原发性肾脏上皮样血管内皮瘤1例报道及文献复习

[目的]探讨肾脏上皮样血管内皮瘤的诊断、鉴别诊断及治疗。[方法]结合文献对1例原发性肾脏上皮样血管内皮瘤的临床资料、病理特征、免疫表型及治疗预后进行分析。[结果]右肾下极肿物,光镜下肿瘤细胞排列成巢状、短梁状、部分区域灶性实性片状;肿瘤细胞呈圆形、多边形和较少的短梭形,瘤细胞胞浆丰富,粉红色,核呈空泡状,有一个不明显的核仁。肿瘤细胞形成小的细胞内管腔,表现为丰富的胞浆内空泡,偶尔含有红细胞,似原始血管管腔;部分区域几个短梭形细胞围成一个小腔隙,为发育较好的血管管腔。免疫表型:VIM、CD34、CD31血管内皮细胞弥漫阳性。随访11个月无复发。[结论]肾脏上皮样血管内皮瘤属罕见的低度恶性肿瘤,具有特征性的组织学结构,其形态学和免疫表型与发生于肾外者相同。治疗上应局部切除并随访。     

肾球旁细胞瘤1例及文献复习

目的:分析肾球旁细胞瘤的临床病理特征、鉴别诊断、生物学行为及预后因素.方法:报道1例肾球旁细胞瘤,通过光镜和免疫组织化学观察其组织病理学特点及免疫学表型特征,并结合临床资料复习相关文献.结果:本例肾球旁细胞瘤患者为女性,55岁,临床无高血压病史,无血钾降低.经肾肿瘤根治术,肉眼检查见肿瘤位于肾上极,大小3.8cm×3.7cm×3.5cm,与周围组织界限清楚.光镜下肿瘤组织呈弥漫性排列,部分区域呈不规则小梁状结构,似血管球瘤样,部分区域似血管外皮瘤样结构,间质内富含薄壁小血管,可见小血管壁玻璃样变性并呈簇状分布,另见大量淋巴细胞浸润.免疫组化示肿瘤细胞表达Vimentin、CD34.结论:肾球旁细胞瘤是一种罕见的良性肿瘤,临床表现缺乏特异性,诊断依靠病理学检查.该肿瘤少部分病例为恶性潜能未定,间质内大量淋巴细胞浸润与愈后关系尚不完全确定.     

腰椎原发性前B淋巴母细胞性淋巴瘤1例报道并文献复习

目的:报道1例腰椎原发性前B淋巴母细胞性淋巴瘤并探讨其临床病理特点、诊断和鉴别诊断。方法:对1例腰椎原发性前B淋巴母细胞性淋巴瘤进行光镜、免疫组化检测和基因重排,并结合文献讨论。结果:镜下肿瘤细胞弥漫分布,细胞大小不一,胞质较丰富,核染色质呈粉尘状。免疫表型:肿瘤细胞CD99、LCA、CD20、CD79a弥漫阳性,CD3灶性阳性,VIM肿瘤细胞阴性,间质阳性,Ki-67增值指数为37%。结论:前B淋巴母细胞性淋巴瘤是一种罕见的高度恶性肿瘤,诊断和鉴别诊断主要依赖其病理形态学特点结合免疫表型。     

小肠组织细胞肉瘤1例报道并文献复习

目的：报道小肠组织细胞肉瘤1例并探讨其临床病理特点、诊断和鉴别诊断。方法：分析1例小肠组织细胞肉瘤光镜、免疫组化结果，并结合文献讨论。结果：镜下肿瘤细胞弥漫分布，细胞为单形或多形性，胞浆丰富，嗜伊红性。瘤细胞体积大，圆或卵圆，部分梭形。核圆或卵圆、可多核型，泡状核，轻到中度异型，核仁明显。免疫表型：肿瘤细胞LCA、CD45RO胞浆阳性，CD68、CD163、S-100、CD117、LYS、HLA—DP、-ATT阳性，SMA、CD1a灶状阳性，ACT、NSE、CD21、CD35、DES、CD23、HIA—DR阴性，Ki-67增殖指数为44％。结论：组织细胞肉瘤是一种少见的淋巴组织恶性肿瘤，诊断和鉴别诊断主要依赖其病理形态学特点并结合免疫表型。     

