近端型上皮样肉瘤3例临床病理分析

目的:探讨近端型上皮样肉瘤的临床病理特征、诊断要点、免疫表型、鉴别诊断及生物学行为.方法:分析3例上皮样肉瘤的临床病理资料,观察其组织病理学形态及免疫表型特征,对其进行随访.结果:女2例,男1例.发病年龄24-34岁.位于头部2例,肩部1例.临床均表现为无痛性生长的单一结节状肿块.肿瘤细胞呈圆形、卵圆形及梭型,含有均质状嗜酸性胞质,细胞核空泡状,核仁明显,一个或多个,见肉芽肿样改变及出血囊性变.肿瘤细胞波形蛋白(Vimentin)、细胞角蛋白(AE1/AE3)、CD34阳性表达,上皮膜抗原(EMA)部分阳性表达.阴性表达的是 CD31、FⅧ因子、Mac387、CD68、S-100蛋白、SMA、HMB-45、desmin、CD240.1例死于发病后14个月,1例于发病6个月后出现复发,另1例随访个5月无复发和转移.结论:近端型上皮样肉瘤的组织病理学特征是:肿瘤细胞呈结节状分布,有上皮样结构,肿瘤细胞有弥漫性嗜酸性胞质,核大,空泡状,核仁明显,核分裂相多见.瘤细胞共同表达Vimentin、AE1/AE3、CD34,部分病例瘤细胞表达EMA;需与上皮样血管肉瘤、滑膜肉瘤、上皮样恶性神经鞘瘤鉴别诊断.其生物学行为高度恶性.     

颈部复发性滤泡树突细胞肉瘤1例并文献复习

目的 分析头颈部滤泡树突细胞肉瘤的临床特征、治疗方式和预后.方法 复习1例颈部复发性滤泡树突细胞肉瘤的治疗及随诊资料,结合相关文献探讨其临床特征以及治疗、预后.结果 颈部滤泡树突细胞肉瘤主要表现为无痛性肿块;肿瘤细胞呈纺锤形/卵圆形,呈束状、旋涡状排列;免疫组化CD21和CD35阳性.复发后二次手术挽救加术后放疗,1年后死于肺转移,未见局部复发.结论 颈部滤泡树突细胞肉瘤是一种少见的低到中度恶性肿瘤,需借助免疫组化的方法与其他组织学形态类似的肿瘤鉴别,治疗多数以手术为主,伴有病理不良预后因素、复发或残存时辅助放疗/化疗.     

妊娠合并肝血管肉瘤破裂1例报道及文献回顾

目的:报道1例妊娠合并肝脏血管肉瘤破裂,结合文献探讨其临床病理特征、诊断及鉴别诊断要点。方法:对1例妊娠合并肝血管肉瘤进行临床病理分析及免疫组化观察。结果:肝血管肉瘤无特殊临床表现及影像学检查,镜下肿瘤组织由大小不一、互相吻合管腔构成,部分区域瘤细胞沿肝窦浸润性生长,管腔细胞异型性明显,核分裂象易见。免疫组化结果:肿瘤细胞表达CD31、CD34、FACTOR VⅢ。结论:肝血管肉瘤为罕见疾病,合并妊娠未见报道。具有独特的临床病理特点,诊断及鉴别诊断主要依靠病理组织学及免疫组化,治疗以手术切除为主,必要时进行化疗及放疗,胎儿应结合孕期综合处理。     

6例髓系肉瘤的临床病理分析

目的 探讨髓系肉瘤（MS）的临床病理学特征、免疫学表型、鉴别诊断、治疗及预后。方法 收集6例MS患者临床病理资料,对标本采用常规石蜡切片行HE染色,EnVision法免疫组化染色,骨髓涂片检查,并对生存进行随访。结果 6例MS中男女各3例,年龄范围23～66岁;发生部位为小肠1例、上腭1例、手掌1例、结肠1例、肺1例、宫颈1例。细胞形态学特点为肿瘤细胞弥漫浸润,散在分布,互不粘附;肿瘤细胞中等大小,形态较单一,细胞核大,呈圆形或卵圆形,胞浆少,染色质细腻而均匀分布,2例核仁清晰,大小不一,部分可见嗜酸性粒细胞。免疫组化标记不同程度表达MPO、CD43、CD117、CD68-KP1、CD13、CD15、Lysozyme,结合病理组织学形态及免疫组化,6例均符合粒细胞肉瘤。随访期内3例伴急性髓系白血病确诊后分别于5、6、11个月死亡,其余3例仍存活。结论 MS为一种罕见以髓系细胞形态学和免疫表型特征的恶性肿瘤,需在形态学的基础上辅以免疫组织化学进行诊断。     

