儿童腺泡状软组织肉瘤13例临床病理学特征

目的探讨儿童腺泡状软组织肉瘤(alveolar soft part sarcoma, ASPS)的临床病理、分子遗传学特点、诊断及鉴别诊断。方法对北京儿童医院2009年8月至2018年11月13例儿童ASPS病例存档切片行HE染色及组织化学染色[包括过碘酸-雪夫(PAS)染色及淀粉酶消化PAS(D-PAS染色)]。采用免疫组织化学染色检测TFE3、INI1、CD68等的表达, 应用荧光原位杂交(FISH)法检测TFE3基因断裂易位情况。结果 13例ASPS中, 男童4例, 女童9例, 年龄1岁2个月至13岁8个月, 平均7.8岁, 5岁以下4例。组织学上, 11例肿瘤细胞呈腺泡状、巢状排列, 2例肿瘤细胞呈实性、弥漫性生长。瘤细胞胞质嗜酸性, 可见明显的空泡现象, 核多形性, 核仁突出, 核分裂象罕见, 3例可见血管浸润。免疫组织化学染色TFE3弥漫核阳性, INI1、CD68、波形蛋白阳性, MyoD1、Myogenin、细胞角蛋白、S-100蛋白等均阴性。7例PAS及D-PAS染色显示肿瘤细胞质内均可见紫红色针状或棒状结晶体。9例行FISH检测, 均显示TFE3基因断裂易位。结论 ...     

儿童和成人腺泡状软组织肉瘤的临床病理学特征

目的 探讨儿童和成人腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理学特征、诊断及鉴别诊断.方法采用免疫组化 EliVision 两步法检测9例 ASPS 中 TFE3、CK、EMA、desmin、SMA、Myogenin、MyoD1、S-100、HMB-45、CgA、Syn...     

腺泡状软组织肉瘤的临床病理分析

目的：观察腺泡状软组织肉瘤的临床及病理形态学特点,并探讨其组织发生方法：对12例腺泡状软组织肉瘤进行光镜观察,并对全部病例做组化及免疫组化染色。结果：12例腺泡状软组织肉瘤,男性4例,女性8例,发病年龄16－38岁（平均24岁）。发生部位主要位于四肢尤其是下肢的深部软组织,与骨髓肌关系密切。镜下瘤细胞呈胞泡状排列,周围为富含血窦的间质,瘤细胞质内含有丰富的嗜酸性颗粒,PAS全部阳性,免疫组化：SMA阳性率75％,desmin阳性率66．6％,myoglobin阳性率25％。结论：支持腺泡状软组织肉瘤的组织发生为横纹肌来源或向横纹肌分化。     

腺泡状软组织肉瘤16例临床病理分析

目的探讨腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理、免疫表型以及分子遗传学特征。方法对16例ASPS进行临床病理学、细胞化学和免疫表型观察,其中2例行FISH检测。结果 16例ASPS中男性6例,女性10例,发病年龄8~58岁(中位年龄31.7岁),临床表现为局部缓慢生长的肿块。肿瘤发生部位包括四肢、肩背部、舌、声带、肺、子宫颈、输尿管。镜下见典型的器官样或腺泡状结构,形成窦隙状血管及纤维间隔,肿瘤胞质内含丰富嗜酸性颗粒。PAS染色显示肿瘤细胞内可有结晶体形成,免疫组化标记肿瘤细胞TFE3阳性,FISH检测肿瘤细胞存在ASPL-TFE3基因融合。结论 ASPS好发于青少年,肿瘤好发部位为四肢,少见部位(舌、声带、子宫颈、输尿管等)发生的ASPS易被误诊为上皮性恶性肿瘤。病理诊断时需与原发或转移性腺癌、副神经节瘤鉴别。肿瘤细胞形成典型的腺泡状结构并且免疫组化标记TFE3和组织蛋白酶K对诊断有意义,诊断时需结合ASPL-TFE3基因融合检测技术。     

儿童和青少年腺泡状软组织肉瘤22例临床病理学特征

目的 探讨儿童和青少年腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理学特征.方法 回顾性分析2012年1月～2020年12月郑州大学第一附属医院收治的22例儿童和青少年ASPS的临床、影像学特征、组织病理学特征和预后情况.结果 22例儿童和青少年ASPS中男性12例,女性1...     