组织细胞肉瘤六例临床病理学及免疫组织化学特点分析

 目的　探讨组织细胞肉瘤（HS）的病理学特点、特异性标志物的意义及相关的鉴别诊断。方法　采用EnVision免疫组织化学方法检测6例发生于不同部位的HS。结果　6例HS中男性5例，年龄1～65岁，中位年龄18岁，女性1例，77岁。组织形态学：肿瘤细胞弥漫浸润，大小不一，部分区域细胞呈上皮样，胞质丰富嗜酸性，核圆形或不规则形，核仁大而嗜酸，核分裂象多见，个别区域可见双核、多核及瘤巨细胞，6例肿瘤细胞间质中均可见多少不等的炎性细胞。免疫表型：6例患者肿瘤细胞CD68、溶菌酶（lysozyme）、LCA阳性，4例灶性细胞S-100阳性或弱阳性，2例个别细胞CD43阳性、1例个别细胞CD45RO阳性，Ki-67阳性率30 %～90 %，而CD21、CD35、CD4、CD1a、MPO、CD30、CD3、CD20、ALK、HMB45均阴性。结论　HS诊断相对比较困难，需结合肿瘤的镜下形态学改变及免疫表型。LCA、CD68、lysozyme是比较特异性的免疫表型，对HS的诊断及鉴别诊断有较大意义。     

垂体原发性淋巴瘤 1 例并文献复习简

目的：报道1例原发于垂体的罕见的弥漫大B细胞淋巴瘤并结合文献探讨其临床病理特点、诊断和鉴别诊断。方法：对1例垂体原发性弥漫大B细胞淋巴瘤进行常规HE染色及免疫组化染色,分析其组织学特点及免疫表型。结果：镜下肿瘤由排列呈片状的小至中等大小的细胞构成,于蝶窦黏膜内弥漫性生长。瘤细胞形态较一致,圆形,核大、深染,异型性明显,染色质粗块状,核分裂多见,胞浆稀少。免疫表型：肿瘤细胞LCA、CD20、CD79α弥漫阳性,CD99弱阳性,FSH、LH、ACTH、GH、Pro、TSH、AE1／AE3、EMA、CD3、CD45RO、CD117、PLAP均阴性,Ki-67显示肿瘤细胞增殖指数约80％。结论：垂体原发性淋巴瘤（PPL）是一种非常罕见的恶性肿瘤,诊断和鉴别诊断主要依赖病理形态学特点和免疫表型。     

胸膜和胸膜外孤立性纤维性肿瘤临床病理分析

目的 探讨胸膜和胸膜外孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床病理学特点和鉴别诊断以及对预后的指导意义.方法 采用光镜和免疫组织化学方法标志对6例胸膜SFT和8例胸膜外SFT进行分析.结果 14例(包括6例胸膜和8例胸膜外)SFT由比例不同的梭形和卵圆形肿瘤细胞组成,间质胶原纤维丰富伴有分支状血管.其中11例有典型的细胞稀少区和细胞丰富区交替分布特点,2例仅含细胞丰富区成分,1例为多发结节兼有上述两种形态特点.免疫组织化学染色结果显示14例肿瘤细胞均表达CD34、bcl-2和CD99,不表达desmin、S-100、EMA、CK(AE1/AE3)和p53.2例SMA呈部分弱阳性.SFT复发者2例,其中1例胸膜恶性SFT呈结节状播散性累及胸膜.结论 胸膜和胸膜外孤立性纤维瘤在组织学形态、免疫表型和生物学行为上相似.免疫组织化学CD34、bcl-2和CD99表达阳性对SFT的诊断和鉴别诊断具有指导意义.     

