恶性外周神经鞘瘤52例临床病理分析

目的观察恶性外周神经鞘瘤的临床病理学特征、诊断及鉴别诊断。方法回顾性分析52例恶性外周神经鞘瘤的临床病理学及免疫表型特征并复习相关文献。结果 52例患者中,男女发病率为1∶1,年龄4~71岁,头颈部18例(35%),四肢12例(23%),躯干9例(17%),深部组织8例(15%),椎管内4例(8%),生殖道1例(2%)。镜下肿瘤组织呈束状或漩涡状排列,瘤细胞短纺锤形、卵圆形、梭形,核分裂象易见。免疫表型:瘤细胞局灶表达S-100蛋白,Ki-67增殖指数10%~70%。结论恶性外周神经鞘瘤罕见,侵袭性高,预后差,其组织形态复杂多样,需与滑膜肉瘤、纤维肉瘤、血管外皮瘤、富于细胞性神经鞘瘤、纤维型脑膜瘤以及平滑肌肉瘤等鉴别。     

丛状神经鞘瘤2例临床病理分析

目的探讨丛状神经鞘瘤的临床病理学特征、诊断及鉴别诊断。方法观察2例丛状神经鞘瘤的临床表现、组织学形态及免疫表型并复习相关文献。结果 2例患者中,男女各1例,年龄分别为16岁和31岁,肿瘤均位于躯干皮肤。镜下见肿瘤在皮下呈多结节状分布,结节内瘤组织以细胞致密区(Antoni A)为主,相对缺乏细胞疏松区(Antoni B)。瘤细胞长梭形或波浪状,呈栅栏状、漩涡状排列,可见verocay小体。免疫表型:肿瘤细胞S-100及vimentin均弥漫阳性,GFAP部分阳性,EMA、CD57及Ki-67均阴性。结论丛状神经鞘瘤是一种较少见的良性周围神经鞘膜瘤,需与丛状神经纤维瘤、丛状纤维组织细胞瘤、丛状恶性外周神经鞘膜瘤等相鉴别。     

微囊性/网状神经鞘瘤的临床病理特点

目的 探讨微囊性/网状神经鞘瘤的形态学和免疫表型特点及鉴别诊断.方法 收集1例发生在颈椎的微囊性/网状神经鞘瘤,根据HE切片和免疫组织化学染色观察形态学及免疫表型特点,并复习相关文献.免疫组织化学采用EnVision两步法.结果 患者男,35岁,因颈部不适就诊,影像学检查见颈5椎体破坏,肿块周围有硬化带,考虑良性病变.手术中见椎体破坏,肿瘤无包膜,结节状,质软,大小约3.5 cm×3.0 cm×1.8 cm.镜下观察,肿瘤无明确包膜,结节状分布,细胞学明确呈两种形态,一种类似于普通神经鞘瘤,但局灶细胞显示明显多形性,似神经鞘瘤伴退变,另一区域呈上皮样细胞,呈网状或蕾丝花边样排列,间质伴显著黏液变性.免疫组织化学示两种区域细胞表达一致,强阳性表达波形蛋白、S-100蛋白、胶质纤维酸性蛋白和神经元特异性烯纯化酶,散在表达CD68、CD10和Ki-67,不表达细胞角蛋白、上皮细胞膜抗原、神经微丝、癌胚抗原、平滑肌肌动蛋白、雌孕激素受体和p53.结论 微囊性/网状神经鞘瘤是神经鞘瘤的一种特殊形态学变型,临床罕见,发生在骨内更为罕见,熟悉其组织学特点和免疫表型有助于与脊索瘤和其他黏液性肿瘤或肉瘤鉴别.     