4例腺泡状软组织肉瘤临床病理分析及文献复习

目的：探讨腺泡状软组织肉瘤（alveolar soft-part sarcoma,ASPS）的临床病理学特征及其鉴别诊断。方法：对4例ASPS的临床资料进行回顾性分析,对标本进行组织病理学观察及免疫组织化学（免疫组化）研究。结果：患者3例为男性,年龄分别为30,25,27岁;1例为女性,34岁。发病部位4例均位于下肢深部软组织内。镜下肿瘤细胞排列成腺泡状或实性,细胞巢间可见窦状血管分隔,肿瘤细胞胞质丰富嗜酸,胞质内可见棒状结晶体。免疫组化：4例均TFE3阳性,3例MyoD1胞质阳性。1例患者随访15年后复发伴肺转移死亡,3例患者随访6个月无瘤存活。结论：ASPS是一种罕见的恶性肿瘤,青少年多见,结合临床、病理学特征及免疫组化,可做出正确诊断。鉴别诊断需除外腺泡状横纹肌肉瘤等血窦丰富的肿瘤,TFE3是该肿瘤的特异性标志物。     

纵隔腺泡状软组织肉瘤1例

纵隔腺泡状软组织肉瘤１例关键词纵隔肿瘤，腺泡状软组织肉瘤中国图书分类号Ｒ７３４．５０２李玉军患者；女，２１岁。胸痛、胸闷半年。胸片及ＣＴ发现前纵隔肿物，大小约１０ｃｍ×７，５ｃｍ×６．２ｃｍ，密度较高，边界欠清。临床诊断恶性胸腺瘤，于１９９２年７月６...     

肺原发性腺泡状软组织肉瘤1例

患者女性,48岁,体检发现右下肺包块,无咳嗽和胸痛病史.胸部X线、CT示:右下肺见大小为8 cm×7 cm×5 cm的肿块,边界清楚,考虑肺癌.四肢未见明显包块,无手术史.行肿块切除术,肿块送病理检查.     

腺泡状软组织肉瘤1例

腺泡状软组织肉瘤１例屈传贵张丽△刘艹倩患者女，３０岁。因左大腿肿块复发２个月，于１９９５年４月１８日门诊手术切除。６年前发现局部枣状大小肿块，１年半前在当地予以切除，肿块鸡蛋大小。本次手术见肿瘤位于股四头肌内，中等硬度，与周围组织粘连。术后随访１２个...     

口腔腺泡状软组织肉瘤2例

口腔腺泡状软组织肉瘤２例宋晓俊王三锡例１：患者女性，２６岁。舌根部肿物１月余。初起时为一蚕豆大小的无痛性包块，不影响进食吞咽，在当地医院做肿物切除活检，病理报告“舌根颗粒细胞肌母细胞瘤”。一月后复发，且生长加快，吞咽时微痛。局部检查：左舌根部可见２．...     

21例腺泡状软组织肉瘤的临床病理分析

目的探讨腺泡状软组织肉瘤的临床病理学特征及其鉴别诊断。方法对21例ASPS的临床资料进行回顾性分析,对标本进行组织病理学观察及免疫组织化学研究。结果21例腺泡状软组织肉瘤,男性11例,女性10例,发病年龄4～56岁,平均25.9岁,发病部位主要位于下肢深部软组织内,镜下肿瘤细胞排列成腺泡状或实性,细胞巢之间可见窦状血管分隔,瘤细胞胞质内含丰富的嗜酸性颗粒。PAS染色,瘤细胞胞质内可见棒状结晶体,免疫组化:MyoD110例阳性,desmin4例阳性,S-1009例阳性,NSE11例阳性,Vim11例阳性,AE1/AE3、CK、EMA、SMA、MSA、Syn全部为阴性。结论ASPS是多见于青少年和青年的罕见肿瘤,但多数早期出现血液转移,切除后易复发,最终预后欠佳,结合临床病理学特征及免疫组化,可作出正确诊断。     

Alveolar soft part sarcoma

Summary The type, differentiation and histogenesis of the tumor cells of alveolar soft part sarcoma (ASPS) have been analyzed in a series of ten cases by a light-microscopic, ultrastructural, immunohistochemical and cytologic investigation and quantitative DNA analysis. Four tumors deviated from ordinary ASPS: three were wholly or partly of the so-called pleomorphic variant of ASPS and a fourth tumor showed calcifications of the psammoma body type. The ultrastructural findings and immunohistochemical demonstration of desmin supported the hypothesis of a rhabdomyomatous differentiation and gave no support to epithelial (negative immunoreactions for cytokeratins, epithelial membrane antigen, HMFG-1 and -2, tissue polypeptide antigen (TPA)) or neuroectodermal (negative for S-100 protein, glial fibrillary acidic protein, neurofilaments) differentiation. The negative immunoreactions for vimentin and myoglobin and the positive reaction for neuron specific enolase (NSE) do not exclude a rhabdomyomatous differentiation since in rhabdomyosarcomas the undifferentiated rhabdomyoblasts generally contain vimentin and the differentiated tumor cells contain myoglobin and rhabdomyosarcoma has previously been reported as being positive for NSE. The production of external lamina material peripherally in the tumor cell nests and around vessels in the vascular septa was demonstrated both ultrastructurally and by immunohistochemistry using antibodies against collagen IV and laminin. The cytologic appearance in smears obtained by fine-needle aspiration from a case of the pleomorphic variant showed some resemblance to that of a carcinoma. The seven tumors with an ordinary cell appearance were found to show a diploid DNA-distribution at a quantitative analysis performed on paraffin sections, while the three tumors wholly or partly of the pleomorphic type showed an additional tetraploid peak.     

