Giant Cells

巨细胞
  • 文章类型: Case Reports
    结肠的慢性特发性溃疡由于其难以捉摸的病因和与其他肠道病理的潜在相似性而构成诊断挑战。如盲肠癌。此病例报告概述了一名68岁女性患者的临床过程,该患者因持续的右下腹疼痛而被送往急诊科(ED)。尽管多次医院就诊会产生不同的诊断,只有在腹腔镜部分结肠切除术后才做出明确的诊断,显示慢性特发性溃疡有透壁性瘢痕和与相邻小肠环的粘连。组织学检查显示大量的溃疡床充满了炎症细胞,包括肉芽组织内的大星状和纺锤状基质细胞,与淋巴增生和疤痕组织一起延伸到固有肌层。这个病例最初的表现很容易被误认为是阑尾炎,憩室炎,癌,或者肠易激综合征,强调在盲肠肿块患者的鉴别诊断中考虑慢性特发性溃疡的重要性。
    Chronic idiopathic ulcers of the colon pose diagnostic challenges due to their elusive etiology and potential resemblance to other intestinal pathologies, such as cecal carcinoma. This case report outlines the clinical course of a 68-year-old female patient who presented to the emergency department (ED) with persistent right lower quadrant pain. Despite multiple hospital visits yielding varied diagnoses, a definitive diagnosis was only made following a laparoscopic partial colectomy, which revealed chronic idiopathic ulcers with transmural scarring and adhesions to adjacent small intestine loops. Histological examination demonstrated a substantial ulcer bed populated by inflammatory cells, including large stellate and spindled stromal cells within the granulation tissue, alongside lymphoid hyperplasia and scar tissue extending into the muscularis propria. The initial presentation of this case could easily be mistaken for appendicitis, diverticulitis, carcinoma, or irritable bowel syndrome, highlighting the significance of considering chronic idiopathic ulcers in the differential diagnosis of patients presenting with cecal masses.
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  • 文章类型: Journal Article
    目的:巨细胞肉芽肿是一种局部非肿瘤性病变,分为两类,根据其发生部位:中枢和外周巨细胞肉芽肿。中央巨细胞肉芽肿是一种骨内病变,即使在手术治疗的病例中也有复发的趋势。多项研究证明,不同病变的临床行为与其组织学特征之间存在关联。这项研究的目的是评估有无复发的病变中AgNOR和Ki67的表达。
    方法:对35例经组织学诊断为中心性巨细胞肉芽肿的患者的档案和记录进行调查。在进行AgNOR染色和Ki67标记后研究组织学特征。数据采用卡方分析,费希尔,和T检验。
    结果:获得的数据表明,有复发的病变的AgNOR染色和Ki67标记计数明显高于无复发的病变。从Ki67强度获得相同的结果。
    结论:目前的研究表明,AgNOR染色和Ki67标记物对预测中央性巨细胞肉芽肿病变复发具有预后价值。
    Giant cell granuloma is a local nonneoplastic lesion that is divided into two categories, based on its site of occurrence: Central and peripheral giant cell granuloma. Central giant cell granuloma is an intraosseous lesion that has a tendency to recure even in surgically treated cases. Several studies have proven that there is an association between different lesions clinical behavior and their histological features. The aim of this study was to evaluate the expression of AgNOR and Ki67 in lesions with and without recurrency.
    Files and records of 35 patients who had been histologically diagnosed with central giant cell granuloma were investigated. Histological features were studied after performing AgNOR staining and Ki67 marker. The data were analyzed by chi-square, Fisher, and T-test.
    Acquired data indicated that the count of AgNOR staining and Ki67 marker was significantly higher in lesions with recurrency than the lesions with no recurrency. The same results were attained from Ki67 intensity.
    The current study indicated that AgNOR staining and Ki67 marker have prognostic value in predicting recurrency of central giant cell granuloma lesions.