甲状腺滤泡树突状细胞肉瘤2例临床病理特征与免疫表型分析

目的探讨甲状腺滤泡树突状细胞肉瘤(FDCS)的临床病理特征与免疫表型特征。方法回顾性分析2例甲状腺FDCS患者的临床病理资料。结果 2例患者中男性1例,女性1例。肿瘤直径为2.6、5.0 cm。肿瘤细胞多呈梭形、卵圆形或圆形;细胞核呈长梭形或圆形,染色质呈细颗粒状或空泡状,核仁小而明显,核分裂相易见;细胞质丰富淡染或嗜酸;细胞间界限不清。肿瘤细胞排列呈片状、束状、席纹状、旋涡状及模糊的结节状结构。间质伴较多淋巴细胞浸润,并可见血管袖套状结构。免疫组化染色示:肿瘤细胞弥漫阳性表达CD21、CD23、CD35(2/2)和vimentin(1/2),以及弱阳性表达CD68(1/2)。结论甲状腺FDCS与滤泡树突状细胞具有相似的组织学形态特征,免疫表型分析对该病的诊断有重要价值。     

腹膜后滑膜肉瘤6例临床病理及分子病理学分析

目的:探讨腹膜后滑膜肉瘤的临床病理特征、免疫表型及分子遗传学改变。方法:收集北京大学国际医院病理科2014年12月至2021年07月期间手术切除腹膜后标本病理诊断为滑膜肉瘤6例,进行光镜观察、免疫表型及分子病理学分析,并复习相关文献。结果:男性2例,女性4例,年龄12~56岁（平均年龄29岁,中位年龄26岁）;肿瘤大小8~20 cm,平均15.2 cm,界限较清,梭形细胞型5例,双相型1例;CKpan、EMA、Bcl-2、CD99、TLE1、Vimentin、CD34、Calponin阳性比例分别为5/6、6/6、6/6、3/6、6/6、5/6、0/6、4/6,INI1呈特殊模式表达6/6,Ki-67高表达;FISH结果显示6例均有SS18-SSX基因融合;3例为SS18-SSX2基因融合,并有伴随基因突变。结论:腹膜后滑膜肉瘤是少见的发病部位,明确诊断常需结合组织病理学形态、免疫组织化学表达及分子遗传学特征进行综合分析。     

鼻腔T细胞淋巴瘤8例分析

目的:探讨鼻腔T细胞淋巴瘤临床病理特点.方法:对8例鼻腔T细胞淋巴瘤的临床病理资料进行回顾性分析.并用免疫组化SP法作LCA、CD3、CD45RO、CD20、CD56、CK等染色.结果:8例发病年龄35岁～70岁,平均年龄60岁,农民7例(7/8).病变位于右鼻腔7例,左鼻腔1例.临床均有鼻塞、流脓涕等症状.病理组织学特点为肿瘤细胞大小不等,呈弥漫排列,核形多不规则,胞浆丰富、透明或淡染;6例以中小细胞为主,2例以中大细胞为主.免疫组化染色:8例肿瘤细胞表达LCA、CD45RO、CD3,不表达CD20、CD56、CK.随访3例,2例死亡,生存期分别为14年零6个月、3年零5个月.结论:鼻腔T细胞淋巴瘤好发于老年农民,右鼻腔多见,病理组织学形态多样、易误诊为癌或炎性病变等.早期正确治疗可改善预后.     

透明细胞肉瘤的病理诊断与鉴别诊断

目的 掌握透明细胞肉瘤的临床病理特点及组织学不典型性。方法 光镜观察3例透明细胞肉瘤组织形态结合免疫组化染色结果分析。结果 女性2例，男性1例，年龄 27－50岁，病变部位主要位于四肢远端，组织学显示，该肿瘤细胞组织形态不典型，特别需要和滑膜肉瘤，上皮样肉瘤，恶性腱鞘巨细胞瘤鉴别。 所有病例HMB－45和S－100均表达阳性。结论 透明细胞肉瘤常发生于20至40岁的中青年，透明细胞肉瘤的病理学特点及鉴别诊断紧密结合临床表现和免疫组化结果。     

乳腺腺泡型横纹肌肉瘤1例及文献复习

目的：探讨乳腺腺泡型横纹肌肉瘤的病理诊断及鉴别诊断。方法：对1例乳腺腺泡型横纹肌肉瘤进行HE切片、免疫组化染色等观察并复习文献进行讨论。结果：镜检肿瘤细胞排列杂乱，可见部分呈腺泡及假腺管状，细胞呈短梭及圆形，胞浆丰富红染，核大偏位。肿瘤细胞特异性表达MyoD1。结论：乳腺腺泡型横纹肌肉瘤是非常罕见的恶性肿瘤，其肿瘤结构、细胞形态都有一定的特征，确诊需免疫组化染色的帮助，应与小细胞类型以及乳腺间叶组织可能发生的其他肉瘤相鉴别，同时横纹肌肉瘤的亚型区分对治疗也很重要。     

