膀胱和前列腺横纹肌肉瘤(附9例报告)

本文报告我院经治本病9例。6例起于膀胱(男女各半);2例起于前列腺;1例晚期既累及膀胱、前列腺,未确定其最初起源。儿童7例(14月～7岁),成人2例(33及35岁各1例)。 病理学上,本病分为四型:1.多形性横纹肌肉瘤,本组1例;2.腺泡状横纹肌肉瘤,本组无此型;3.胚胎性横纹肌肉瘤,本组4例;4.葡萄状肉瘤,本组4     

横纹肌肉瘤的临床病理分析

目的：探讨横纹肌肉瘤的诊断和鉴别诊断中免疫组织化学标记的意义及不同类型横纹肌肉瘤对预后的影响。方法：应用病理形态学和免疫组化方法对４７例横纹肌肉瘤进行研究。结果：４７例横纹肌肉瘤中胚胎性２８例,腺泡状１６例,其中７例伴广泛侵润、复发和转移,多型性３例;结蛋白（ｄｅｓｍｉｎ）和肌球蛋白（ｍｙｏｇｌｏｂｉｎ）的阳性率分别为（９８％）和（８３．２％）。结论 和胚胎性横纹肌肉瘤相比,腺泡状横纹肌肉瘤具有更     

小儿胆道葡萄状肉瘤(附1例及文献复习)

目的：提高对小儿胆道胚胎型横纹肌肉瘤(葡萄状肉瘤)的认识，减少误诊。方法：对本病例及国内文献报道的胆道胚胎型横纹肌肉瘤(葡萄状肉瘤)病例共16例进行分析。结果：男11例，女5例。年龄1～6岁。黄疸16例，发热8例，右上腹痛6例，陶土色大便9例，尿黄6例。肝肿大13例，脾肿大2例，右上腹肿块2例。临床误诊为肝炎5例。B超考虑葡萄状肉瘤仅2例，CT考虑胆总管横纹肌肉瘤仅1例。病检诊断：胚胎型横纹肌肉瘤3例，葡萄状肉瘤13例。7例切除并化疗、放疗，随访最长的8个月未复发。1例部分切除并化疗，3个月后随访残留灶缩小，病情稳定。2例行姑息手术后2个月左右死亡。1例切除后2个月检查已复发。结论：小儿胆道横纹肌肉瘤为胚胎型横纹肌肉瘤(葡萄状肉瘤)，发病年龄1～6岁，男性比女性多见。其主要症状为阻塞性黄疸，常伴有发热、肝肿大等，临床易误诊为肝炎。B超、CT等辅助检查有助于诊断，确诊需经病理检查。本病恶性程度高，预后差，应积极外科治疗并配合化疗、放疗，可延长生存时间。     

横纹肌肉瘤的病理鉴别诊断

本文重点讨论了横纹肌肉瘤的病理鉴别诊断问题。在33例误诊病例中,包括脂肪肉瘤及纤维肉瘤各5例,未分化癌及未分化腺癌4例,恶性纤维组织细胞瘤及神经母细胞瘤各3例,恶性神经鞘瘤各2例,滑膜肉瘤、间皮肉瘤、腺泡状软组织肉瘤、恶性血管内皮瘤、恶性组织细胞瘤、皮肤非典型性纤维黄色瘤、腹壁外韧带状瘤、淋巴肉瘤、网织细胞肉瘤、何杰金氏病、多形性肉瘤等各1例。探讨了容易发生误诊的各种原因。(1)在瘤细胞形态多变,多形性明显,单核及多核瘤巨细胞多见时,易误诊为脂肪肉瘤、纤维肉瘤、恶性纤维组织细胞瘤、恶性神经鞘瘤、皮     