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  • 文章类型: Case Reports
    背景:结节性硬化症(TSC)是一种罕见的,以各种器官系统错构瘤和肿瘤性病变为特征的复杂遗传性疾病。随着放射学和基因检测技术的发展,TSC的诊断标准于2012年在国际共识会议上更新.口腔内纤维瘤长期以来与TSC相关。然而,TSC患者中巨细胞性血管纤维瘤(GCA)的发生率极为罕见.这里,我们报告了第一例TSC患者牙龈组织中的GCA。
    方法:一名41岁女性首次访问口腔颌面外科,Chonnam国立大学牙科医院,抱怨牙龈肿大。临床检查显示与TSC相关的几种表现,包括口内纤维瘤,面部血管纤维瘤,牙釉质坑,指甲纤维瘤,“五彩纸屑”皮肤损伤,低黑素性黄斑,还有一块浅绿色的补丁.口内检查显示左下颌骨有6.0×5.0厘米的牙龈过度生长。进行了手术切除,随后的组织病理学检查证实了GCA的诊断。在手术的24个月内没有复发的证据。
    结论:我们报告了第一例TSC患者牙龈组织中的GCA。这份报告将有助于更好地了解这种罕见的疾病。然而,需要进一步的病例报告来阐明GCA和TSC之间的关系.
    BACKGROUND: Tuberous sclerosis complex (TSC) is a rare, complex genetic disorder characterized by hamartomas and neoplastic lesions in various organ systems. With the development of radiology and gene testing, the diagnostic criteria for TSC were updated in 2012 at the International Consensus Conference. Intraoral fibromas have long been associated with TSC. However, the incidence of giant cell angiofibroma (GCA) in TSC patients is extremely rare. Here, we report the first case of GCA in the gingival tissue of a patient with TSC.
    METHODS: A 41-year-old woman first visited the Department of Oral and Maxillofacial Surgery, Chonnam National University Dental Hospital, complaining of gingival enlargement. Clinical examination revealed several manifestations associated with TSC, including intraoral fibromas, facial angiofibromas, dental enamel pits, ungual fibromas, \"confetti\" skin lesions, hypomelanotic macules, and a shagreen patch. Intraoral examination revealed a 6.0 × 5.0 cm gingival overgrowth on the left mandible. Surgical excision was performed, and subsequent histopathological examination confirmed the diagnosis of GCA. There was no evidence of recurrence within the 24- months of surgery.
    CONCLUSIONS: We report the first case of GCA in the gingival tissue of a patient with TSC. This report would contribute to an improved understanding of this rare disease. However, further case reports are necessary to clarify the relationship between GCA and TSC.
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  • 文章类型: Case Reports
    背景技术特发性巨细胞心肌炎(IGCM)是一种罕见且经常致命的心肌炎类型。它主要影响年轻人,并有可能导致心力衰竭和危及生命的心律失常。IGCM似乎依赖于CD4阳性T淋巴细胞的活化,并且可以通过旨在降低T细胞功能的治疗显示出改善。我们介绍了一例65岁的患者,该患者具有指南指导的药物治疗(GDMT)难以治疗的急性心力衰竭的特征,由于IGCM。还介绍了IGCM的自然史和治疗方法。病例报告一名患有多种合并症的65岁女性因进行性非缺血性心力衰竭的室性心动过速入院,对GDMT无反应。这导致了进一步的调查,包括心内膜活检,显示炎症浸润,在没有肉芽肿形成的情况下,多核巨细胞和淋巴细胞,提示IGCM的诊断。植入心脏复律除颤器(ICD)用于心脏猝死的二级预防,并开始对患者进行联合免疫抑制治疗。由于许多合并症,她被认定不适合做心脏移植.不幸的是,她最终死于继发于这种疾病的并发症。结论IGCM仍然是一个具有挑战性的临床诊断,如果没有心脏移植,长期结果较差。该病例强调了在对常规疗法无反应的患者中考虑心力衰竭的非典型原因的重要性。及早认识和适当的管理,涉及医学和介入方法,对改善IGCM患者的预后至关重要。
    BACKGROUND Idiopathic giant cell myocarditis (IGCM) is an uncommon and frequently fatal type of myocarditis. It primarily affects young individuals and has the potential to result in heart failure and life-threatening arrhythmias. IGCM seems to be dependent on activation of CD4-positive T lymphocytes and can show improvement with treatment aimed at reducing T-cell function. We present a case of a 65-year-old patient who presented with features of acute heart failure refractory to guideline-directed medical therapy (GDMT), due to IGCM. A review of the natural history and treatment of IGCM is also presented. CASE REPORT A 65-year-old woman with multiple comorbidities was admitted to our hospital for ventricular tachycardia in the setting of progressive non-ischemic heart failure, unresponsive to GDMT. This led to further investigation, including an endomyocardial biopsy, which revealed inflammatory infiltration, with multinucleated giant cells and lymphocytes in the absence of granuloma formation, prompting a diagnosis of IGCM. An implantable cardioverter-defibrillator (ICD) was placed for secondary prevention of sudden cardiac death and the patient was initiated on combined immunosuppressive therapy. Owing to numerous comorbidities, she was determined to be unsuitable for a heart transplant. Unfortunately, she eventually died from complications secondary to the disease. CONCLUSIONS IGCM remains a challenging clinical diagnosis with a poor long-term outcome without heart transplantation. This case highlights the importance of considering atypical causes of heart failure in patients who do not respond to conventional therapies. Early recognition and appropriate management, involving medical and interventional approaches, are crucial in improving outcomes for patients with IGCM.
