Marchiafava-Bignami病2例报告并文献复习

目的 :探讨 Marchiafava- Bignami病 (MBD)的病因、临床表现、影像特点及诊断。方法 :分析 2例 MBD并复习文献。结果 :2例男性酗酒者均有情感淡漠、步态障碍、认知损害等脑病表现。脑 MRI均发现大片对称的胼祗体 (CC)T1 WI低信号 ,T2 WI高信号病变 ,但脑 CT未发现明显 CC病变。均予维生素 B1 治疗 ,例 1好转 ,例 2死亡。结论 :MBD的临床表现缺乏特异性 ,CT对 MBD不敏感。酗酒和 /或营养不良史 ,脑病表现和 MRI发现大片对称的 CC病变支持 MBD的诊断。     

Spitz痣2例临床病理分析

目的分析Spitz痣的临床病理学特征及鉴别诊断。方法对2例Spitz痣进行组织学观察和免疫组化标记,同时复习相关文献。结果Spitz痣由梭形细胞和上皮样细胞构成,梭形细胞常成束状排列,并与表皮垂直,上皮样细胞排列成巢或散在分布。结论Spitz痣是一种具有独特临床病理特征的黑色素细胞良性肿瘤,应注意与恶性黑色素瘤相鉴别。     

膀胱副神经节瘤2例报告并文献复习

目的 探讨膀胱副神经节瘤的临床病理及免疫组化特征.方法 对2例膀胱副神经节瘤进行组织病理学观察及免疫组化分析并复习文献.结果 2例大体肿物均有完整包膜.镜下肿瘤细胞排列呈巢状及条索状,其中1例伴有鳞状细胞或腺样分化的器官样结构.细胞大小不等,细胞核圆形、椭圆形、梭形,核分裂罕见.瘤细胞巢间有丰富的大、小血窦.免疫组化瘤细胞CgA、Syn、S-100阳性,CK、CK7、HMB45、CEA阴性.结论 膀胱副神经节瘤是较罕见的肿瘤,诊断依赖于组织病理形态及免疫组化.     

Spitz痣误诊1例报告并文献复习

目的：提高对Spitz痣临床病理特征的认识。方法：对1例Spitz痣的误诊进行分析，并结合文献分析其临床病理特点。结果：Spitz痣是一种较少见的良性黑色素细胞瘤，常见于面部和四肢。结论：Spitz痣的诊断主要依据临床表现、组织学特点、免疫组化表达。     

皮肤Rosai-Dorfman病2例报告并文献复习

目的 探讨皮肤Rosai-Dorfman病(CRDD)的临床病理学特征、免疫学表型和鉴别诊断.方法 分析2例CRDD患者的临床表现,对标本进行病理学和免疫组织化学染色观察并文献复习.结果 CRDD镜下病理特征为真皮层较多的体积较大的组织细胞浸润,呈多边形或椭圆形,胞质丰富,淡嗜酸性,胞浆内吞噬有数量不等的淋巴细胞、浆细胞或其他炎细胞,免疫组化染色显示组织细胞表达S-100蛋白、CD68,但CD1a和CD34表达阴性.结论 CRDD病是一种少见的组织细胞增生性病变,在病理诊断方面,易误诊为其他疾病,应与其他组织细胞增生性疾病相鉴别.     

脂质沉积性肌病2例报告并文献复习

目的：探讨脂质沉积性肌病（LSM）的临床和肌肉病理特点。方法：分析2例脂质沉积性肌病患者的临床特点、实验室检查、肌活检资料,并复习相关文献。结果：LSM的主要临床特点为四肢近端肌无力和对运动不耐受,肌萎缩多不明显,血清肌酶轻中度升高,肌电图多呈肌源性损害;肌肉病理显示肌纤维空泡变性,脂滴明显增多,电镜也证实肌纤维内脂滴堆积。结论：LSM是一种少见的肌病类型,以肢体近端肌无力和对运动不耐受为主要表现,临床上多长期误诊,确诊依靠肌肉病理检查。     

骨外骨肉瘤1例报告并文献复习

目的探讨骨外骨肉瘤的临床病理特征、病理诊断和鉴别诊断要点。方法对1例罕见的小腿骨外骨肉瘤进行临床和病理组织学观察并复习相关文献。结果病理检查:(1)巨检:瘤体有假包膜,切面灰白,周边区质脆,中央区质地较硬,界限尚清。(2)镜检:肿瘤细胞多形性,核呈卵圆形或不规则形,染色质粗,无明显核仁,核分裂多见;并可见肿瘤细胞产生的不规则点滴状、网格状骨样基质。结论骨外骨肉瘤是一种罕见的软组织高度恶性肿瘤,极易误诊,其诊断主要依靠组织病理学特征,并结合临床表现和影像学检查。     

