脊索瘤样脑膜瘤1例并文献复习

目的:探讨脊索瘤样脑膜瘤(chordoid meningiomas,CM)的临床与病理特点.方法:应用组织病理学、组织化学以及免疫组织化学方法对1例CM进行观察,同时复习相关文献进行讨论.结果:CM病理特点为黏液样基质中有呈分叶状或条索状排列的肿瘤细胞,细胞质呈淡染或嗜酸性染色,部分细胞呈液滴状,分布于黏液样基质中,类似脊索瘤样细胞;肿瘤细胞中亦可见典型脑膜上皮细胞区域.组织化学染色阿尔辛蓝-过碘酸-Schiff反应(alcian blue and peridic acid-schiff's reacyion,AB/PAS)呈阳性反应.免疫组织化学染色显示:肿瘤细胞波形蛋白(vimentin)、上皮膜抗原(epithelial membrane antigen,EMA)表达阳性,孕激素受体(progesterone receptor,PR)灶状阳性,广谱细胞角蛋白(pan cytokeratin,CKpan)、S-100钙结合蛋白(S-100 calcium binding protein,S-100)表达阴性.结论:CM是发生于颅内的较少见的脑膜瘤亚型,需要与脊索瘤、黏液性软骨肉瘤及转移性黏液腺癌等鉴别.     

副脊索瘤一例

患者男，２５岁。右肩背肿物２年余，无任何症状，于１９９６年４月２０日入院。体检：右侧肩背部肿物略突出皮肤表面，手术切除肿物２ｃｍ×２ｃｍ×１ｃｍ，包膜完整，切面呈灰白色。镜下观察：肿瘤细胞呈巢状多结节分布，被纤维结缔组织分隔成小叶状。瘤细胞呈较一致的圆形，胞质丰富，大部分呈空泡状，少部分胞质嗜酸，棱卵圆形，空泡状，可见核仁，未见核分裂象。大部分区域可见淡蓝色粘液样背景。组织化学染色：淡蓝色粘液样基质呈阿尔辛蓝阳性，部分胞质ＰＡＳ阳性。网织纤维染色：网状纤维丰富，呈小巢状包绕瘤细胞。免疫组化染色：…     

脊索瘤2例临床病理特征并文献复习

目的：探讨脊索瘤的临床病理特征、诊断及鉴别诊断、治疗和预后。方法：对本研究组的2例脊索瘤的临床病理特征和免疫组织化学特点进行分析,并结合相关文献复习。结果：脊索瘤瘤细胞呈分叶状结构,细胞散在或呈条索及小巢状,液滴状细胞体积较大,胞浆中含大小不等的空泡;另一种体积小呈星芒状,且胞浆内无空泡。免疫组织化学显示,1例瘤细胞CK、EMA、S-100、Vimentin阳性表达,另1例CK、EMA、Vimentin阳性表达,而S-100阴性。结论：脊索瘤发病率低,但根据其常见的发病部位及特征性的组织形态,结合免疫组织化学方法,有助于其诊断及鉴别诊断。     

良性脊索细胞瘤3例并文献复习

目的探讨良性脊索细胞瘤(benign notochordal cell tumor,BNCT)的临床病理学特征、诊断和鉴别诊断。方法回顾性分析3例BNCT临床病理学和免疫表型特征,并复习相关文献。结果 3例BNCT中女性2例,男性1例,年龄分别为27、59和29岁。眼观:3例肿瘤均为胶冻状碎组织,灰白、灰黄色,质软、脆。镜检:肿瘤由胞质透亮的脂肪样细胞和胞质淡染细胞构成,两种细胞呈巢状或片状排列。肿瘤细胞形态温和,未见核分裂象。肿瘤组织中未见分叶状结构、细胞外黏液样基质和坏死。免疫表型:肿瘤细胞均高表达CKpan、CK7、CK18、EMA、vimentin和S-100。结论 BNCT是一种罕见的良性脊索源性肿瘤,预后较好。BNCT需与脊索瘤、转移癌和脂肪组织鉴别。     

