耳鼻喉粘液表皮样癌15例临床病理分析

目的:探讨耳鼻喉粘液表皮样癌的临床病理特征,并探讨其诊断和鉴别诊断。方法:收集15例粘液表皮样癌,观察其临床病理特点并进行免疫组化分析。结果:15例均为男性,平均年龄55岁。其中高分化者14例,低分化者1例。肿瘤细胞CK、CEA、EMA、CK8均呈阳性表达。结论:粘液表皮样癌是一种较少见的耳鼻喉肿瘤,根据其组织学改变和免疫组化染色结果,可与其他肿瘤鉴别,掌握组织学分类有助于临床的正确治疗和预后判断。     

Immunohistochemical and electron microscopic assessment of childhood rhabdomyosarcoma. Increased frequency of diagnosis over routine histologic methods

Histologic examination was carried out in 65 cases of childhood rhabdomyosarcoma (RMS), 53 embryonal, and 12 alveolar. Cross-striations were seen on light microscopy in 12 (23%) embryonal and 4 (33%) alveolar tumors. The capacity of immunohistochemical staining (PAP technique) to increase diagnostic accuracy was assessed, using antibodies against myoglobin, the MM isoenzyme of creatine kinase, desmin, calcium magnesium-dependent ATPase of sarcoplasmic reticulum and calsequestrin. Myoglobin was detected in 16 (30%) embryonal and eight (67%) alveolar RMS, higher numbers than obtained by viewing cross-striations on light microscopy. The creatine kinase antibody was slightly better than the antibody to myoglobin and 15 of 25 (60%) embryonal RMS were positive when both specificities were used. The remaining three antibodies were less useful. Of 13 (two alveolar and 11 embryonal) RMS studied by electron microscopy, four showed cross-striations, contained late myoblasts, and were positive for myoglobin. Three additional cases showed only late myoblasts and one of these was positive for myoglobin. Thus, 16 of 25 (64%) of the embryonal and seven of nine (78%) of the alveolar RMS showed either positive immunostaining or ultrastructural features of RMS. This study indicates that a combination of immunohistochemical staining, using antimyoglobin and anticreatine kinase (MM isoenzyme) antibodies, and electron microscopy are useful markers in the diagnosis of childhood RMS.     

梭形细胞横纹肌肉瘤的临床病理特点

目的 研究梭形细胞横纹肌肉瘤(RMS)的临床病理特点及鉴别诊断.方法 回顾性分析8例梭形细胞RMS患者的临床病理资料,对其临床表现、预后、组织形态和免疫组化结果进行分析.结果 梭形细胞RMS可见于儿童和成年人,常发生于头颈部、宫颈和睾丸旁,多表现为无痛性肿块,其预后较差.梭形细胞RMS中瘤细胞多排列成束状、席纹状,其间常混杂着一些肥胖、短梭形或多角形、胞浆红染、核深染偏位的横纹肌母细胞.免疫组化结果为波形蛋白(vimentin)、肌调节蛋白(MyoD1)、结蛋白(desmin)、肌动蛋白(actin)和肌红蛋白(myoglobin)抗原阳性,S-100蛋白、甲胎蛋白(AFP)、胎盘碱性磷酸酶(PLAP)、角蛋白(CK)和c-kit蛋白(CD117)抗原阴性.结论 梭形细胞RMS是一种少见的RMS,预后较差,肌源性标记(desmin)与MyoD1和myogenin的联合应用,有助于RMS的诊断及鉴别诊断.     