类似胃肠间质瘤的胃梭形细胞间叶源性肿瘤31例临床病理分析

目的比较发生于胃的类似胃肠间质瘤(gastrointestinal stromal tumor, GIST)的梭形细胞间叶源性肿瘤,探讨其临床病理特征的关系。方法采用HE和免疫组化EnVision两步法检测31例与GIST类似的胃梭形细胞肿瘤,分析各类肿瘤的组织病理学特点、免疫表型及分子病理学特征。结果 31例类似GIST的胃梭形细胞间叶源性肿瘤,分别为平滑肌瘤14例,炎性纤维性息肉9例,神经鞘瘤5例,钙化性纤维性肿瘤1例,炎性肌纤维母细胞瘤1例,恶性周围神经鞘膜瘤1例。31例与GIST类似的梭形细胞肿瘤免疫组化均表达vimentin,不表达CD117和Dog-1;14例平滑肌瘤弥漫表达α-SMA和desmin;9例炎性纤维性息肉弥漫表达CD34;5例神经鞘瘤弥漫表达S-100,局部表达EMA;1例炎性肌纤维母细胞瘤表达α-SMA,局部表达CD34和actin;1例恶性周围神经鞘膜瘤弥漫表达NSE和SOX10,不表达S-100、CD99、CD34、EMA等。31例与GIST类似的梭形细胞肿瘤除1例恶性周围神经鞘膜瘤Ki-67增殖指数约20%,其余Ki-67增殖指数均10%。结论诊断类似GIST的胃梭形细胞间叶源性肿瘤,应综合考虑肿瘤的临床病理特点、免疫表型以及分子病理学特征,避免误诊。     

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。发生部位分别为椎管内、肢体及腹腔,其中2例为多发病变。术前病程14天～30年,大多表现为缓慢生长的肿块,可伴感觉障碍,最大径1～20.5 cm,平均5.8 cm。镜下大部分肿瘤边界清楚,厚薄不等的纤维性包膜中可见淋巴细胞浸润,丰富的梭形细胞呈束状、编织状或漩涡状排列,与经典型神经鞘瘤Atoni A区类似,细胞有轻度异型性,可见核分裂象(0～8个/10 HPF),间质内血管壁玻璃样变性。免疫表型:30例S-100、SOX10及H3K27me3均弥漫阳性,12例GFAP阳性(12/30,40%),2例CD34小灶阳性(2/30,6.7%),Ki-67增殖指数1%～20%。28例患者获得随访,随访时间5～62个月,4例局部复发。结论 CS是一种罕见的良性神经源性肿瘤,免疫组化有助于与恶性外周神经鞘瘤及滑膜肉瘤鉴别。多数患者预后良好,复发与病变部位和手术切缘是否阳性直接相关。     

原发性肺内神经鞘瘤2例临床病理特征分析

目的 探讨原发性肺内神经鞘瘤的临床病理学特征。方法 回顾性分析2例原发性肺内神经鞘瘤的临床表现、组织学形态及免疫表型,并复习相关文献。结果 2例均为中老年患者,男、女性各1例,男性患者因体检发现肺部阴影就诊;女性患者因发现肺张力性病变就诊,病变分别位于左肺上叶和左肺下叶,最大径分别为2.2 cm和1.8 cm。镜下见瘤细胞与周围肺组织分界清楚,其内瘤细胞由稀疏区和密集区交替排列,少部分区域可见栅栏状结构,大部分区域瘤细胞呈洋葱皮样或漩涡状结构排列,可见Verocay小体结构。免疫表型：瘤细胞S-100、SOX10、vimentin、CD56、BCL-2和TTF-1均阳性,desmin、SMA、p63、Napsin A、CK5/6、GFAP、CKpan、EMA和STAT6均阴性,Ki-67增殖指数为3%～5%。术后分别随访5个月和13个月,均未见肿瘤复发或转移。结论 原发性肺内神经鞘瘤是一种良性的神经源性肿瘤,诊断需依靠病理检查及免疫表型,治疗以手术切除为主,预后良好。     

神经内神经束膜瘤16例临床病理学分析

目的 探讨神经内神经束膜瘤(intraneural perineurioma, InP)的临床病理学特征。方法 回顾性分析16例InP的临床病理学及免疫表型特征，并复习相关文献。结果 16例InP患者中男性5例，女性11例，年龄5～56岁，平均年龄27.4岁，14例为单发病变(分别位于尺神经、正中神经、桡神经、腓总神经),2例为多发病变(分别位于双侧臂丛神经及C1-3神经根)。患者术前病程2～360个月，常表现为病变神经对应区域缓慢进展的运动功能障碍，部分有感觉功能异常；影像学提示局部神经增粗呈纺锤形或长梭形，边界尚清，肌电图提示周围神经损伤；光镜下见梭形的神经束膜细胞以轴突-施万细胞复合体为中心形成假洋葱球样结构。电镜下见长而细的神经束膜细胞胞质内富含吞噬囊泡，与其伴随的施万细胞一起围绕轴突呈同心圆状排列。免疫表型：神经束膜细胞EMA、Glut-1、Claudin-1阳性，中心的轴突-施万细胞复合体NF、S-100及SOX10阳性。Ki-67增殖指数<5%。结论 InP是一种罕见的起源于神经束膜的良性肿瘤，易与肥大性周围神经病、腓骨肌萎缩症、混杂性神经鞘膜肿瘤、神经鞘瘤、神经纤...     