Wolffian附件肿瘤3例并文献复习

目的探讨Wolffian附件肿瘤(Wolffian adnexal tumor,WAT)的临床病理学特征、鉴别诊断及其预后。方法对3例WAT进行临床病理学观察和免疫组化标记,并复习相关文献。结果 3例患者年龄分别为25、49及55岁,肿瘤分别位于后腹膜、左侧输卵管系膜及右侧卵巢。镜下肿瘤细胞呈弥漫实性或筛网状分布,弥漫实性区可见成片稍肥胖的梭形或卵圆形细胞及紧密排列的管状结构,管腔内衬立方或柱状上皮;瘤细胞无明显异型,核分裂罕见;管周有PAS阳性的基膜样物质。免疫表型:3例瘤细胞CKpan、vimentin、Calretinin均阳性,CD99阳性1例,α-inhibin阳性2例,PR、CK7、EMA均阴性。结论 WAT是一种罕见的具有低度恶性潜能的肿瘤,需进行长期密切随访。     

肋缘肌间恶性血管球瘤1例报道并文献复习

目的 探讨恶性血管球瘤(malignant glomus tumor,MGT)的临床病理特点、诊断和鉴别诊断.方法 对1例发生在右侧第11肋弓处的MGT进行组织学和免疫组化观察,结合文献复习.结果 右侧肋缘下肿物大小6 cm×7 cm,质韧,可活动,边界清晰,无压痛,放射痛.上腹部增强CT:右侧第11肋弓处可见一软组织肿块影,边界光滑,其内密度不均匀,中心可见斑片状低密度影,大小5.8 cm×4.17 cm,局部肋骨可见斑片状骨质破坏.光镜下肿瘤细胞围绕裂隙状血管弥漫性生长并浸润周围脂肪组织.瘤细胞大小较一致,圆形或多角形,胞质丰富,淡红染,细胞界限清晰.核圆形或卵圆形,染色质粗糙,核仁明显,有异型,核分裂9个/10 HPF,可见病理性核分裂.肿瘤组织间纤维间质少,有丰富扩张的小血管,可见多灶坏死和出血.免疫表型:肿瘤细胞表达SMA、h-caldesmon、vimentin、p53、BCL-2、CD99、Collagen IV和Ki-67,p63、HMB-45、CK、EMA、desmin、S-100、CD34、TTF-1、calretinin、CD68、Lysozyme、CD3、CD20、CD30为阴性.结论 MGT是罕见的软组织恶性肿瘤.确诊必须依赖于临床特点、组织形态及免疫组化结果,且需要和具有血管周上皮样细胞分化特点的肿瘤及血管外皮细胞瘤等其他肿瘤予以鉴别.     

颈静脉球瘤1例及副神经节瘤文献复习

目的探讨颈静脉球瘤的临床病理特征和副神经节瘤的生物学行为。方法对1例颈静脉球瘤进行组织学观察和免疫组化标记,同时复习相关文献。结果颈静脉球瘤的组织学改变类似于其他部位的副神经节瘤,免疫组化标记:瘤细胞Syn、CgA和NSE均阳性,EMA、CK、HMB45、CD34和CD99均阴性。结论颈静脉球瘤是一种起源于副神经节的少见肿瘤,呈侵袭性生长,有低度恶性的生物学行为。根据肿瘤的组织学特征,结合CT或MR I影像学资料和免疫组化标记可以作出诊断。     

