伴有肉瘤样成分的生殖细胞肿瘤

性腺和性腺外生殖细胞肿瘤中存在肉瘤样成分是一种少见的现象，临床后果和生物学意义尚不清楚。作者报道了46例伴有肉瘤样成分的生殖细胞肿瘤（GCTSC），21例位于性腺（睾丸18例、卵巢3例）、纵隔23例和腹膜后2例。男性43例、女性3例，年龄17～74岁，平均27岁。组织学上，91％GCTSC（42例）为成熟或未成熟畸胎瘤，单纯型或混合型，其中25例为单纯型，17例为混合性各种其他类型的生殖细胞肿瘤和畸胎瘤，剩下的为不伴有畸胎瘤样成分的生殖细胞肿瘤。肉瘤样成分包括胚胎性横纹肌肉瘤29例、血管肉瘤6例、平滑肌肉瘤4例、未分化肉瘤3例、[第一段]     

睾丸旁胚胎性横纹肌肉瘤一例

患者男,17岁。发现睾丸旁肿块1年,于2 0 0 3年4月4日入院。肿块起初约花生米大小,无疼痛、肿胀等不适,逐渐增大至鸡蛋大小,未发现其他症状。体检:左侧睾丸正常,附睾尾部可触及一大小约4 5cm×3 0cm×2 5cm实性肿块,质地硬,表面不平整,无压痛,透光实验阴性。B超显示:左附睾尾部实性肿块。临床诊断左侧睾丸旁肿瘤,行睾丸旁肿瘤切除术。术中见左侧睾丸正常,附睾头、体部良好,尾部查见一约4cm×3cm大小肿块,将肿块完整切除。病理检查:结节状肿物一个,体积5 0cm×3 6cm×3 1cm ,表面光滑,已先行切开,切面灰白,有光泽,与周围界限清楚,无明显包…     

心脏原发胚胎性横纹肌肉瘤临床病理学观察

心脏胚胎性横纹肌肉瘤非常罕见,是一种原始的具有胚胎性骨骼肌表型的软组织肉瘤,主要由原始间叶细胞和早期幼稚发育阶段的横纹肌细胞所组成。多发生于婴幼儿,好发于头颈部和泌尿生殖系统。我们报道1例心脏原发胚胎性横纹肌肉瘤并复习相关文献,探讨其临床病理特征、诊断及鉴别诊断。     

16例肝脏未分化性胚胎性肉瘤术前误诊分析

目的:探讨肝脏未分化性胚胎性肉瘤(undifferentiated embryonal sarcoma of the liver,UESL)的临床特点,加深对该病的认识,提高术前诊断准确性.方法:回顾性分析16例经手术病理证实、术前误诊UESL患者的临床、影像及病理资料,并对比分析.结果:16例患者中诊断为原发性肝癌9例,肝母细胞瘤3例,肝内恶性占位4例.12例以腹痛不适就诊;超声显示实性为主肿瘤,而CT及MRI呈囊性为主的肿瘤,瘤内成分混杂;增强后病灶内囊壁下软组织、分隔及边缘可见强化.术后病理见肿瘤切面呈囊实性,内见不同程度出血坏死、凝胶样区域和肿瘤组织,癌细胞间可见特征性嗜酸性小体.结论:未分化胚胎性肉瘤临床罕见,临床资料无明显特异性,超声与CT、MRI表现不一致性为重要鉴别要点,有助于提高术前诊断率.     

小儿耳鼻部胚胎性横纹肌肉瘤2例并文献复习

目的 探讨胚胎性横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)的临床病理特征,提高其诊断及鉴别诊断水平.方法 对2例小儿ERMS进行临床资料及病理形态学观察,结合免疫表型进行分析并复习相关文献.结果 例1男婴,左侧耳道持续性流脓伴发热1周,CT示:左侧外耳道、中耳及乳突内见高密度充填影;例2女婴,发现右侧外鼻部灰白色肿物5个月.2例均行手术切除,镜下例1见异形增生的细胞,胞质红染,呈短梭形、球拍样,伴少量淋巴细胞、单核细胞浸润;例2致密的纤维组织中见灶状分布的异形小圆细胞.免疫表型:2例desmin均阳性,1例Myogenin阳性,Ki-67增殖指数60%～80%.结论 耳鼻部ERMS临床表现缺乏特征性,病理学诊断有困难,当瘤细胞分化差或未分化时可结合免疫表型来鉴别诊断.     

