嗅神经母细胞瘤9例临床病理及免疫组化分析

目的探讨嗅神经母细胞瘤的临床病理形态学及免疫组化特点。方法收集9例嗅神经母细胞瘤的临床和病理资料，用形态学方法和免疫组化染色进行研究。结果按：Kadish分期：A期1例，B期7例，C期1例。组织学特征：瘤细胞呈小圆形或卵圆形，被含有多数毛细血管的纤细结缔组织划分为大小不等的片状或梁索状团块，核圆形或卵圆形，染色质细，胞质少，有Homer-Wright、Flener菊形团，细胞间可见胞质突起形成的嗜酸性纤维。7例中5例NSE、synaptaphysin、chomogranin A( )，阳性率达71．4％；3例NF( )，4例S-100( )，1例vimentiin( )，1例keratin( )．LCA、HMB45、desmin和EMA均为(-)。结论NSE、synaptaphysin、chomograninA对诊断嗅神经母细胞瘤有较高的特异性，其他免疫组化联合检测有助于诊断及鉴别诊断。     

Exercise-unrelated sudden death as the first event of anomalous origin of the left coronary artery from the right aortic sinus

Congenital anomalous origins of the coronary arteries represents a rare but well-described cause of myocardial ischemia and sudden death. Left coronary artery (LCA) arising from the right sinus of Valsalva is a rare congenital coronary anomaly that seems to be commonly associated with sudden death in young trained athletes. The possibility of a coronary artery anomaly should always be considered in young individuals with a history of chest pain or syncope, particularly if the episodes are triggered by exercise. We describe a case of congenital LCA anomaly in an asymptomatic 10-year-old girl with no family history of sudden death; no previous unexplained syncopal episodes or exercise-induced symptoms were reported. She experienced a cardiac arrest while she was resting at school and was not recoverable despite early emergency department admission and intensive prolonged cardiopulmonary resuscitation attempts. Post-mortem pathological findings revealed a single origin from the right sinus of Valsalva for both right and left coronary arteries. The LCA was compressed between the aorta and the pulmonary trunk. Histologic features suggested recent ischemia. Although sudden death can be the first manifestation of this condition, it is important to be particularly aware of prodromic symptoms: exertional dyspnea, chest pain, syncope or dizziness. Recognition during life of this coronary anomaly is mandatory to prevent the risk of sudden death and to plan surgical correction if clinically indicated.     

乳腺原发性恶性淋巴瘤3例

目的 探讨乳腺原发性恶性淋巴瘤组织学类型、临床病理特点及鉴别诊断。方法 收集3例乳腺原发性恶性淋巴瘤临床病理资料,对其进行HE染色和免疫组化标记。结果 3例乳腺原发性恶性淋巴瘤,2例为弥漫性大B细胞淋巴瘤,1例为粘膜相关淋巴组织淋巴瘤;LCA、CD20和bcl-2染色均为阳性,AEI／AE3、CIM5RO、CD10和bcl-6阴性。结论 乳腺原发性恶性淋巴瘤罕见,诊断主要依靠病理组织形态学和免疫组织化学标记。     

B1型胸腺瘤25例临床病理研究

目的 探讨B1型胸腺瘤的临床病理特征。方法 113例胸腺瘤按WHO（1999）胸腺瘤诊断标准分类，对其中25例B1型胸腺瘤运用免疫组织化学标记（CKAEI/AE3、CKpan、EMA、vimentin、LCA和CD20）结合临床影像特征和病理组织形态进行研究。结果 B1型胸腺瘤发病年龄13-69岁，25例均伴有重症肌无力，其中17例呈浸润性生长。组织学表现弥漫增生的淋巴细胞内出现小-中等大小散在分布的肿瘤性胸腺皮质上皮细胞，细胞多数呈圆形，卵圆形，浆少，染色质细，有/无核仁，部分出现纤维组织分割形成不完全性结节，并有血管湖，微囊和胸腺髓质分化。免疫组化显示肿瘤细胞表达CK（pan和AE1/AE3）和vimentin，淋巴细胞表达LCA和CD20。结论 B1型胸腺瘤好发于40岁左右的男性重症肌无力患者，具有浸润性生长特性，是以胸腺皮质上皮细胞为肿瘤成分的低度恶性肿瘤，需与胸腺原发性淋巴瘤鉴别。     