血管瘤样纤维组织细胞瘤临床病理观察

为了探讨血管瘤样纤维组织细胞瘤的病理学特征、免疫学表型、诊断及鉴别诊断,分析观察2例血管瘤样纤维组织细胞瘤HE和免疫组织化学(En Vision二步法)染色并复习文献。血管瘤样纤维组织细胞瘤好发于儿童和青少年的四肢,主要有4个组织病理学特点:1)组织细胞样细胞、肌样细胞多结节状增生;2)假血管瘤样腔隙;3)瘤周形成假包膜及淋巴细胞为主的慢性炎细胞浸润;4)瘤细胞表达Vimentin和CD68。组织学上应与皮肤动脉瘤样纤维组织细胞瘤、恶性纤维组织细胞瘤、淋巴结转移性恶性肿瘤、血管瘤(血管内皮瘤和血管周细胞瘤)或血肿、肌纤维母细胞瘤及淋巴结滤泡树突状细胞肉瘤等鉴别。初步研究结果提示,血管瘤样纤维组织细胞瘤是一种少见分化不明确低度恶性潜能的软组织肿瘤,具有特征性的组织学特点。     

发生颅外转移的脑膜血管周细胞瘤临床病理观察

目的探讨脑膜血管周细胞瘤发生颅外转移的临床病理特征、诊断与鉴别诊断要点。方法对9例发生颅外转移的脑膜血管周细胞瘤进行临床病理分析、组织形态学及免疫组化染色观察,结合文献对其,临床表现、病理形态特点及鉴别诊断进行探讨。结果患者年龄32～59岁,男性3例,女性6例。转移部位有肝、肺、骨、腹腔、皮下等。转移间隔时间0．5～10年不等,光镜下瘤细胞为形态大小较一致的短梭形细胞,瘤细胞间富于小血管,呈裂隙状或“鹿角状”,核卵圆形,具有非典型性,核分裂象多少不等,WHOII级或III级。免疫组化瘤细胞波形蛋白均呈强阳性表达,CD34阳性程度不等,弥漫阳性或局灶阳性。结论脑膜血管周细胞瘤临床呈侵袭性,易复发和转移到中枢神经系统外,鉴别诊断包括孤立性纤维性肿瘤、脑膜瘤及间叶性软骨肉瘤等。     

横纹肌样脑膜瘤临床病理观察

目的:探讨横纹肌样脑膜瘤(RM)的临床病理特点和鉴别诊断。方法:回顾分析4例RM的临床表现、组织形态学及免疫表型,并复习相关文献。结果:4例RM患者平均年龄20.5岁,男女各半。受累部位:颅内3例,椎管内1例。临床主要表现为颅压升高和神经系统压迫症状。光镜下肿瘤细胞呈横纹肌样特点,嗜酸性胞浆及偏位核。免疫组织化学染色显示肿瘤细胞EMA和Vim弥漫或灶状阳性,GFAP、HMB45和PR阴性。结论:横纹肌样脑膜瘤非常罕见,具有横纹肌样的细胞形态学特点,免疫组化是正确诊断横纹肌样脑膜瘤的重要依据。     

Juxtaglomerular cell tumor of the kidney

The authors describe a juxtaglomerular cell tumor (JGCT) which caused severe hypertension in a 58-year-old man. Light microscopy showed a circumscribed tumor composed of interlacing cords and occasional nodules of relatively uniform cells with no mitotic activity. Rhomboid crystals characteristic of "prerenin" were present within the cytoplasm of tumor cells, and there was a close relationship between the tumor and unmyelinated nerve axons. Intracytoplasmic renin was demonstrated by immunofluorescence, and tumor granules were shown to contain zinc by electron-beam microanalysis. Review of 14 prior cases, with additional follow-up of 9, showed that no patient had developed recurrence, metastasis, or another tumor. Four patients, however, are hypertensive but probably because of secondary tumor effects rather than recurrent hyperreninism. The distinction of JGCT from hemangiopericytoma with renal involvement is important because of the high mortality associated with the latter. The authors conclude that JGCT is benign, but patients with JGCT may remain hypertensive postnephrectomy because of hypertensive angiopathy.     