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  • 文章类型: Review
    星形胶原瘤,也被称为硬化性纤维瘤,是一种相对罕见的良性皮肤肿瘤,由成纤维细胞的增殖组成,显示出I型胶原蛋白的产生增加。它可能看起来像一个孤独的,散发性病变,或者,特别是当多个,与Cowden综合征有关.巨细胞胶原瘤的组织病理学外观类似于瘤状胶原瘤,并添加了小花型巨细胞。在这里,我们报道了在患有Cowden综合征的个体中出现的多发性巨细胞胶原瘤的发现。在对已发表文献的回顾中,这种组织病理学变异似乎很少与Cowden综合征相关.
    Storiform collagenoma, also known as sclerotic fibroma, is a relatively rare benign cutaneous tumor consisting of a proliferation of fibroblasts that shows increased production of type I collagen. It may appear as a solitary, sporadic lesion, or, especially when multiple, associated with Cowden syndrome. Giant cell collagenoma has a histopathologic appearance similar to that of storiform collagenoma with the addition of floret-type giant cells. Herein, we report the finding of multiple giant cell collagenomas arising in an individual with Cowden syndrome. In a review of the published literature, this histopathologic variant appears to be rarely observed in association with Cowden syndrome.
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  • 文章类型: Case Reports
    肝外胆管梭形和巨细胞型未分化癌是一种罕见的恶性肿瘤。我们报告了一例涉及胆总管的病例,该病例是我们医院收治的一名72岁男性黄疸患者。诊断成像,包括腹部计算机断层扫描和磁共振成像,发现远端胆总管有肿块,伴有肝内和肝外胆管扩张和局部淋巴结肿大。内镜逆行胆管造影显示胆总管远端狭窄,活检证实腺癌.该患者接受了内窥镜逆行胆道引流,然后进行了保留胃的胰十二指肠切除术和区域淋巴结切除术。显微镜检查显示,肿瘤主要由基质内的梭形和巨大的非典型细胞组成。免疫组织化学分析显示肿瘤细胞表达细胞角蛋白和间充质标志物,证实梭形和巨细胞型未分化胆总管癌的诊断。观察到Ki-67标记指数高于80%。术后,两个月时发现腹腔内淋巴结复发,在三个月时发现了多个肝转移。患者在手术后7个月死亡。回顾并讨论了与这种罕见疾病有关的文献。
    Spindle and giant cell type undifferentiated carcinoma of the extrahepatic bile duct is an uncommon malignancy. We report a case involving the common bile duct in a 72-year-old male with jaundice who was admitted to our hospital. Diagnostic imaging, including abdominal computed tomography and magnetic resonance imaging, revealed a mass in the distal common bile duct, accompanied by dilatation of both intra- and extrahepatic bile ducts and regional lymph node enlargement. Endoscopic retrograde cholangiography demonstrated stenosis in the distal common bile duct, with a biopsy confirming adenocarcinoma. The patient underwent endoscopic retrograde biliary drainage followed by a subtotal stomach-preserving pancreaticoduodenectomy with regional lymphadenectomy. Microscopic examination revealed that the tumor predominantly comprised spindle and giant atypical cells within the stroma. Immunohistochemical analysis showed the tumor cells expressing cytokeratins and mesenchymal markers, confirming the diagnosis of spindle and giant cell type undifferentiated carcinoma of the common bile duct. Ki-67 labeling index was observed to be above 80%. Postoperatively, intra-abdominal lymph node recurrence was noted at two months, and multiple liver metastases were identified at three months. The patient died seven months post-surgery. The literature pertaining to this rare disease is reviewed and discussed.