伴神经鞘膜分化的硬化性上皮样纤维肉瘤临床病理观察

目的探讨伴神经鞘膜分化的硬化性上皮样纤维肉瘤的临床病理特征。方法对1例伴神经鞘膜分化的硬化性上皮样纤维肉瘤进行组织病理学观察及免疫组化标记,并复习相关文献。结果肿瘤位于左腹壁浅筋膜下,与神经解剖关系密切。光镜下,肿瘤细胞呈上皮样,单个或巢状埋藏于广泛胶原变性的基质中。胞质透亮或呈弱嗜酸性,胞核卵圆形点彩状或空泡状,核仁明显。大部分区域瘤细胞核异型性较小。电镜下瘤细胞质内可见丰富的粗面内质网及中间丝,部分细胞见细长的胞质突起及外板结构,显示神经鞘膜分化。瘤细胞vimentin（＋）,S-100蛋白、NSE和desmin灶性（＋）,CD117弱（＋）,EMA、CK、CD34、SMA、actin、HMB45、LCA和CD68均（-）。结论伴神经鞘膜分化的硬化性上皮样纤维肉瘤具有典型硬化性上皮样纤维肉瘤的光镜特点,但在临床表现、超微结构及免疫组化上有其特异性。     

肋骨原发性上皮样肉瘤临床病理分析

目的探讨肋骨原发性上皮样肉瘤的临床病理特征、免疫表型及生物学行为。方法对1例原发于肋骨的上皮样肉瘤标本,通过光镜、免疫组化及电镜等方法进行病理组织学观察,并回顾分析相关文献报道。结果本例肿瘤原发于肋骨。肿瘤细胞结节状排列,浸润性生长,瘤细胞大部分由相对独特的上皮样细胞组成,核卵圆形,胞质丰富,嗜酸性,玻璃样。肿瘤中心常见变性坏死。免疫组化示EMA、CK和vimentin( )。电镜显示肿瘤细胞有大量的细丝、细胞间桥和细胞连接。结论原发于肋骨的上皮样肉瘤比较罕见,形态学相对复杂多样,诊断较困难。根据其组织学和病理学特征,结合免疫组化染色结果可以做出明确诊断。     

炎性肌纤维母细胞瘤的影像表现与病理研究

目的:分析炎性肌纤维母细胞瘤的影像表现与病理研究.方法:回顾性分析2013年1月-2020年1月我院手术治疗的10例确诊的炎性肌纤维母细胞瘤患者的CT、MRI影响表现及病理检查结果.结果:10例患者中,2例病灶于胃部,2例病灶于肺部,1例病灶为腹腔,1例病灶为大腿软组织,2例病灶为肠系膜,1例病灶为输尿管,1例病灶为腹...     

肺炎性假瘤的临床病理分析

目的：为提高对肺炎性假瘤的认识及诊治水平，探讨肺炎性假瘤的病理特片。方法：对26例手术切除并经病理证实的炎性假瘤，应用免疫组化LSAB法，进行了5种抗体（Vim、LCA、KP－1、AE1／AE3及VS38－C）的检测。将检测结果与常规病理和临床资料进行分析。结果：HE染色瘤体中淋巴细胞比例与LCA阳性率、组织细胞比例与瘤体间质中KP－1阳性率基本吻合（分别有1例出现轻微差异）。浆细胞数与VS38－C的阳性表达率间存在着明显的差异，除1例阳性表达外，所有浆细胞对VS38－C均无反应。Vim在2例瘤体间质中呈轻度阳性表达。AE1／AE3在瘤组织中呈现不均匀分布，阳性率平均为＋。结论：炎性假瘤与肺癌在影像学上难以鉴别；经检测炎性假瘤中的浆细胞可能多数来自淋巴细胞；假瘤癌变者应系瘤体中残存肺泡上皮和支气管上皮恶变；进一步确立了炎性假瘤应手术切除。     

Mature mediastinal bronchogenic cyst with left pericardial defect: A case report

BACKGROUNDMediastinal bronchogenic cysts and pericardial defects are both rare. It is extremely rare that both occur simultaneously. To the best of our knowledge, this is the first case of a coexistent bronchogenic cyst and pericardial defect reported in China. We performed a literature review and found a relationship between bronchogenic cysts and pericardial defects, which further revealed the correlation between the bronchus and pericardium during embryonic development.CASE SUMMARYA 14-year-old boy attended a local hospital for ankylosing spondylitis. Chest radiography showed an enhanced circular-density shadow near the left mediastinum. The patient had no chest symptoms and the physical examination was normal. Because of the mediastinal occupation, the patient visited our department of chest surgery for further treatment. During surgery, a left pericardial defect was observed. The bronchogenic cyst was removed by thoracoscopic surgery, but the pericardial defect remained untreated, and a satisfactory outcome was achieved after the operation. The patient was diagnosed with a mediastinal tumor. The pathological diagnosis of the tumor was a bronchogenic cyst.CONCLUSIONThis case further reveals the correlation between the bronchus and pericardium during embryonic development.     