四例肾球旁细胞瘤的临床病理学观察

目的 观察肾球旁细胞瘤(JGCT)的临床特征、形态特点、免疫表型,明确其诊断、鉴别诊断、组织发生及预后。方法 对4例组织标本采用光镜观察,PAS、Sweet嗜银组化染色,免疫组化检测CK8、E-cadherin／CK7、CD10、Vim、Actin、desmin、calponin、CD34、HMB45、CD31、S100、Chr、Syn及CD117。电镜观察超微结构。对患者进行随访。结果 4例JGCT患者临床表现为青年人难以控制的严重高血压。肿瘤包膜完整、体积较小。镜下肿瘤细胞以片状排列为主,当有肾小管结构时可见乳头、葱皮样排列;间质含丰富的薄壁血管及少量的厚壁血管;瘤细胞偏小,多角或短梭形,轻度嗜酸,胞膜不清,可见核异型及核分裂相;肿瘤内见多量的肥大细胞,3例见小管结构,并表达远端小管标志物E-cadherin／CK7。肿瘤细胞Vim、Actin、CD34均为( );4例标本电镜下均见特征性的菱形结晶。患者均无复发和转移。结论 JGCT青年人多见,有严重的高血压。病理为独特的形态及超微结构,免疫表型Vim、Actin、calponin、CD34阳性,发生于肾球旁细胞,是独立类型的肾良性间叶肿瘤。     

骨外骨肉瘤病理特征与鉴别诊断4例报道并文献复习

目的 探讨骨外骨肉瘤（extraskeletal osteosarcoma, EOS）的临床病理特征、免疫表型及病理诊断与鉴别诊断要点。方法 收集4例EOS,对其临床、病理组织学及免疫表型进行观察并复习相关文献。结果 4例患者均为成年男性,平均年龄35.5岁。主要表现为无痛性或有触痛性软组织肿块。3例发生于下肢,1例发生于上肢。2例为肌纤维母细胞型EOS,1例为小细胞型EOS,1例为恶性纤维组织细胞型EOS。病理检查：(1)肉眼观见瘤体最大径平均为4cm,切面灰白,质软质脆,界限清楚。(2)镜检:肌纤维母细胞型EOS呈小叶状结构,细胞呈梭形或卵圆形。小细胞型EOS瘤细胞形态一致,弥漫分布。恶性纤维组织细胞型EOS瘤细胞多形性,伴多核巨细胞。（3）免疫组织化学：4例均表达Vim,OC,1例表达OCT。4例均不表达S100、desmin、CD34、NSE及SMA。结论 EOS是一种罕见的软组织肿瘤,其诊断主要依靠组织病理学特征,免疫组织化学标记可帮助诊断和鉴别诊断。     

Chromosome abnormalities in juxtaglomerular cell tumors

BACKGROUND: Juxtaglomerular cell tumors (JGCT; also known as reninomas) are considered benign tumors of the kidney, although there have been reports of both malignant behavior and a JGCT-related death. The clinicopathologic features of these rare tumors are well established, whereas nothing is known about their cytogenetic characteristics. METHODS: The authors reported the first karyotype of a JGCT and also performed comparative genomic hybridization (CGH) and interphase fluorescence in situ hybridization (IP-FISH) analyses on the above-mentioned tumor as well as on another JGCT from which live cells were not available for karyotyping. Both tumors were also examined by electron microscopy and immunohistochemistry. RESULTS: The karyotype was 57 approximately 64,XX,-X,-1,-4,-6,-9,+10,-11,-13,-14, -15,+20,-22[cp10]/60 approximately 61,idem, add(19)(p13)[cp2]/46,XX[3]. The IP-FISH results were in accordance with the karyotypic findings for the first tumor, whereas Tumor 2 was found to be diploid for most investigated chromosomes, except for trisomy for chromosomes 4 and 10 and monosomy for chromosomes 9 and X. By CGH, gain of chromosomes 10 and 20 but no losses were detected for Tumor 1, whereas for Tumor 2, gain of chromosomes 4 and 10 as well as loss of chromosomes 9 and X and most of chromosome arm 11q were found. The immunohistochemical profiles were identical. Both tumors were positive for vimentin and CD34, focally positive for smooth muscle actin, and negative for cytokeratin, CD31, and actin. CONCLUSIONS: Gain of chromosome 10, as well as loss of chromosomes 9 and X and most of chromosome arm 11q, might be important pathogenetic events in JGCT.     

