤(gastrointestinal stromal tumor, GIST)的临床病理特点。  方法  对胜利石油管理局胜利医院、胜利油田中心医院、东营市人民医院2002年1月至2012年12月收治的157例胃肠间质瘤病例, 观察CD117、CD34、SMA免疫组织化学标记的表达。观察并发胃肠道癌病例的临床病理特点, 重点观察肿瘤异型性、核分裂活性、细胞增殖活性标记物Ki-67的表达特点, 与未并发胃肠道癌的病例进行比较。  结果  157例中并发胃肠道癌者24例, 占15.3%。其中男14例, 女10例, 男女之比为1.4:1。年龄41岁~66岁, 中位年龄55岁。24例中7例位于食管中段或下段, 15例位于胃壁, 2例位于空肠。肿瘤直径0.6~3.8 cm, 平均(1.50±0.85) cm, 4例有轻度异型性, 其余无异型性。核有丝分裂0~5个/50HPF, 平均(0.79±1.83)个/ 50 HPF, Ki-67指数0~7.72, 平均2.16±3.26。并发癌瘤包括食管癌5例, 胃食管交界处癌2例, 胃癌15例, 肠癌2例。作为对照, 未并发消化道癌的胃肠道间质瘤患者133例, 其中男74例, 女59例, 男女之比为1.25:1。年龄43~71岁, 中位年龄54岁。114例发生于胃, 13例位于肠, 6例食管。肿瘤直径2.4~15.5 cm, 平均(6.11±7.09) cm。82例显示不同程度的异型性, 68例诊断为中危险度, 14例为高危险度。核有丝分裂0~53个/50 HPF, 平均(3.81±23.67)个/50 HPF。Ki-67指数0~39.21, 平均6.22±16.96。并发癌的胃肠间质瘤较未并发癌者比较, 男女病例比值较高, 瘤体平均直径较小, 核分裂指数和Ki-67阳性指数显著较低(分别为t=1.981, P < 0.05;t=1.993 5, P < 0.05)。  结论  15.3%胃肠间质瘤是并发癌。并发的胃肠间质瘤多数没有特殊临床症状, 因癌手术后标本大体检查时发现。其增殖活性显著低于未并发癌的胃肠间质瘤, 可能属于发生早期的肿瘤      

110例乳腺间期癌的临床病理特征分析

目的 分析顺义区三轮乳腺癌筛查中110例间期癌患者的临床及病理特征。方法 将顺义区历年乳腺癌发病个案信息与北京市妇幼保健网络信息系统中适龄女性乳腺癌筛查数据进行关联,查询乳腺间期癌患者。将间期癌患者与同时期在我院乳腺中心就诊的乳腺癌患者进行特征比较。结果与门诊收治的原发性乳腺癌相比,间期癌患者年龄偏小、超声肿物较小,差异均有统计学意义（P＜0.05）。与病灶大小相近的门诊收治的乳腺癌患者相比,真正间期癌X线检查恶性钙化和浸润性导管癌的比例均较高,差异均有统计学意义（P＜0.05）。间期癌患者在乳腺癌筛查中转诊X线检查比例很低（0.91%）,而110例间期癌确诊时有103例接受了乳腺X线检查,其中53例（48.2%）存在恶性钙化表现。结论 乳腺癌筛查中,年龄偏小的女性更易发生间期癌。由于间期癌患者中接近50%在诊断时存在X线下恶性钙化表现,因而若在以超声检查为主的乳腺癌筛查过程中结合乳腺X线检查,或许可以避免部分间期癌的发生。