脑原发性恶性淋巴瘤免疫组化及病理形态学分析─附6例报告

总结6例脑原发性恶性淋巴瘤，分析其临床症状及光镜、电镜、免疫级化特点。结果表明颅内多发病灶及颅压增高为临床特征。组织学特点为弥漫分布，围绕血管排列。LCA、L26、UCHL1、GFAP、EMA、NSE等免疫组织化学染色及1例电镜检查证实为B细胞性，并对鉴别诊断作了讨论。     

子宫体原发性弥漫性大B细胞淋巴瘤临床病理观察

目的探讨子宫体原发性弥漫性B细胞淋巴瘤的病理诊断与鉴别诊断要点。方法对2例子宫体原发性弥漫性B细胞淋巴瘤进行临床病理特点观察,使用LCA、CD30、CD79a、CD68、CD45RO、CD3、CD20和CEA等免疫组化染色辅助诊断,并文献复习。结果子宫体原发性弥漫性B细胞淋巴瘤临床诊断困难,镜下病理形态多样,可见子宫内膜和/或肌层内淋巴样细胞增生,免疫组化B细胞标记（＋）。结论子宫原发性弥漫性B细胞淋巴瘤少见,临床易漏诊且侵袭性较高,因此准确、完整的病理诊断对预后治疗十分重要,免疫组化有辅助诊断作用。     

Intrahepatic biliary cystadenoma: A case report

BACKGROUNDBiliary cystadenoma (BCA) is a rare benign tumor, accounting for only 5% of reported cystic lesions of the liver. Given its potential for malignancy and high rate of recurrence, surgical resection is the preferred treatment. Therefore, early and accurate preoperative diagnosis is critical to the choice of treatment. We here report the first male case of BCA in our hospital, diagnosed by our team and confirmed by pathological biopsy. This article aims to improve the understanding of this disease and help make a correct diagnosis to better manage it.CASE SUMMARYA 58-year-old man with irregular abdominal discomfort came to our clinic and was found to have a distended abdomen during physical examination. Computed tomography and magnetic resonance imaging both showed a huge cystic mass in the liver. The patient underwent left hepatic lobectomy, cholecystectomy, and liver cyst fenestration, and most of the masses had decreased in size as of the 6-mo follow-up. The pathological diagnosis was consistent with BCA, and no recurrence was detected after the surgery. BCA occurred mainly in middle-aged women. To the best of our knowledge, this patient is the 11th male case of BCA reported in the literature.CONCLUSIONThe combination of magnetic resonance imaging and magnetic resonance cholangiopancreatography is of great significance for the early accurate diagnosis of the disease and the choice of surgical methods.     

Mucinous appendiceal neoplasm: A case report

BACKGROUNDPrimary appendiceal tumors are histologically diverse and have an insidious onset and few specific clinical manifestations. In the majority of cases, these tumors are discovered after appendectomy during pathological exam of the resected tissue. Treatment may include appendectomy (simple or radical) and right hemicolectomy depending on factors such as histological type, tumor size and lymph node/organ involvement. The aim of this case study is to describe a rare case of a giant appendicular mucocele and raise awareness of this condition and its management options and follow-up protocol.CASE SUMMARYWe present the case of a 43-year-old patient who presented to our emergency department with mild right lower quadrant pain. After the initial check-up and imaging exams, he underwent surgery, where a giant 20 cm × 13 cm appendicular tumor was found and resected. Appendicular mucocele was suspected due to the macroscopic appearance and was later confirmed by the pathological exam. The patient’s postoperative evolution was uneventful, and after discharge, he was included in our follow-up program.CONCLUSIONIn conclusion, mucinous appendiceal neoplasms embody a rare pathology; they are asymptomatic or have few, unspecific clinical signs and in many cases are discovered after appendectomy.     