富于细胞性神经鞘瘤2例临床病理分析

目的 探讨富于细胞性神经鞘瘤临床病理学特点及其诊断与鉴别诊断.方法 对2例富于细胞性神经鞘瘤进行临床资料分析、组织学形态和免疫表型观察.结果 富于细胞性神经鞘瘤好发于腹膜后,临床上表现为缓慢生长的无痛性肿块.大体肿瘤呈圆形、椭圆形,包膜完整,切面实性,灰白、灰黄色.镜下肿瘤具有完整的包膜,在包膜外或包膜下可见淋巴细胞聚集灶,形成袖套样结构.免疫组化肿瘤细胞S-100、GFAP和CD57均弥漫强阳性,不表达CK(AE1/AE3)、desmin、SMA、CD34、CD117和DOG1.结论 富于细胞性神经鞘瘤是一种少见的假肉瘤性病变,大体表现、组织学特点是其诊断及鉴别诊断的主要依据,免疫组化标记有助于诊断和鉴别诊断.     

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…     

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.     

Pain and Effusion and Quadriceps Activation and Strength

Context:

Quadriceps dysfunction is a common consequence of knee joint injury and disease, yet its causes remain elusive.

Objective:

To determine the effects of pain on quadriceps strength and activation and to learn if simultaneous pain and knee joint effusion affect the magnitude of quadriceps dysfunction.

Design:

Crossover study.

Setting:

University research laboratory.

Patients or Other Participants:

Fourteen (8 men, 6 women; age = 23.6 ± 4.8 years, height = 170.3 ± 9.16 cm, mass = 72.9 ± 11.84 kg) healthy volunteers.

Intervention(s):

All participants were tested under 4 randomized conditions: normal knee, effused knee, painful knee, and effused and painful knee.

Main Outcome Measure(s):

Quadriceps strength (Nm/kg) and activation (central activation ratio) were assessed after each condition was induced.

Results:

Quadriceps strength and activation were highest under the normal knee condition and differed from the 3 experimental knee conditions (P < .05). No differences were noted among the 3 experimental knee conditions for either variable (P > .05).

Conclusions:

Both pain and effusion led to quadriceps dysfunction, but the interaction of the 2 stimuli did not increase the magnitude of the strength or activation deficits. Therefore, pain and effusion can be considered equally potent in eliciting quadriceps inhibition. Given that pain and effusion accompany numerous knee conditions, the prevalence of quadriceps dysfunction is likely high.Key Words: arthrogenic muscle inhibition, central activation failure, voluntary activation, muscles

Key Points

• Knee pain and effusion resulted in arthrogenic muscle inhibition and weakness of the quadriceps.
• The simultaneous presence of pain and effusion did not increase the magnitude of quadriceps dysfunction.
• To reduce arthrogenic muscle inhibition and improve muscle strength, clinicians should employ interventions that target removing both pain and effusion.