儿童横纹肌肉瘤的病理学研究

目的探讨儿童横纹肌肉瘤的病理形态学特点。方法复习和分析30例儿童横纹肌肉瘤的形态学和免疫组织化学染色结果。结果30例横纹肌肉瘤患儿中,男性22例,女性8例。组织学类型包括胚胎性（20／30,66．67％）、腺泡状（8／30,26．67％）和梭形／硬化性（2／30,6．67％）横纹肌肉瘤3种。发生部位依次为躯干（15／30,50．00％）、头颈（7／30,23．33％）、四肢（6／30,20．00％）和泌尿生殖系统（2／30,6．67％）。免疫组织化学染色示肌特异性肌动蛋白（MSA）、平滑肌肌动蛋白（SMA）、结蛋白（DES）、肌细胞生成素（MyoG）和肌调节蛋白（NyoDl）均有不同程度的阳性表达。结论胚胎性、腺泡状和梭形／硬化性横纹肌肉瘤是儿童横纹肌肉瘤的主要类型,组织学上主要表现为原始的小圆细胞,部分病例可见横纹肌母细胞。肿瘤细胞不同程度表达NSA、SMA、DES、MyoG和MyoDl等肌源性标志物。鉴别诊断主要包括神经母细胞瘤、外周原始神经外胚层肿瘤等。     

鼻咽部横纹肌肉瘤临床病理、免疫组化及超微结构观察

本文报道经病理确诊的鼻咽部横纹肌肉瘤14例,男性6例,女性8例,年龄最小2.5岁,最大48岁,平均12.7岁。组织学类型胚胎性横纹肌肉瘤8例、葡萄状横纹肌肉瘤3例、腺泡状横纹肌肉瘤3例;组织学分级Ⅰ级5例、Ⅱ级4例、Ⅲ级5例。本文着重讨论了掌握横纹肌肉瘤组织形态特点、超微结构诊断标准及免疫组织化学vimentin、desmine、HHF—35、myoglobin表达规律及合理评价在病理诊断与鉴别诊断中的价值及三者相结合的必要,有助于提高确诊率。同时还讨论了影响RMS预后等综合因素。     

电镜在横纹肌肉瘤诊治中的价值

研究电镜对横纹肌肉瘤诊断的价值。方法 对８例光镜下诊断为横纹肌肉瘤及可疑病例，通过电镜观察细胞的超微结构。结果 ８例中，３例为误诊；５例为横纹肌肉瘤，我形性１例，腺泡状１例，胚胎性３例。据此制定治疗方案，取得满意临床疗效。     

小儿横纹肌肉瘤病理学研究——115例病理统计分析

本文统计分析了全国15个单位1955—1985年115例小儿横纹肌肉瘤。年龄最大13岁,最小15天,平均为3.98岁;男性78例,女性37例。按照Horn和Enterlineio分类标准,分为胚胎型(53.04％)、腺泡型(13.91％)、葡萄状型(7.8％)和多形型(3.8％)。38.5％发生在泌尿生殖道,尤其是膀胱。结合文献对小儿横纹肌肉瘤的诊断和发病情况进行了讨论。     

骨原发性腺泡状肉瘤免疫组化和超微结构研究

５例罕见的骨原发性腺泡状肉瘤免疫组化检查显示，波形蛋白，结蛋白和肌红蛋白为阳性，支持本病组织起源与横纹肌有关，电镜检查见瘤细胞内有特殊结晶。研究结果表明，骨原发性腺泡状肉瘤与软组织的腺泡状肉瘤相似。     