腹膜后结节型节细胞神经母细胞瘤1例报道及文献复习

目的探讨结节型节细胞神经母细胞瘤(GNBn)的病理形态特征、组织分型、预后及诊断要点。方法对1例GNBn进行病理组织学观察和免疫组化检测，并复习文献。结果肿瘤含有肉眼可见并伴有出血的神经母细胞瘤性结节和神经节细胞及成熟神经鞘细胞构成的间质成分。神经母细胞瘤性结节NSE和CgA( )，GFAP、LCA和CK(-)。结论GNBn是一种罕见的混合性肿瘤，仔细的大体检查及广泛取材是诊断的关键；根据肿瘤内结节性质进行组织分型对治疗和预后评估有重要意义。     

A case of Castleman's disease with "downhill" varices in the absence of superior vena cava obstruction

Downhill esophageal varices", classically defined as those that develop in the upper region of the esophagus, are less common than the "uphill" type, which is usually produced by portal hypertension. Various causes of downhill varices have been reported, but mediastinal tumor is the most common responsible lesion. Castleman's disease, or angiofollicular lymph node hyperplasia, is a rare pathological process of unknown etiology that usually develops in the mediastinum. We report the case of a 60-year-old woman whose large esophageal varices were detected incidentally. The cause was a mediastinal mass which was diagnosed as Castleman's disease on histopathological examination of a surgical specimen. This patient's varices most likely formed as a result of copious blood drainage from the tumor into the esophageal veins. Evidence for this was the lack of the classic downhill pattern, the absence of superior vena cava obstruction, and the fact that the varices resolved after the tumor was removed. It is our opinion that this type of varices should be renamed, and we suggest that "overflow varices" would be an appropriate term.     

卵巢原发性恶性黑色素瘤1例及文献复习

目的 探讨卵巢原发性恶性黑色素的临床病理特征,及其与卵巢转移性肿瘤的鉴别诊断。方法 对１例原发于卵巢囊性畸胎瘤的恶性黑色素瘤标本,通过光镜及免疫组化等方法进行病理组织学观察。结果 组织学特征为：肿瘤细胞大小不等,形态多样,界限不清,呈透明细胞和／或印戒细胞样;瘤细胞内外可见一些粗大的黑色素颗粒,瘤组织内血管较多,核分裂现象多见,〉１０个／１０ＨＰＦ。免疫组化ＨＭＢ４５、Ｖｉｍｅｎｔｉｎ及Ｓ－１００     

心脏肿瘤的超声影像与手术病理对照分析

目的:通过对比心脏肿瘤的超声影像特征与手术病理及临床随访结果,总结心脏肿瘤的超声影像特征,提高超声.影像的诊断水平。方法:回顾性对比分析2003年1月一2019年10月我院经彩色多普勒超声心动图检查发现的疑似心脏肿瘤病变63例,对照分析声像图特征与手术病理结果。结果:63例病例中男27例(43%),女36例(57%),年龄4月~83岁,平均35.5岁,手术病理证实良性肿瘤49例,其中黏液瘤28例,其它类型良性心脏肿瘤21例,原发性恶性肿瘤11例,转移瘤2例,血栓1例。超声诊断良性黏液瘤28例,与手术病理结果符合率为100%;23例超声不能判断肿瘤病理类型(20例考虑良性,3例考虑恶性),手术病理结果证实21例为良性,1例为恶性,1例为血栓;12例超声诊断恶性肿瘤,与手术病理定性完全相符。结论:超声预测黏液瘤准确率高,心脏肿瘤如侵犯心脏系统,并伴有心包积液,更有可能为恶性。超声是诊断心脏肿瘤的主要检查手段,通过特殊的声像图特征,结合病史,可避免或减少误诊和漏诊的发生。     

鼻咽部原始神经外胚层肿瘤1例报告并文献复习

目的:提高对鼻咽部原始神经外胚层肿瘤(PNET)病理诊断的认识,减少误诊。方法:报告1例鼻咽部原始神经外胚层肿瘤患者的临床资料、病理特征,并复习相关文献。结果:该病临床无特异性,病理特征有肿瘤细胞体积较小,呈圆形或卵圆形,间质较少,有聚集成团倾向,肿瘤弥漫表达CD99,并不同程度地表达NSE、Syn、Vim、S-100等。结论:PNET是一种预后非常差的恶性小圆细胞肿瘤,认识其临床病理特点及免疫组化表型对于该恶性肿瘤的诊断及临床治疗意义重大。     

Multiple sclerosis and brain tumor: a diagnostic challenge

Multiple sclerosis may present as a lesion indistinguishable clinically and radiologically from brain tumor. A case of multiple sclerosis is reported with clinical and radiologic features of a brain tumor. A brain biopsy was later undertaken that provided pathological confirmation of the diagnosis of this demyelinating disease. This case report in conjunction with those in the literature indicate that conservative treatment with steroid therapy and serial computed tomography should establish the diagnosis of multiple sclerosis without the need for surgical intervention.