右心房黏液瘤临床病理分析并文献复习

目的：探讨右心房黏液瘤的临床病理学特点及诊断要点。方法：对2例右心房黏液瘤进行临床资料、病理形态学及免疫组织化学观察,并结合文献对其诊断及鉴别诊断进行探讨。结果：镜下黏液瘤细胞呈单个散在、条索状或呈血管形成的指环样结构排列,黏液样基质在血管周围形成晕环;瘤细胞形成原始或分化的血管趋势,基质内淋巴细胞、浆细胞浸润;瘤细胞细长、梭形或星状,胞浆嗜酸性,胞核卵圆形、圆形或细长形;瘤细胞聚集呈球状,胞浆嗜酸性,胞核卵圆形或梭形。结论：右心房黏液瘤,非常少见,因此缺乏对其认识,从而易与其他肿瘤混淆导致误诊。提高对心房黏液瘤的认识,对避免误诊是至关重要的。     

骨外黏液样软骨肉瘤病理与鉴别诊断附4例报道并文献复习

目的 探讨骨外黏液样软骨肉瘤(extraskeletal myxoid chondrosarcoma,EMC)的临床病理特点、免疫表型及病理诊断与鉴别诊断要点.方法 收集4例EMC,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40.2岁,平均病程约30.3个月.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例发生于胸壁.病理检查:(1)眼观见瘤体最大径平均6.5 cm,切面呈多结节状,有黏液感,界清.(2)镜检见肿瘤呈分叶状生长,瘤细胞排列呈索状、簇状或纤细网状.3例间质富于黏液样基质,1例富于软骨样基质,血管较稀疏.免疫组化:4例均表达Vim(4/4),3例表达S-100蛋白(3/4),2例表达NSE(2/4),2例表达CgA和Syn(2/4),1例表达CKpan(1/4),1例Ki-67阳性率为50%,3例Ki-67阳性率均<5%,4例均不表达SMA和EMA.结论 EMC是一种罕见的软组织肿瘤,其诊断主要依靠发生部位和组织病理学特征,免疫组化标记可帮助诊断和鉴别诊断.     

Infant pleuropulmonary blastoma: report of a rare case and review of literature

In infants, pleuropulmonary blastoma is a rare but aggressive tumor. The typical histopathological presentation includes the aggregation of malignant primitive small cells, usually observed in sheets. So as to provide proper and timely treatment, the differential diagnosis includes pulmonary blastoma, sarcomatoid mesothelioma, fetal rhabdomyoma, synovial sarcoma, and primitive neuroectodermal tumor. Herein, we will present one male pediatric patient with pleuropulmonary blastoma. The patient was a 4-month-old male infant, who had a prolonged cough and dyspnea for 4 months that was complicated by cyanosis for 3 days. A physical examination revealed a solid mass in the right lung that was sized 9.0 × 6.0 × 4.0 cm and had a grayish-white cross section. The boundary between the mass and lung tissue was clear; the mass already occupied a great portion of the lung. A microscopic examination suggested that the tumor was composed of round or orbicular-ovate primitive fetal cells. The cells were medium sized, having little cytoplasm, but had a clearly visualized nucleolus and active karyokinesis. The tumor mass was biphasic, namely, fasciculated sarcoma (composed of spindle-shaped cells and short spindle-shaped cells) and malignant fibrous histiocytoma containing well-differentiated cartilage islands or cartilaginous nodes. Immunohistochemistry was performed for further detection: vimentin (+), S-100 protein (+), CK (AE1/AE3), EMA and TTF-1 in residual epithelial components (+), NSE (focal +), SMA (mesenchymal cells, focal +), CD99 (weak +), Bcl-2 (weak +), desmin (-), myoglobin (-), calretinin (-), calponin (-), FLI (-), MyoD-1 (-), and CD34 (-). Pleuropulmonary blastoma is extremely rare but highly aggressive neoplasm in children. Its typical histopathological presentation is the aggregation of primitive malignant small cells. Combining imaging and histopathological examinations and clinical data should help in determining the diagnosis of pleural pulmonary blastoma.     