孤立性纤维性肿瘤6例临床病理分析

目的探讨孤立性纤维性肿瘤（SFT）的临床及病理形态特征及免疫组化特点。方法结合文献对6例孤立性纤维性肿瘤的组织学、免疫组化特点进行分析。结果患者年龄37～65岁,平均50.1岁,病程2个月～2年,临床表现主要为局部肿块及压迫症状,肿瘤直径为3.0～5.0cm。免疫组化结果：CD34阳性6例,bcl-2阳性6例,Vim阳性6例,CD99阳性5例,desmin阳性2例,FVIII均阴性。结论SFT的临床诊断应结合临床特点、组织学形态、免疫组化特点综合考虑,其组织学构像并不能准确地判断其预后,患者术后的定期随访是必要的。     

Metastases to the breast: differential diagnosis from primary breast carcinoma

Eight patients with breast metastases from primary tumors other than breast carcinoma were studied: 3 malignant melanomas, 2 rhabdomyosarcomas, 1 malignant mesothelioma, 1 appendiceal carcinoid, and 1 epidermoid cervical carcinoma. All had mammographic, histopathologic, and immunohistochemical examinations. The main problem was differential diagnosis from primary breast carcinoma. History of extramammary primary tumor was helpful but breast metastasis was the first clinical feature in 2 cases. Patients had noticed palpable, round, rapid growth masses which were mammographically benign. Pathologic diagnosis was difficult and immunohistochemical studies necessary, whenever the proliferation had histologic features of primary breast carcinoma or when no primary tumor was known. However, some histologic features were of value for diagnosis of metastasis: atypical histologic features for a primary breast carcinoma, a well-circumscribed tumor with multiple satellite foci, the absence of an intraductal component, and the presence of many lymphatic emboli. In adults, the most frequent types of tumors metastasizing in the breast are malignant melanoma and neuroendocrine-like tumors, especially small cell carcinoma and carcinoid. In children, rhabdomyosarcoma is the most common. Accurate diagnosis of breast metastasis is important to avoid unnecessary mastectomy and to implement an appropriate systemic therapy.     

Pleuropulmonary blastoma in an adult: an initial case report.

BACKGROUND: Pleuropulmonary blastoma (PPB) is a unique dysontogenetic neoplasm of childhood. Its primitive, sarcomatous features are analogous to those of other dysembryonic or dysontogenetic tumors, such as Wilms tumor, hepatoblastoma, neuroblastoma, and embryonal rhabdomyosarcoma. PPB typically presents in young children, most younger than 5 years, as a pulmonary and/or pleural-based tumor with cystic, solid, or combined cystic and solid features. These neoplasms are characterized histologically by primitive mesenchymal or a mixture of primitive and sarcomatous components and generally have an unfavorable clinical outcome: death occurs within 1-2 years after diagnosis. METHODS: Clinicopathologic and radiographic findings of a man age 36 years with a cystic and solid mass in the left hemithorax were reviewed and compared with previously studied cases of PPB. RESULTS: Pathologic examination of the mass revealed a cystic and solid neoplasm composed of malignant mesenchymal cells that were immunoreactive for vimentin and muscle specific actin and focally for desmin. The architectural and cytologic appearances as well as the immunohistochemical profile were those of type II PPB. CONCLUSIONS: To the authors' knowledge, all previously reported cases of PPB occurred in children age 12 years or younger. They believe that this case represents the first occurrence of PPB in an adult and documents the finding that, although it is uncommon, adults can develop primitive neoplasms that are usually associated with the pediatric population. In addition, the clinicopathologic features observed in the authors' adult patient were consistent with their experience with this tumor type in children. The patient died less than 1 year after diagnosis.     

Myoglobin: an evaluation of its role as a marker of rhabdomyosarcomas

Tumour markers now have an established role in tumour diagnosis and patient management. However, antibodies used to detect these tumour markers have in some instances proved unreliable, with a low rate of sensitivity and specificity. In this study we wished to evaluate the role of a commercial antibody to myoglobin as a marker of rhabdomyosarcomas. The purpose of this investigation was to assess the sensitivity and specificity of myoglobin antiserum as a marker of rhabdomyosarcomas. This was performed by reacting a large number of tumours (sarcomas, carcinomas and melanomas) with a polyclonal anti human myoglobin antiserum. Staining was demonstrated in 60% of rhabdomyosarcomas. Only two tumours from a total of 226 non-skeletal muscle tumours showed a positive reaction (0.88%). One was a leiomyosarcoma and the other had been classified as an undifferentiated sarcoma but a rhabdomyosarcoma was included in its differential diagnosis. It is of interest that both had been earlier irradiated. This antiserum was therefore a specific but not a very sensitive tumor marker. Its rate of staining of rhabdomyosarcomas is compared with the results in the literature. A great disparity is found and the reasons for this are discussed.     