环磷酰胺对人腺泡状横纹肌肉瘤细胞裸鼠移植瘤生长和形态结构的影响

目的 检测环磷酰胺(cyclophosphamide,CTX)对人腺泡状横纹肌肉瘤细胞株PLA-802裸鼠移植瘤生长和形态结构的影响.方法 16只裸鼠右腋皮下接种PLA-802移植瘤细胞悬液后分成空白对照组(5只)、生理盐水对照组(5只)和20 mg/kg CTX治疗组(6只).接种后第7天开始,经腹腔注射给药,隔日1次,共注射10次.每2天测量1次肿瘤大小绘制移植瘤生长曲线.接种后第26天杀鼠摘取移植瘤称重,组织经制片染色后用光镜和电镜检查.结果 ①CTX治疗组移植瘤生长被明显抑制(P＜0.05),抑瘤率85.55%.②治疗后瘤细胞疏松排列成腺泡状,由纤维间质分隔,血管稀少;瘤细胞椭圆形、网拍状或短梭形;核椭圆或类圆形,大小不一;核分裂相减少.电镜见瘤细胞质内有不等量肌(微)丝,甚至有横纹肌肌原纤维结构形成.结论 CTX对人腺泡状横纹肌肉瘤PLA-802移植瘤生长具有一定程度的抑制作用,治疗后瘤组织形态结构改变.     

Rhabdomyosarcoma of nose and paranasal sinuses--a therapeutic challenge

Rhabdomyosarcoma is a highly malignant tumour of striated muscle and it is the most common soft tissue sarcoma in the paediatric age group. It is very uncommon in the nose and paranasal sinuses. Pinkish nasal mass and recurrent epistaxis are the most common presenting symptoms. Histologically embryonal rhabdomyosarcoma is the most common variant. The present series deals with seven such cases of different histopathological types including the embryonal, botryoid, and alveolar varieties in the paediatric age group. Multimodal treatment including limited surgery followed by radiotherapy and chemotherapy resulted in survival ranging from 3 days to more than 3 years, which was also influenced by the severity of the disease at presentation and its aggressiveness.     

Botryoid rhabdomyosarcoma of vagina--two case reports

Botryoid rhabdomyosarcoma, previously considered a type of embryonal rhabdomyosarcoma, has clinicopathological features distinctive enough to warrant its classification as a separate entity. It almost always occurs in children under 5 years of age. Two cases of botryoid rhabdomyosarcoma are reported here because of their relative rarity.     

Orbital rhabdomyosarcoma. Immunohistochemical studies of seven cases.

An immunohistochemical analysis using a series of mono- and polyclonal antibodies against myoglobin, desmin, vimentin, actin, NSE, S-100 protein and keratin was done on alveolar rhabdomyosarcoma (3 cases), embryonal (3) and pleomorphic rhabdomyosarcoma of the orbit (1). All tumors were stained by monoclonal antibodies to desmin and vimentin, whereas 6 of the 7 cases were stained by myoglobin. The results indicate that desmin and vimentin are highly sensitive immunohistochemical markers for the diagnosis of rhabdomyosarcoma, especially in poorly differentiated ones.

     

Tissue Factor Activity a marker of alveolar macrophage maturation in rabbits.effects of granulomatous pneumorlitis

<正> Experiments were carried out to examine relationships between alveolarmacrophage maturity and amounts of tissue factor (Clotting Factor Ⅲ)inthese cells under physiologic conditions and during immunologically inducedpneumonitis.Using discontinuous density gradient centrifugation,alveolarmacrophages from healthy rabbits were rapidly isolated into five subpop-ulations at different stages of maturation,as demonstrated by morphologicand morphometric evaluation.Very large amounts of tissue factor activitywere found in fully mature cells that were purified in the lowest densitysubpopulation and assayed without preliminary in vitro stimulation orculture.In the remaining four subpopulations of increasing density,amounts oftissue factor were found to progressively diminish in direct correlation withdeclines of cell maturity.These differences at mean levels were as great as35-fold.In addition,blood monocytes had <1/219 and<1/6 of the activityof the fully mature and the least mature subpopulations,respectively.After16 h culture of the five isolated subpopulations in the absence of lymphok-ines or of significant numbers of lymphocytes,tissue factor activity increa-sed in inverse correlation with the preincubation stage of cell maturity(2,387)and 109% in the least mature and most mature subpopulations,resp-ectively).These increases required protein synthesis and were accompaniedby morphologic and morphometric changes which indicated cellularmaturation during the period of tissue factor activity generation in vitro,thus further demonstrating relationships between macrophage maturity andtissue factor content.In additional experiments,direct correlations between cell maturity and tissue factor activity content were also found in activatedalveolar macrophage populations from rabbits with Bacillus CalmetteGuering (BCG)-induced granulomatous pneumonitis.However,as comparedwith controls,the BCG populations had increased total amounts of tissuefactor activity due to the presence of large numbers of mature alweolarmacrophage forms that had high levels of the procoagulant.Thus,tissuefactor activity in alveolar macrophages is a marker of cellular maturationin vivo and in vitro.Increased amounts of this initiator of the extrinsicclotting pathway,as found in alveolar macrophage populations from animalswith granulomatous pneumonitis induced by BCG hypersensitivity,suggestthat alveolar macrophage tissue factor may contribute to the pathology ofimmune lung diseases.     