Clinicopathological findings of primary esophageal malignant melanoma: report of six cases and review of literature

We studied images and histopathological features of primary esophageal malignant melanoma to explore the clinical pathological features, diagnosis, differential diagnoses, and treatment. Immunolabelling was conducted on six cases of esophageal malignant melanoma using histological and immunohistochemical techniques. Combined with the related literature, the clinical manifestations, imaging, histopathological and immunohistochemical features, treatment, and prognosis of primary esophageal malignant melanoma were observed and analyzed. The six patients with primary esophageal malignant melanoma were all male with an average age of 63.4 years. Poor food intake was observed in all patients, and the symptoms showed progressive aggravation. Endoscopic feed tube revealed dark brown and black nodular and polypoid lesions, 1/4-1/2 loop cavity. Tumor histopathology revealed the following characteristics: tumor cells arranged in nests, sheets and cords, round or polygonal, abundant and red-stained cytoplasm, melanin granules in the cytoplasm, heterogeneous nucleus sizes, centered or deviated nuclei, clearly identifiable nucleoli, and apparent pathological mitosis. The immune phenotype was as follows: tumor cells had diffuse expression of HMB45, Melan A, and S100. The cells were CK negative, and the Ki67-positive cell number was 40%-45%. Primary esophageal malignant melanoma is rare with high malignancy and poor prognosis. Immunohistochemical staining is helpful for diagnosing this tumor. The differential diagnosis includes low differentiated carcinoma, primitive neuroectodermal tumor, esophageal sarcomatoid carcinoma, esophageal lymphoma, and other tumors.     

软骨样脂肪瘤1例报道及文献复习

目的探讨软骨样脂肪瘤的临床、病理学特点和鉴别诊断。方法报道1例右小腿软骨样脂肪瘤的临床资料、光镜、组化、免疫组化及电镜观察结果,结合文献讨论。结果镜下见肿瘤由3种成分以不同比例混合构成:①较成熟脂肪细胞体积小的单泡状和多泡状脂肪母细胞,胞质淡染为主,少数细胞呈嗜酸性,核形多样,无异型性及核分裂象,排列成片状和巢状;②脂肪母细胞间黏液透明性软骨样基质;③多少不等的成熟脂肪细胞。PAS染色见脂肪母细胞的胞质内含许多可被淀粉酶消化的深红染颗粒,提示存在糖原。AB染色见黏液透明性软骨样基质呈阳性反应,并部分耐透明质酸酶消化,提示含有硫酸软骨素。脂肪母细胞和成熟脂肪细胞表达S-100蛋白,其中脂肪母细胞对S-100蛋白的表达与脂肪母细胞分化成熟程度相关。电镜观察可见处于不同发育阶段的脂肪母细胞,脂肪母细胞周围被絮状的软骨样基质围绕。结论软骨样脂肪瘤是一种十分罕见的良性脂肪细胞肿瘤的特殊类型,具有独特的组织学形态,应注意与黏液型脂肪肉瘤和骨外黏液样软骨肉瘤等肿瘤鉴别。     

Renal mucinous tubular and spindle cell carcinoma: report of four cases and literature review

Mucinous tubular and spindle cell carcinoma of the kidney (MTSCC-K) is an unusual renal tumor. It is important to increase the recognition of the clinicopathological features of MTSCC-K and improve its clinical and differential diagnosis. This report described four cases of MTSCC-K with clinical, imaging, and pathological examination and showed that the tumor boundaries of MTSCC-K were clear, and tumor cells arranged into tubules and cord-like beams, between which was lightly stained myxoid stroma. The tumor cells were smaller and cube- or oval-shaped, with single small eosinophilic nucleoli, low-grade nuclei, and little nuclear fission. The myxoid stroma was scattered around lymphocytes and plasma cells. Immunohistochemical markers including CK7, CD117, EMA (epithelial membrane antigen), vimentin, and CK8/18, showed positive expression in tumor cells, but the tumor cells were negative for CD10 and villin. The proliferation index of Ki-67 was 5-10%. Since MTSCC-K is a rare low-grade malignancy, with unique histological and immunohistochemical characteristics, it is important for clinicians and pathologists to have a defined awareness of this tumor type in order to decrease the rate of misdiagnosis.     