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  • 文章类型: Review
    背景:富含巨细胞的孤立性纤维瘤(GCR-SFT),以前被称为巨细胞血管纤维瘤,是一种罕见的软组织肿瘤,通常发生在眼眶,但很少出现在深部器官中。这里,我们介绍了一例发生在膀胱的GCR-SFT,这是一种不寻常的SFT组织学亚型。
    方法:一名56岁男子在体格检查中偶然发现膀胱左后壁有一个大小为4.5×4.3×4.0cm的肿块。经腹腔镜根治术后病理证实为GCR-SFT。组织病理学,肿瘤是一个界限清楚的,由温和的梭形卵圆形肿瘤细胞与低细胞和高细胞区域交替组成的非包囊性病变,鹿角状血管和散布的大型深色多核巨细胞内衬假血管间隙。梭形卵圆形细胞和多核巨细胞显示CD34和核STAT6的强烈和弥漫性表达。此外,通过RT-PCR检测NAB2ex4-STAT6ex5融合基因的标志。根据风险分层标准将患者分类为具有低复发或转移风险。患者在手术后34个月接受定期随访,没有局部复发或转移的证据。
    结论:这是首例报道的膀胱中发生GCR-SFT并伴有NAB2ex4-STAT6ex5融合的病例。建议对肿瘤进行完整的手术切除和长期随访,以确保没有局部复发或转移。
    BACKGROUND: Giant cell-rich solitary fibrous tumour (GCR-SFT), previously referred to as giant cell angiofibroma, is an uncommon soft tissue tumour that classically occurs in the orbit but very rarely presents in deep organs. Here, we present a case of GCR-SFT occurring in the urinary bladder, which is one of the unusual histological subtypes of SFT.
    METHODS: A 56-year-old man was incidentally found to have a mass measuring 4.5 × 4.3 × 4.0 cm located in the left posterior wall of the bladder by computed tomography during a physical examination. The lesion was confirmed as GCR-SFT by pathological examination after laparoscopic radical surgery. Histopathologically, the tumour was a well-circumscribed, nonencapsulated lesion that was composed of bland spindle-ovoid tumour cells alternating with hypocellular and hypercellular areas, staghorn-like vasculatures and scattered large dark-stained multinucleate giant cells lining pseudovascular spaces. The spindle-ovoid cells and multinucleate giant cells showed strong and diffuse expression of CD34 and nuclear STAT6. In addition, the hallmark of the NAB2ex4-STAT6ex5 fusion gene was detected by RT‒PCR. The patient was classified as having a low risk of recurrence or metastasis according to the risk stratification criteria. The patient underwent regular follow-up for 34 months after surgery, and there was no evidence of local recurrence or metastasis.
    CONCLUSIONS: This is the first reported case of GCR-SFT occurring in the urinary bladder with underlying NAB2ex4-STAT6ex5 fusion. Complete surgical excision of the tumour and long-term follow-up are recommended to ensure no local recurrence or metastasis.
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  • 文章类型: Journal Article
    背景:培养的小鼠滋养层干细胞(mTSC)在FGF4下保持增殖/正常干性(NS),导致正常分化(ND)。缺氧,或高渗压力迫使滋养细胞巨细胞(TGC)分化。缺氧,高渗,和基因毒性苯并(a)芘(BaP),在烟草烟雾中发现,强制下调分化抑制剂(Id)2,使TGC分化。低氧和高渗胁迫通过SAPK依赖性HAND1增加诱导TGC。在这里,我们测试BaP是否在诱导SAPK和HAND1的同时强制mTSC到TGC。
    方法:用免疫印迹法检测Hand1和SAPK活性,在接触BaP72小时后,在Tfinal测定mTSC到TGC的生长和分化,NS,ND,维甲酸(RA),或山梨糖醇。通过实时成像仪自动对细胞核染色的细胞进行显微成像,并由ImageJ/FIJI分析,BiotekGen5,AIVIA专有人工智能(AI)软件或开源,CellPose人工智能/AI软件。
    结果:BaP(0.05-1μM)激活了SAPK和HAND1,而不减少生长。对于2-4N循环核和>4N分化的TGC核,TSC到TGC的分化越来越准确,使用ImageJ/FIJI,第五代,AIVIA,或CellPoseAI软件。AIVIA和CellposeAI软件与人类的准确性相匹配。BaP对SAPK激活/HAND1增加的最低影响比mESC的类似影响敏感>10倍。RA诱导44-47%的第一谱系TGC分化,但相同的RA剂量仅诱导1%的第一谱系mESC分化。
    结论:首先,这些试验数据表明mTSC可用于高通量筛选(HTS),以预测迫使TGC分化的毒物暴露.第二,对于类似的应激暴露,mTSC比mESC分化更多的细胞,第三,开源AI可以取代人类显微照片量化,并实现预测流产的HTS。
    Cultured mouse trophoblast stem cells (mTSC) maintain proliferation/normal stemness (NS) under FGF4, which when removed, causes normal differentiation (ND). Hypoxic, or hyperosmotic stress forces trophoblast giant cells (TGC) differentiate. Hypoxic, hyperosmotic, and genotoxic benzo(a)pyrene (BaP), which is found in tobacco smoke, force down-regulation of inhibitor of differentiation (Id)2, enabling TGC differentiation. Hypoxic and hyperosmotic stress induce TGC by SAPK-dependent HAND1 increase. Here we test whether BaP forces mTSC-to-TGC while inducing SAPK and HAND1.