肝脏未分化胚胎性肉瘤一例

患者女，15岁。右上腹疼痛伴右肩背部牵涉痛1个月于2002年8月813入院。CT示肝右叶直径120mm类圆形低密度灶，密度不均，考虑肝右叶恶性肿瘤。遂行肝叶包块切除术。     

横纹肌样分化胃癌1例

正患者男性,68岁,因剑突下阵发性疼痛1个月余,伴恶心呕吐行胃镜检查。结果示胃窦部慢性胃炎,伴有糜烂,贲门-胃底黏膜下隆起;腹部CT示胃底小弯侧旁异常密度影,大小4.3 cm×4.2 cm,待除外胃肠道间质瘤,其周围多个淋巴结略大;实验室检查:血常规中性粒细胞计数略高,其余无异常。患者行全胃切除术+周围淋巴结清扫。患者术后2个月死亡。病理检查眼观:全胃切除标本,胃底小弯侧见一大小5.5 cm×5.5 cm×6.5 cm肿物,向浆膜侧     

具有神经内分泌分化的肺粘液样软骨肉瘤

目的：探讨肺原发性具有神经内分泌分化的粘液样软骨肉瘤的病理特征。方法：通过HE、组化、免疫组化及电镜观察1例肺粘液样软骨肉瘤。结果：肿瘤由粘液样基质和疏网状结构的梭形及圆形细胞、软骨母细胞样细胞和小圆形细胞组成,3种细胞梯度移行,形成典型粘液样软骨肉瘤及类似原始性神经外胚层肿瘤结构。组化染色显示AB（pH2.5),TB(pH4.0)阳性,Grimelius嗜银颗粒阳性。免疫表型：S－100蛋白、vimentin,NSE,Syn阳性,小圆细胞和软骨母细胞样细胞还显示HBA71、CgA、Leu7、EMA阳性。超微结构;瘤细胞胞质分布致密核心颗粒。结论：证实该例为肺原发性具有神经内分泌分化粘液样软骨肉瘤。     

肝脏未分化胚胎性肉瘤1例

患者男性,7岁,发现上腹部肿物6天入院。影像学示左肝部巨大囊实性包块,肝功能正常,血清AFP阴性。临床拟诊为左肝错构瘤,行左半肝切除术。     

去分化软骨肉瘤11例临床病理分析

目的探讨去分化软骨肉瘤的临床、影像学及病理学特点。方法收集11例去分化软骨肉瘤患者的临床及影像资料,对组织进行病理分析。结果患者以男性多见(7/11),平均年龄57.1岁,肿瘤多位于长管状骨及骨盆。影像学显示广泛的骨质破坏,病灶内可有钙化或骨化,病理性骨折3例。眼观:病理软骨成分常位于骨内,去分化肉瘤成分主要位于骨外。镜下包括高分化软骨肉瘤和低分化间叶源性肉瘤两种成分。随访材料中6例死亡,平均存活14个月。结论去分化软骨肉瘤检:呈少见的的软骨肉瘤亚型,去分化类型多样,只有加深对其的认识,才能提高诊断的准确率。     

Intraosseous rhabdomyosarcoma of the mandible: a case report

Rhabdomyosarcoma is the most common soft-tissue sarcoma of the head and neck region in children and adolescents. Oral cavity involvement is relatively uncommon, with tongue, soft palate, hard palate, and buccal mucosa being the sites of predilection. This report presents a rare case of intraosseous oral rhabdomyosarcoma arising in the mandibular bone of a 6-year-old child. Clinical, radiologic, and histopathologic features and possible pathogenesis are discussed.     