妊娠合并肝血管肉瘤破裂1例报道及文献回顾

目的：报道1例妊娠合并肝脏血管肉瘤破裂,结合文献探讨其临床病理特征、诊断及鉴别诊断要点。方法：对1例妊娠合并肝血管肉瘤进行临床病理分析及免疫组化观察。结果：肝血管肉瘤无特殊临床表现及影像学检查,镜下肿瘤组织由大小不一、互相吻合管腔构成,部分区域瘤细胞沿肝窦浸润性生长,管腔细胞异型性明显,核分裂象易见。免疫组化结果：肿瘤细胞表达CD31、CD34、FACTOR VⅢ。结论：肝血管肉瘤为罕见疾病,合并妊娠未见报道。具有独特的临床病理特点,诊断及鉴别诊断主要依靠病理组织学及免疫组化,治疗以手术切除为主,必要时进行化疗及放疗,胎儿应结合孕期综合处理。     

Pulmonary metastases from adrenal cortical carcinoma: Results of resection

Adrenal cortical carcinoma is uncommon. There is little controversy concerning treatment of the primary tumor. However, data concerning the treatment of pulmonary metastases are sparse. In order to assess the results of therapy, we reviewed our 14-year experience. Records of 24 patients admitted to our institution from 1973 to 1991 with the diagnosis of adrenal cortical carcinoma and pulmonary metastases were reviewed. Ten patients underwent pulmonary resection, 12 received chemotherapy, and 2 no therapy. In the pulmonary resection group, treatment consisted of wedge resection in 8 patients and lobectomy in 2. For the 10 patients undergoing resection of their pulmonary metastases, the 5-year survival was 71% (median not reached at 5 years). This was significantly longer than those not resected, with no one alive at 3 years (median survival 11 months). Our data suggest that those patients who are able to have their pulmonary metastases resected survive significantly longer than those who do not. © 1993 Wiley-Liss, Inc.     

Anomalous unilateral single pulmonary vein: Two cases mimicking arteriovenous malformations and a review of the literature

Total anomalous pulmonary venous drainage is a rare congenital anomaly. It usually involves a pulmonary to systemic venous shunt and most cases have a septal defect in order to survive. Anomalous pulmonary venous drainage with pulmonary venous shunting is an extremely rare and entirely benign entity. We present two such cases, in which there was atresia of the left superior pulmonary vein and drainage via a tortuous collateral vein to the left inferior pulmonary vein. This collateral was mistaken on plain film and CT for a pulmonary arteriovenous malformation. Awareness of this anomalous unilateral single pulmonary vein and its radiological appearances may help in avoiding unnecessary pulmonary angiography.     

Turbulent flow artefact mimicking central pulmonary emboli in pulmonary hypertension: A report of two cases

Many artefacts can mimic pulmonary emboli (PE) on CT pulmonary angiography (CTPA), most commonly in the peripheral pulmonary arteries (PA's). We describe flow‐related artefact mimicking PE in the central PA's of two patients, both with pulmonary arterial hypertension (PAH). To our knowledge, this is the first report of this PE mimic in the central PA's.     

Systemic‐pulmonary artery shunt: A rare cause of false‐positive filling defect in the pulmonary arteries

Computed tomographic (CT) pulmonary angiography is widely used for evaluation of pulmonary embolism. Intraluminal filling defects in the contrast enhanced pulmonary arteries are considered diagnostic of pulmonary emboli. We report a false‐positive case where filling defects in the pulmonary artery were due to mixing of blood from a complex shunt or vascular malformation between the systemic arteries and pulmonary artery.     

Uncommon pulmonary metastasis presenting as pulmonary infarction with tumour emboli in two cases

We report two cases of pathologically proven pulmonary metastases presenting as pulmonary infarction with tumour emboli. In two cases, high-resolution CT showed multiple small subpleural consolidations in both lungs. The patients had breast cancer and uterine cervix cancer, respectively. Although various pulmonary diseases show subpleural consolidations on CT, pulmonary metastases by tumour emboli should be primarily considered in differential diagnosis especially when the patient has known malignancy.     