Quadriceps weakness is a common consequence of traumatic knee joint injury^1,2 and chronic degenerative knee joint conditions.^3,4 Arthrogenic muscle inhibition (AMI), a neurologic decline in muscle activation, results in quadriceps weakness and hinders rehabilitation by preventing gains in strength.⁵ The inability to reverse AMI and restore muscle function can lead to decreased physical abilities,⁶ biomechanical deficits,⁷ and possibly reinjury.⁵ Furthermore, researchers^8,9 have suggested that quadriceps weakness resulting from AMI may place patients at risk for developing osteoarthritis in the knee. In light of the substantial influence of quadriceps AMI on these clinically relevant outcomes, we need to improve our understanding of the factors that contribute to this neurologic decline in muscle activity so efforts to target and reverse it can be implemented and gains in strength can be achieved more easily.Joint injury and disease are accompanied by numerous sequelae (ie, pain, swelling, tissue damage, inflammation), so ascertaining which one ultimately leads to neurologic muscle dysfunction is difficult. Whereas a joint effusion can result in AMI,^10–12 the effects of pain are less understood despite many clinicians attributing AMI to pain. Using techniques that introduce knee pain without accompanying injury may provide insights into the role of pain in eliciting AMI.The degree of knee joint damage may play a role in the quantity of AMI that manifests. Hurley et al^13,14 demonstrated that quadriceps AMI, measured using an interpolated-twitch technique, was greater in patients with extensive traumatic knee injury (eg, fractured tibial plateau, ruptured medial collateral ligament, and medial meniscectomy) than patients with isolated joint trauma (ie, isolated anterior cruciate ligament [ACL] rupture). Similarly, patients with more knee joint symptoms (ie, greater number of symptoms and increased severity of symptoms) may present with greater magnitudes of quadriceps inhibition. Recently, investigators¹⁵ have suggested that patients with more pain display less quadriceps strength, supporting this tenet. Given that effusion and pain often present simultaneously with joint injuries and diseases, such as ACL injury and osteoarthritis, examining both the isolated and cumulative effects of these sequelae appears warranted to determine if they influence the magnitude of muscle inhibition.Experimental joint-effusion and pain models are safe and effective experimental methods that allow for the isolated examination of their effects on muscle function. The effusion model, whereby sterile saline is injected directly into the knee joint capsule,⁷ produces a clinically relevant magnitude of the joint effusion that may be present with traumatic injury. Effusion is thought to activate group II afferents responding to stretch or pressure,^16–18 which in turn may facilitate group Ib interneurons and result in quadriceps AMI.⁵ The pain model involves injecting hypertonic saline into the infrapatellar fat pad to produce anteromedial knee pain similar to that described in patients with patellofemoral pain syndrome.¹⁹ Pain is considered to initiate AMI through activation of group III and IV afferents that act as nocioceptors to signal damage or potential damage to joint structures.^16–18 The firing of these afferents then may lead to facilitation of group Ib interneurons, the flexion reflex, or the gamma loop, ultimately resulting in quadriceps inhibition.²⁰ Thus, these models allow us to create symptoms that are associated with knee injury and have the added benefit of providing a way to examine their effects in isolation.Therefore, the purpose of our study was to determine the effects of pain on quadriceps strength and activation and to learn if simultaneous pain and knee joint effusion would affect the magnitude of quadriceps dysfunction. We hypothesized that pain alone would result in quadriceps inhibition and that the magnitude of inhibition would be greater when effusion and pain were present simultaneously.     

即早基因c-fos与脑血管病及学习记忆

即早基因c-fos是广泛存在于原核细胞和真核细胞的高度保守基因.在正常情况下,c-fos基因参与细胞生长、分化、信息传递、学习和记忆等生理过程,而在病理情况下c-fos基因表达及调控变化与多种疾病的发生和发展有关.C-fos在中枢神经系统的某些部位可有基础水平的表达,但表达很低,当受到如脑缺血、脑出血、痫性发作、应激等刺激后,其在数十分钟内做出反应,在对外界刺激-转录耦联的信忠传递过程中起着核内第三信使的重要作用.     

Opportunities and challenges in the prevention and control of cancer and other chronic diseases: children's diet and nutrition and weight and physical activity

OBJECTIVE: The purpose of this article is to review the role of behavioral research in disease prevention and control, with a particular emphasis on lifestyle- and behavior-related cancer and chronic disease risk factors--specifically, relationships among diet and nutrition and weight and physical activity with adult cancer, and tracking developmental origins of these health-promoting and health-compromising behaviors from childhood into adulthood. METHOD: After reviewing the background of the field of cancer prevention and control and establishing plausibility for the role of child health behavior in adult cancer risk, studies selected from the pediatric published literature are reviewed. Articles were retrieved, selected, and summarized to illustrate that results from separate but related fields of study are combinable to yield insights into the prevention and control of cancer and other chronic diseases in adulthood through the conduct of nonintervention and intervention research with children in clinical, public health, and other contexts. RESULTS: As illustrated by the evidence presented in this review, there are numerous reasons (biological, psychological, and social), opportunities (school and community, health care, and family settings), and approaches (nonintervention and intervention) to understand and impact behavior change in children's diet and nutrition and weight and physical activity. CONCLUSIONS: Further development and evaluation of behavioral science intervention protocols conducted with children are necessary to understand the efficacy of these approaches and their public health impact on proximal and distal cancer, cancer-related, and chronic disease outcomes before diffusion. It is clear that more attention should be paid to early life and early developmental phases in cancer prevention.