新疆儿童胚胎性横纹肌肉瘤的临床病理研究

目的 探讨新疆儿童胚胎性横纹肌肉瘤（ERMS）的临床病理及免疫组化特点。方法回顾性分析新疆维吾尔自治区人民医院病理科2008年1月至2014年12月存档的20例ERMS患儿的临床病理资料、组织学特点及免疫学表型。结果 20例ERMS中男性11例,女性9例,发病年龄10个月～18岁,平均年龄6.4岁,85.0％的患儿在10岁前发病;以少数名族为主,其中维吾尔族12例,汉族5例,其他民族3例;原发部位包括头颈部10例、四肢3例、泌尿生殖系统3例、躯干2例、肺部及盆腔各1例,临床表现因发病部位不同而各异。20例ERMS中有1例为葡萄状亚型,肿瘤组织Vimentin、Desmin、MyoD1、Myogenin及CD99的阳性率分别为100.0％、90.0％、55.0％、90.0％和50.0％。结论 ERMS好发于10岁以前的儿童,男性多见,多发生于头颈部位,临床表现因发病部位不同而各异,少数民族明显高于汉族,组织形态学及免疫组化对诊断ERMS有重要参考价值。     

36例儿童横纹肌肉瘤疗效分析

目的:探讨儿童横纹肌肉瘤的临床特点、治疗方法及疗效。方法:分析我院2011年6月至2017年6月收治的36例横纹肌肉瘤患儿的临床资料，总结临床特点，评判疗效及预后。结果:36例患儿中男20例，女16例，中位年龄3.5岁；原发于头颈部16例，躯干13例，泌尿生殖系统4例，四肢3例；其中胚胎型30例，腺泡型5例，多型形1例。IRS临床分期Ⅰ期1例，Ⅱ期11例、Ⅲ期16例、Ⅳ期8例，其中低危1例，中危23例，高危12例。3例接受手术+放疗，15例接受手术+化疗，18例接受以手术、化疗及放疗为主的综合治疗；总病例中CR 17例，PR 6例，SD 5例，PD 8例，CR率47.2％，3年OS率为63.9%。结论:儿童横纹肌肉瘤好发于头颈部及躯干，病理类型以胚胎型为主，手术、化疗联合放疗为主的综合规范治疗方案是提高儿童横纹肌肉瘤疗效的重要途径。     

新疆儿童肾母细胞瘤的临床病理研究

目的 探讨儿童肾母细胞瘤的临床病理特征、免疫表型及鉴别诊断.方法 回顾性分析22例儿童肾母细胞瘤(包含3例会诊病例)的临床病理资料,观察肿瘤的组织形态学及免疫表型特点.结果 22例儿童肾母细胞瘤年龄6个月~11岁,≤5岁者共19例(86%),男/女比例1:1.75,维族16例,汉族6例,维族明显高于汉族.肿瘤最大径4~17 cm(平均9.9 cm).镜下:肿瘤主要由3种成分构成:未分化的胚芽组织、间叶成分和上皮成分,以混合型最多.免疫组化:AE1/AE3,Vimentin,WT1阳性率分别为73.7%,84.2%,89.5%;Ki-67值为10%~90%(平均57.4%).肿瘤伴横纹肌及神经内分泌分化时Desmin(26.3%),NSE(52.6%)阳性.结论 肾母细胞瘤是儿童常见的肾原发性恶性肿瘤,肿瘤呈三相分化,免疫组化表达AE1/AE3、Vimentin、WT1、Desmin和NSE,有助于诊断和鉴别诊断.