Rhabdomyosarcoma of the Breast：A Clinicopathologic Study and Review of the Literature

Background  Rhabdomyosarcoma (RMS) is an uncommon malignancy of the breast. The aim of this study was to summarize its clinicopathologic features and biological behavior.

Methods  Five primary or secondary breast RMSs were collected. Their clinicopathological characteristics and all published literature about breast RMS were reviewed. Immunohistochemical study of desmin, myogenic differentiation 1 (MyoD1), myogenin, leukocyte common antigen (LCA), vimentin, cytokeratin (AE1/AE3), E-cadherin, neuron specific enolase (NSE), CD99, chorioallantoic membrane 5.2 (CAM5.2) and epithelial membrane antigen (EMA) expression were performed.

Results  The five patients were all female with ages ranging from 16 to 46 years old (mean, 30 years). Three were metastatic breast RMSs, two embryonal and one solid variant alveolar, with the primary tumor sites the right labium majus, left nasal meatus and nasopharynx, respectively. The other two, one embryonal and one alveolar, were primaries. Grossly, the surgical specimens revealed round or oval, well-demarcated but nonencapsulated masses. Their cut surfaces consisted of homogeneous grayish yellow or white tissue. Microscopically, most tumor cells were poorly differentiated small round, oval or small polygons with eosinophilic cytoplasm. All cases were positive for vimentin, desmin, MyoD1 and myogenin. One embryonal RMS also had a few cells with perinuclear staining of AE1/AE3. The other markers were negative.

Conclusions  Although primary or metastatic RMS in breast was almost confined to young adolescent females, our cases suggested that it can also happen to the middle-aged women. Embryonal RMS has a certain metastatic potential. MyoD1 and myogenin are two useful markers when making differential diagnosis. Axillary lymph node status and age may play a role in the prognosis of primary breast RMS patients.

     

LACTOSE TOLERANCE TEST BY HYDROGEN BREATH METHOD IN CHINESE

Our digestive disease section performed lactose tolerance tests in t31 normal Chinese using the Hydrogen Breath Microlyzer Type CM made by Quintron Instrument Corp., USA. Subject age rang- ed from 4 t0 58 years. There were 81 males and 50 females, a male to female ratio of l.62:1. 103 (78.63%) of the subjects were lactose intolerant, their pulmonary alveolar air hydrogen being greater than 20 ppm, similar to the percentages found in orientals investigated in the USA. Comparison by age groups showed that Iactose intolerance in adults was 85.85%, lactose intolerance. Lactose intolerance was 4T83% In children it was 47.83%. The alveolar air hydrogen gas peak in children was significantly lower than that of adults. The appearance of the hydrogen gas peak was delayed in all children's age groups. This sug gests that lactose intolerance is probably develop- mental in all with this defect. We consider that H7 breath test is of diagnostic value in many alimen- tary disturbances. It is simple to perform and worth further study.