胆囊癌肉瘤1例报告并文献复习

目的：探讨胆囊癌肉瘤的临床病理特征、诊断及鉴别诊断、治疗、误诊的原因和预防。方法：分析1例胆囊癌肉瘤的临床表现,对标本进行组织病理学和免疫组织化学染色观察,并复习文献,了解该肿瘤的特征。结果：胆囊癌肉瘤罕见,女性多于男性,恶性度高,预后差。组织学检查显示有癌和肉瘤成分,免疫组织化学对鉴别诊断有帮助。手术及放、化疗均疗效不好。结论：原发于胆囊的癌肉瘤较罕见,预后与肿瘤分期有关。临床实践中要特别重视本病与胆囊的一些相似疾病鉴别。避免误诊。     

肾集合管癌2例报告并文献复习

目的：分析肾集合管癌的临床和病理特征，提高对肾集合管癌的认识。方法：回顾性分析2例肾集合管癌的临床、影像学和病理学特征并结合文献复习。结果：临床症状缺乏特异性，主要表现为腰腹痛和肉眼全程血尿。肿瘤主要位于肾髓质，缺乏包膜，侵袭性生长，转移早。CT平扫时部分肿瘤密度略低于肾实质，增强后肿物强化不明显，低于肾实质，有时难与早期结核病灶区分。病理特点镜下肿瘤呈弥漫性或腺管状、乳头状排列，肿瘤间质纤维组织反应性增生，多量浆细胞和淋巴细胞浸润。免疫组化：肿瘤细胞CK表达均为阳性，Virnetin为阴性或弱阳性。结论：肾集合癌是一种起源于肾髓质集合管的罕见性肾肿瘤，恶性度高、预后差。     

骶管非砂粒体型黑色素性神经鞘瘤1例报告并文献复习

目的 探讨非砂粒体型黑色素性神经鞘瘤(NPMS)的临床病理特点、诊断及鉴别诊断要点,提高病理医生对NPMS的认识及诊断水平.方法 应用HE染色对1例发生于骶管的NPMS进行组织形态学观察,并复习相关文献.结果 光镜下NPMS由长形及卵圆形上皮样瘤细胞组成,多数瘤细胞胞质内可见黑色素颗粒并将细胞核掩盖.肿()变,但无坏死,未见病理性核分裂象.结论 NPMS是一种罕见的周围神经肿瘤,具有特征性的组织病理学特点,易与其他含有色素的软组织肿瘤混淆.多数NPMS为良性,少数病例会恶变,术后应长期随访复查.     

股骨促结缔组织增生性纤维瘤2例报告并文献复习

目的探讨股骨促结缔组织增生性纤维瘤(DF)的临床病理特征及诊断与鉴别诊断。方法对2例股骨DF进行病理形态和免疫组化观察,结合临床资料进行分析并复习相关文献。结果组织学特点:肿瘤由梭形瘤细胞和大量胶原构成,胶原纤维密集而粗大,呈波浪状或束状,瘤细胞核形态温和,肿瘤多呈浸润性生长。免疫组化:瘤细胞-βcatenin(2/2)、Vimentin(2/2),Desmin、SMA局灶(+)。结论骨DF具有较强的局部侵袭能力,切除不彻底容易复发,应手术广泛全切。联合检测-βcatenin、SMA、Vim、Desmin等标记物对骨DF诊断和鉴别诊断具有重要意义。     

成人神经母细胞瘤4例报告并文献复习

目的 探讨成人神经母细胞瘤(adult neuroblastoma ANB)的临床病理特点、诊断及鉴别诊断.方法 回顾盛京医院诊治的4例ANB的临床、病理特征及免疫表型特点.结果 4例ANB患者发病年龄22～33岁,男女比例3∶1.临床主要表现为胸腹部包块、隐痛不适.形态学及免疫表型与小儿NB表现相同:瘤细胞圆形深染,大小较一致,可见坏死及钙化.瘤细胞表达NSE、NF、Syn.结论 ANB是一种极少见的恶性肿瘤,预后差,在临床和病理诊断时要考虑到此病的可能性.     