Ollier's disease associated with ovarian juvenile granulosa cell tumor

A six-year-old girl had Ollier's disease (OD) associated with juvenile granulosa cell tumor (JGCT). The clinical manifestations were right hemicorporal deformity due to multiple skeletal enchondromas and precocious pseudopuberty. After the removal of a Stage Iai JGCT, hormonal symptoms disappeared. Neither the ovarian tumor recurred nor the enchondromas underwent sarcomatous change after a follow-up period of 7 years. A review of the literature showed five previous cases, three associated with OD and two with Mafucci's syndrome (MS). In these cases, patients were young and the ovarian tumors were homolateral to the hemicorporal side involved by enchondromatosis. Data provided from these cases emphasize the notion of a generalized mesodermal dysplasia. JGCT behave in association with the OD in its usual fashion of hormonal production and low-degree aggressiveness.     

左上颌窦低度恶性纤维黏液样肉瘤1例并文献复习

目的:报道1例左上颌窦低度恶性纤维黏液样肉瘤探讨其临床病理特点、诊断和鉴别诊断.方法: 对1例左上颌窦低度恶性纤维黏液样肉瘤进行光镜和免疫组化检查,并结合文献讨论.结果: 肿瘤分两个区域:胶原化区域和黏液样区域,两种区域交替排列,瘤细胞为梭形或不规则形,染色质呈细块状,偶可见核仁.免疫表型:肿瘤细胞 AE1/AE3、EMA、CK、ACT、LCA、CD1α、S-100均阴性,SMA、CD68局灶性阳性,LYS弱阳性,Vim、CD163、CD45RO阳性.结论: 低度恶性纤维黏液样肉瘤是一种罕见的低度恶性肿瘤,诊断和鉴别诊断主要依赖其病理形态学特点并结合免疫表型.     

影响甲状腺癌术前诊断正确性的因素

[目的]探讨甲状腺癌术前诊断与术后病理诊断不符的可能原因。[方法]回顾分析48例甲状腺癌的临床特点和诊治经过，总结术前诊断与术后病理诊断的符合情况，并分析术前、术后诊断不符的因素及可能导致的后果。[结果]48例甲状腺癌术前诊断与术后病理诊断不符25例，其中术前诊断甲状腺良性肿瘤或类肿瘤19例，桥本氏甲状腺炎1例．术前诊断淋巴结炎性病变5例，25例中再次手术18例。[结论]对甲状腺癌的临床和影像学特点认识不足是术前诊断与术后病理诊断不符的主要原因，术前、术后诊断不符往往导致首次治疗不足。     

Juvenile granulosa cell tumor of the ovary in children: a clinical study of 15 cases

Juvenile granulosa cell tumor (JGCT) in children accounted for 12% of all ovarian tumors treated in the Institut Gustave-Roussy (IGR) Pediatric Department from 1967 to 1985. The median age of the 15 girls was 8 years 7 months (range, 22 months to 15 years 7 months). Precocious pseudopuberty was present in six of the seven girls under 8 years. Of the other seven girls, one developed virilization symptoms. Surgery was the first treatment in each case. According to the Wollner classification, there were six stage I, one stage II, six stage III (including four ruptured tumors), and one stage IV JGCT cases. One patient was not available for staging. An adjuvant treatment (five chemotherapy and one radiotherapy combined with chemotherapy) was administered to six patients. Eleven girls are alive and free of disease, with a median follow-up of 6 years (range, 2 to 18 years). Four girls relapsed 6 to 17 months after surgery and died. Two of these relapses occurred in bone. The prognosis for JGCT in children is favorable for the lower stages when treated with surgery, but the best treatment for extensive and recurrent disease has yet to be determined.     

9例低度恶性肌纤维母细胞肉瘤的临床病理观察

目的：探讨低度恶性肌纤维母细胞肉瘤(LGMS)的临床病理特征及其治疗。方法：分析9例LGMS的临床和病理特征，并进行免疫组化标记。结果：9例均行手术切除，其中3例第一次手术为广泛切除术。本病肿瘤细胞特点为胞浆嗜酸而核两端尖，弥漫性广泛表达SMA和MSA。7例获随访资料，2例分别于术后5个月和7年复发，后者半年后2次复发并转移至舌。结论：LGMS是光镜下可辨认的低度恶性肉瘤，其临床重要性在于与假肉瘤性病变鉴别，手术切除干净并密切随访以避免复发。