Alveolar soft part sarcoma: immunological evidence of rhabdomyoblastic differentiation

Two cases of alveolar soft part sarcoma have been studied immunocytochemically using antisera against epithelial membrane antigen, lysozyme, keratins, S-100 protein, desmin, vimentin, fetal myosin, slow myosin, alpha-skeletal muscle actin, alpha-smooth muscle actin and myoglobin. The neoplastic cells were negative with all antisera employed with the exception of the alpha-skeletal muscle actin antiserum which stained the cytoplasm of numerous neoplastic elements, including the crystalloid rods, typical cytoplasmic inclusions of these tumours. It is suggested that the presence of this protein indicates rhabdomyoblastic differentiation of these tumours.     

Alveolar soft part sarcoma

Summary The many different theories on the histogenesis of alveolar soft part sarcoma (ASPS) have caused great confusion. Owing to the recent rapid advance in immunohistochemical studies, two major hypotheses have been proposed. One group of researchers supports the idea that ASPS shows myogenic differentiation, while the other group opposes the idea. This confrontation is essentially one between a group that believes in the immunohistochemically demonstrated presence of desmin in ASPS and a group that denies it. In the present study we detected desmin in 6 of 10 formalin-fixed paraffin sections (although there were differences due to the use of five commercially available types of anti-desmin antibodies). When acetone-fixed paraffin sections and periodate-lysin-paraformaldehyde (PLP)-fixed frozen sections were used in one and three cases, respectively, they were found to be desmin positive, regardless of the type of antibody. The consistent positivity for all anti-desmin antibodies in the cases treated with acetone or PLP is very suggestive of a myogenous origin of ASPS. It is important to take into consideration the fact that formalin-fixed paraffin sections are not very suitable for immunohistochemical study of desmin.     

滤泡树突状细胞肉瘤的临床病理观察

目的 探讨和分析滤泡树突状细胞肉瘤(FDCS)的临床病理特点及鉴别诊断.方法 应用组织学、免疫组织化学(EnVision法)标记及EBER原位杂交,对8例FDCS进行临床和组织病理学分析,并复习相关文献.结果 8例FDCS中男性5例,女性3例,平均年龄50岁.发生部位淋巴结4例,扁桃体、鼻咽部、肝、脾各1例.组织学:瘤组织呈席纹状、束状、弥漫性、旋涡状或结节状,肿瘤细胞呈合体样,境界不清,胞质较丰富,均质嗜伊红或细颗粒状,核呈卵圆形、短梭形或圆形,染色质稀疏或呈空泡状、点彩状,核仁明显,核分裂象多少不等,肿瘤细胞间见有散在淋巴细胞混杂.间质内可见假血管腔及血管周围淋巴鞘现象.其中肝脏1例以大量小淋巴细胞弥漫分布为背景,梭形或卵圆形的瘤细胞散在分布其中,瘤细胞核染色质细腻,部分区域细胞有轻度异形,核不规则、空泡状,有核仁.免疫组织化学瘤细胞均表达CD21、CD35、clusterin,部分表达CD68、上皮细胞膜抗原、S-100及内皮生长因子受体,Ki-67不同程度表达.EBER两例表达.结论 FDCS是一种非常少见的恶性肿瘤,易复发和转移,明确诊断需要结合病理形态学和免疫表型.     

Alveolar soft part sarcoma of the uterine cervix

A rare case of an alveolar soft part sarcoma of the uterine cervix in an 8 year old girl is presented. The patient was admitted because of genital bleeding lasting for 7 months. A polypoid tumor, 2times1.5 cm in diameter, was found in her external uterine os and was surgically resected. Microscopically, the tumor consisted of a uniform sheet of tumor cells in the cytoplasm which contained granules and which were stained with periodic acid-Schiff, both before and after the diastase digestion. Alveolar arrangement of the tumor cells was manifested with reticulin silver impregnation. Dense, membrane bound granules were evident at an ultrastructural level in the cytoplasm of the tumor cells. An immunohistochemical examination demonstrated a positive reaction for anti-desmin, anti-myoglobin, anti-HHF35 and anti-neuron specific enolase in the cytoplasm.     

Alveolar soft part sarcoma presenting as a breast mass in a 13‐year‐old female

Adolescent patients with breast lesions represent a unique population of patients whose differential diagnoses differ from adults. We report a clinically unsuspected case of alveolar soft part sarcoma (ASPS) presenting in the breast of a 13‐year‐old female. ASPS is a rare neoplasm that is usually present in the head, neck, or lower extremities. This rare case presentation gives us the opportunity to review the differential diagnosis of adolescent breast lesions. We also will review diagnostic features of ASPS on fine‐needle aspiration. These are relatively rare disease processes about which many cytopathology professionals may be unfamiliar. Diagn. Cytopathol. 2009. © 2008 Wiley‐Liss, Inc.