肺原发性恶性黑色素瘤伴多器官转移1例报告并文献复习

目的：探讨肺原发性恶性黑色素瘤的临床和CT特点。方法：报告1例肺原发性恶性黑色素瘤的详细临床及CT资料．并结合献,对该罕见病的临床和CT表现等做一复习。结果：CT显示肺原发瘤边缘分叶状改变’肺原发瘤和肝、脾转移瘤易发生坏死、囊变,纵隔淋巴结及肺转移瘤以多发结节状病灶为主。增强扫描肝、脾脏转移瘤多呈“牛眼征”。结论：CT有助于肺原发性恶性黑色素瘤的诊断,在一定程度上可以区分原发性和转移性黑色素瘤。     

The immunopathology of regression in benign lichenoid keratosis, keratoacanthoma and halo nevus

BACKGROUNDRegression is a phenomenon present in a variety of cutaneous lesions. It is likely that similar immunologic mechanisms explain the phenomenon of spontaneous regression occurring in the various lesions.METHODSTwenty-seven specimens, nine each of halo nevus, keratoacanthoma, and benign lichenoid keratosis, including three examples each of predominantly early, mid, and late regression were examined with antibodies to HLA-II, CD1a, CD3, CD4, CD8, CD20, CD34, CD56, and CD68.RESULTSEpidermotropism of inflammatory cells, including CD1a positive, CD68 positive, CD3 positive, and CD8 positive cells, was present in benign lichenoid keratosis and keratoacanthoma, but not in halo nevus. In halo nevus, the nests of halo nevus cells tended to be infiltrated by CD1a positive, CD68 positive, CD3 positive, and CD8 positive cells. The blood vessels exhibited endothelial cell swelling with luminal narrowing and disruption within the dermis of all lesions. The CD1a positive cells were increased in number in lesional epidermis except in keratoacanthoma lesions where the density of CD1a positive cells was increased in the epithelial lip, but decreased within the epithelial portion of the keratoacanthoma proper. Conversely, the CD8 positive cells were scarce in the dermis below the epithelial lip of the keratoacanthoma, but increased in the dermis of the neoplastic epithelium. CD1a positive cells were also seen throughout the dermal portion of the lesion, particularly at the lesion base. In halo nevus, the CD1a positive cells and CD68 positive cells within the lesions were larger than those in non-lesional skin, indicating activation. The composition of the inflammatory infiltrate varied within each lesion type according to stage of regression, but T-lymphocytes predominated.CONCLUSIONCytotoxic T-cells may be the final common denominator of regression in benign lichenoid keratosis, keratoacanthoma, and halo nevus. In halo nevus, cytotoxic T-cells may play the predominant role in regression. In keratoacanthoma and benign lichenoid keratosis, cytotoxic T-cells play a pivotal role, but additional mechanisms may also be involved in the phenomenon of regression. Benign lichenoid keratoses progress through stages of regression accompanied by varying proportions of inflammatory cells, including CD3, CD4, and CD8 positive T-lymphocytes, natural killer cells, macrophages and Langerhans cells.     

Lance Adams syndrome: two cases report and literature review

Hypoxic myoclonus, also known as Lance Adams syndrome, is a rare syndrome that results from the serious brain damage caused by cerebral hypoxia that often follows cardiopulmonary resuscitation. This current case report describes two patients with post-hypoxic myoclonus, both of whom received cardiopulmonary resuscitation. The neurological symptoms of these two patients were significantly improved by the administration of clonazepam and sodium valproate sustained-release tablets. The report presents a literature review detailing the pathogenesis, diagnosis and treatment of Lance Adams syndrome. The timely diagnosis and treatment of Lance Adams syndrome can significantly improve the quality of life of patients. Valproic acid, clonazepam and other antiepileptic drugs can be used. Whether levetiracetam is effective for cortical myoclonus requires further clinical study.     

鼻腔原发性透明细胞癌1例报告并文献复习

目的探讨鼻腔原发性透明细胞癌的临床病理特点和鉴别诊断要点。方法结合文献对1例鼻腔透明细胞癌的临床资料、病理特征、免疫表型及治疗预后进行分析。结果典型的组织学特征表现为多边形上皮样瘤细胞组成,排列成巢状,由富含薄壁毛细血管的纤维组织分隔。瘤细胞大小不一,胞质丰富透亮,呈空泡状,核小,核分裂罕见。免疫组化:肿瘤细胞显示EMA(+)、CKpan(+)、CD10(-)、CK7(-)、HMB45(-)、S-100(-)、Calponin(-)、SMA(-)、GFAP(-)、TG(-)。结论鼻腔原发透明细胞癌是罕见的肿瘤,其诊断和鉴别诊断主要依靠病理组织学、免疫组织化学。