胸腔镜辅助小切口肺癌根治术102例

背景与目的：随着胸腔镜（Video—assisted thoracoscopic surgery，VATS）技术的迅速发展，现在大多数肺癌的外科治疗可以安全地在胸腔镜下手术完成，一些胸外科医生很关注胸腔镜手术的安全性和彻底性。胸腔镜辅助小切口（Video—assisted mini—thoracotomy，VAMT）扩展了手术的适应范围，使解剖上彻底的肺叶切除术及淋巴结清扫更容易。本文旨在探讨和总结胸腔镜辅助小切口肺叶切除术的临床价值。方法：总结102例胸腔镜辅助小切口下肺叶切除的手术方法及临床效果。结果：全组无手术死亡及严重并发症。平均手术时间与术后胸腔引流管时间分别为126分钟和3．5天，术中出血平均165ml。术后疼痛轻、恢复快，3年生存率87．8％和传统开胸手术组无差异。结论：胸腔镜辅助小切广1肺叶切除及淋巴结清扫安全、彻底，效果确切。只要严格掌握手术适应证，运用合理的手术方法和技巧，一般可以达到与常规开胸同样的治疗效果，扩展了胸腔镜手术的适应范围。     

Pulmonary scintigraphy in suspected pulmonary embolism: How often does it change clinical management?

The effect of pulmonary scintigraphy on patient management in suspected pulmonary embolism has been assessed in 158 patients. A change in management occurred in 30% following scintigraphy. The major effect was a reduction in the total number of patients who were anticoagulated. Heparin was stopped in 42 of the 74 patients to whom it had been administered prior to the scintigram.     

贝莱联合肺癌合剂在老年性肺癌放疗中的疗效观察

目的:对贝莱联合肺癌合剂在老年性肺癌放疗中减轻放射性肺损伤的疗效进行评价。方法:62例老年性肺癌接受放疗,随机分为治疗组(放疗 贝莱 肺癌合剂)31例和对照组(单纯放疗)31例,进行为期至少6个月的治疗观察。所有病例均随访2年。贝莱用法:30mg/次,每日3次;肺癌合剂每日1剂,从放疗首日开始治疗组服用直至放疗结束,并连续服用3个月。放疗方案:采用西门子6MV-X直线加速器,单次剂量1.8Gy~2.0GY/次,1次/日,每周5次。总剂量约为60GY左右。结果:62例老年性肺癌放疗后,治疗组出现急性放射性肺炎6.45%,对照组为25.8%。两组对比有显著差异(P<0.05)。结论:贝莱联合肺癌合剂在老年性肺癌放疗中能减少和预防放射性肺损伤的发生。     

Anomalous unilateral single pulmonary vein versus scimitar syndrome: Comparison of two paediatric cases and a review of the literature

The 'scimitar sign' on plain chest radiograph is most commonly attributed to the presence of a scimitar vein, which is a variant of partial anomalous pulmonary venous drainage (PAPVD). An anomalous unilateral single pulmonary vein (AUSPV) is a very rare anomaly, albeit benign, which can also present with the scimitar sign. Despite a similar radiographic appearance, these two anomalies have very different prognostic implications. Moreover, differentiation between the two is important in the paediatric population to prevent unnecessary investigations. We present two paediatric patients who presented with the scimitar sign on plain chest radiograph, with one having PAPVD and the other having AUSPV, and provide a review of the literature on AUSPV, an anomaly which most likely shares a similar pathogenesis to the scimitar vein of PAPVD.     

同时支气管肺动脉联合成形术治疗中央型肺癌

背景与目的：近年来支气管肺动脉联合成形术愈来愈广泛的应用于肺功能不佳的肺癌患者中，该术式符合最大限度地切除肺癌和最大限度地保留肺功能的肺癌手术原则，该项手术技术有着很好的前景。我们总结近年来采用支气管肺动脉联合成形术治疗中央型肺癌的体会来探讨该项手术的应用指征和方法。方法：1993年12月-2003年12月，对63例中央型肺癌施行支气管肺动脉联合成形肺叶切除，包括支气管肺动脉双袖状切除术43例，其中右肺上叶袖切＋肺动脉袖切20例；左肺上叶袖切＋肺动脉袖切23例；切除肺动脉的长度为1．5～4．5cm，平均2．5cm．支气管肺动脉双禊形切除术3例；其中右肺上叶禊切＋肺动脉楔切2例；左肺上叶楔切＋肺动脉楔切1例。支气管袖切并肺动脉禊切17例，其中右肺上叶袖切＋肺动脉楔切5例；左肺上叶袖切＋肺动脉禊切12例。结果：本组无死亡及吻合门瘘发生：结论：支气管肺动脉联合成形术使肺癌手术指征扩大，符合最大限度地切除肿瘤及最大限度保留肺功能的肺癌手术基本原则，是一种安全、有效、可行的术式。