    Hand1 and SAPK activity were assayed by immunoblot, mTSC-to-TGC growth and differentiation were assayed at Tfinal after 72hr exposure of BaP, NS, ND, Retinoic acid (RA), or sorbitol. Nuclear-stained cells were micrographed automatically by a live imager, and assayed by ImageJ/FIJI, Biotek Gen 5, AIVIA proprietary artificial intelligence (AI) software or open source, CellPose artificial intelligence/AI software.
    BaP (0.05-1μM) activated SAPK and HAND1 without diminishing growth. TSC-to-TGC differentiation was assayed with increasingly accuracy for 2-4 N cycling nuclei and >4 N differentiating TGC nuclei, using ImageJ/FIJI, Gen 5, AIVIA, or CellPose AI software. The AIVIA and Cellpose AI software matches human accuracy. The lowest BaP effects on SAPK activation/HAND1 increase are >10-fold more sensitive than similar effects for mESC. RA induces 44-47% 1st lineage TGC differentiation, but the same RA dose induces only 1% 1st lineage mESC differentiation.
    First, these pilot data suggest that mTSC can be used in high throughput screens (HTS) to predict toxicant exposures that force TGC differentiation. Second, mTSC differentiated more cells than mESC for similar stress exposures, Third, open source AI can replace human micrograph quantitation and enable a miscarriage-predicting HTS.
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  • 文章类型: Case Reports
    巨细胞心肌炎(GCM)是一种罕见但致命的疾病,可导致心力衰竭。心脏静止的生存需要机械循环支持或双心室辅助装置(BiVAD),并且延长生存极为罕见。药物超敏反应综合征(DIHS)是一种严重的皮肤不良反应。据报道,一些DIHS病例与GCM的发作有关。我们介绍了一例28岁女性,该女性在DIHS后类固醇逐渐减少期间发展了GCM。她处于持续的心脏停滞状态,但在BiVAD的支持下存活了74天。我们的病例值得注意,因为随着时间的推移,三次获得的组织病理学标本有助于诊断这种特殊情况。我们还回顾了先前关于GCM和DIHS合并病例的文献。我们发现两个可能相关,并且大多数GCM病例发生在类固醇逐渐减少期间DIHS的3个月内。
    Giant cell myocarditis (GCM) is a rare but fatal disease that can lead to cardiac failure. Survival with a cardiac standstill requires mechanical circulatory support or a biventricular assist device (BiVAD) and prolonged survival is extremely rare. Drug-induced hypersensitivity syndrome (DIHS) is a severe cutaneous adverse reaction. Some cases of DIHS are reportedly associated with the onset of GCM. We present a case of a 28-year-old woman who developed GCM during steroid tapering after DIHS. She went into continuous cardiac standstill but survived for 74 days under BiVAD support. Our case is noteworthy because the histopathologic specimens obtained on three occasions contributed to the diagnosis of this particular condition over time. We also reviewed previous literature on concomitant cases of GCM and DIHS. We found that two are potentially associated and most cases of GCM occur within 3 months of DIHS during steroid tapering.
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  • 文章类型: Case Reports
    巨细胞性心肌炎(GCM)是一类罕见的、预后最差的心肌炎亚型,病程凶险,死亡及心脏移植率高,早期识别及诊断GCM尤为重要。该病的确诊依赖于有创的心内膜心肌活检,但往往不作为临床实践的首要常规检查。该文报道了1例经无创、无辐射的心脏磁共振拟诊为GCM且经心内膜心肌活检证实的病例,旨在进一步认识心脏磁共振在体诊断GCM的价值。.
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