胆囊原发性恶性黑色素瘤1例及文献复习

目的探讨胆囊原发性恶性黑色素瘤的I临床病理特征、组织发生及鉴别诊断。方法对1例原发于胆囊的恶性黑色素瘤进行光镜观察,并行组织化学及免疫组化染色标记。结果组织学特征为：在上皮和固有层交界处黑色素肿瘤细胞呈巢状或片状分布,肿瘤细胞大小不等,形态多样。瘤细胞内外可见粗大的黑色素颗粒,Masson—Fontana染色阳性,免疫表型HMB45、vimentin及S-100蛋白肿瘤细胞呈阳性表达。结论胆囊原发性恶性黑色素瘤罕见,诊断必须结合病史、全身检查及随访资料,以排除转移性恶性黑色素瘤。     

Clinicopathological features of primary hepatic diffuse large B-cell lymphoma: a report of seven cases and a literature review

We studied the imaging and histopathological features of primary hepatic diffuse large B-cell lymphoma in order to explore the clinicopathological features, diagnosis, differential diagnoses, and treatment. Immunolabelling was performed in seven cases of primary hepatic diffuse large B-cell lymphoma using histological and immunohistochemical techniques. The clinical manifestations; imaging, histopathological, and immunohistochemical features; treatment; and prognosis of primary hepatic diffuse large B-cell lymphoma were observed and analyzed in light of the relevant literature. The average age of the seven patients was 63.4 years. Moreover, bulge of the upper right abdomen and progressive athrepsia and anemia were observed in all seven patients. Computed tomography (CT) revealed the presence of multiple solid hypodense lesions. Further, CT also revealed an enhanced irregular focus. Histopathological analysis revealed the following characteristics: heavy infiltration composed mainly of medium-sized round cells with a lightly stained cytoplasm, prominent nucleoli and vesicular nuclei, nuclear fission and visible sky star phenomena. The tumor cells showed diffuse expression of CD19, CD20, and CD79a, with the percentage of Ki67-positive cells being 75%-80%. All these findings indicated that primary hepatic diffuse large B-cell lymphoma is rare and generally has a poor prognosis. Biopsy and immunohistochemical staining are helpful in its diagnosis. Further, the differential diagnoses include secondary liver diffuse large B-cell lymphoma, low/undifferentiated cancer of the liver, hepatoblastoma, leukemia of the liver, and other tumors. Early surgery and chemotherapy can have a good curative effect.     

血管肌纤维母细胞瘤2例报道及文献复习

目的 探讨血管肌纤维母细胞瘤(AMF)的临床病理特征及诊断和鉴别诊断。方法 对2例AMF进行组织学观察和免疫组化S—P法标记，抗体为vimentin、desmin、SMA、S—100蛋白、CD34、ER、PR、CK等。结果 1例为30岁女性，表现为外阴囊肿；1例为51岁男性，表现为腹股沟区精索肿块。眼观：肿瘤境界均清楚；镜检：肿瘤均由相互交错分布的细胞密集区和细胞稀疏区组成，其间血管丰富，多为毛细血管至中等大薄壁血管。瘤细胞成巢或束状围绕血管周围排列。可见红细胞漏出。其中1例尚可见成群分布的脂肪细胞存在。免疫表型：瘤细胞vimentin(2／2)、ER(2／2)、PR(2／2)、SMA(1／2)、CD34(2／2)阳性，SMA(1／2)、S—100蛋白(1／2)灶性阳性，desmin、CK阴性。结论 AMF是一种少见的好发于外阴生殖道的间质肿瘤，可能来源于血管周具有多向分化潜能的干细胞，可向肌纤维母细胞分化。在组织形态上AMF应与侵袭性血管黏液瘤、富细胞性血管纤维瘤、浅表性血管黏液瘤、纤维上皮性间质息肉、梭形细胞脂肪瘤相鉴别。