Embryonal rhabdomyosarcoma of the chest wall: a case report and review of the literature

Embryonal rhabdomyosarcoma is a soft-tissue sarcoma which has a predilection for the head and neck area, genitourinary tract and the extremities. We report a rare case of embryonal rhabdomyosarcoma of the chest wall in an 8-year-old girl, presenting as a destructive tumor in the rib and clinically and radiologically mimicking Ewing's sarcoma. Histopathological examination showed a small round cell tumor. Immunohistochemically, the positivity for muscle markers desmin and myogenin in the tumor cells proved to be useful for making a definitive diagnosis of embryonal rhabdomyosarcoma. Cytogenetic analysis revealed a high level of aneuploidy in the tumor cells, with double-minutes and additional chromosomal structural aberrations. The patient is responding well to chemotherapy.     

Intraductal tubulopapillary neoplasm of pancreas with stromal osseous and cartilaginous metaplasia; a case report

Recently, a surgically resected case of intraductal tubulopapillary neoplasm (ITPN) with stromal osseous and cartilaginous metaplasia was encountered. A CT scan showed calcification at the tail of the pancreas two years before the operation. In the resected specimen, macroscopically, the main pancreatic duct was dilated and filled with a whitish solid mass without mucinous material. The tumor showed mainly a solid and papillary growth pattern. The tumor cells had no evidence of acinar differentiation. The tumor cells, at the tail of the pancreas, invaded focally to surrounding pancreatic parenchyma with stromal desmoplastic and fibrosclerotic reaction and also formed nodular stromal osseous and cartilaginous metaplasia. The tumor did not invade extrapancreatic tissue and showed no lymph node metastasis. As there were no signs of chronic calcifying pancreatitis, it is hypothesized that the metaplastic stroma was formed by a stromal reaction due to the tumor growth. It is thought, therefore, that the intraducal component of the tumor had existed at least for two years. This case suggests that ITPN is a relatively indolent tumor with a better prognosis than that of other types of invasive ductal adenocarcinoma of the pancreas.     

Embryonal rhabdomyosarcoma (sarcoma botryoides) of the common bile duct: A case report

A case of sarcoma botryoides of the common bile duct is described. A review of the literature reveals 24 reported cases of this entity. The clinical features of the disease and the poor prognosis associated with it are illustrated by a detailed presentation of the case. Although rare, this condition must be included in the differential diagnosis of jaundice in infancy or early childhood. Pathologic findings of diagnostic value are re-emphasized.     

伴骨及软骨化生的肺髓脂肪瘤1例

目的:探讨肺髓脂肪瘤的临床病理特点、诊断、鉴别诊断及肿瘤发生的来源。方法:复习1例肺髓脂肪瘤的临床资料,行组织学和免疫组织化学观察。结果:63岁患者CT强化扫描示左肺主支气管内一类圆形不均匀密度肿物,边缘光整。大体检查:肿瘤2 cm×1 cm×0.5 cm大小,切面灰白或灰黄色,质地不均,部分区域质硬如骨样组织。光镜显示:低倍镜见骨小梁组织环形围绕脂肪和造血样组织,局灶可见软骨组织。高倍镜见粒、红、巨核细胞系,三系细胞形态无异常。免疫组织化学染色显示:髓细胞髓过氧化物酶(+),巨核细胞CD61(+)。结论:肺髓脂肪瘤非常罕见,确诊依赖病理学检查,形态学典型,认识该疾病有助于避免临床影像学的误诊。     

Primary pulmonary rhabdomyosarcoma: a case report

We report a case of primary pulmonary rhabdomyosarcoma in a 52-year-old woman. The diagnosis was established after radical right pneumonectomy, mainly based on immunohistochemistry and electron microscopy findings. This type of tumor is rare in adults. Its primary origin is suggested when a pulmonary meta-stasis of rhabdomyosarcoma from another site or a component of a mixed tumor are ruled out. The best treatment is surgery. The prognosis is poor.     

喉多形性横纹肌肉瘤一例

患者男,32岁。因“声嘶2年,加重半年”于2005年11月6日入院。外院行喉镜活检示“恶性肿瘤”。喉镜示左侧披裂可见2cm×2cm大小实性肿物,遮盖左侧梨状窝,表面光滑。CT示喉咽部左后侧壁肿块影,向腔内突出,咽腔左侧变窄（图1）。术中见肿物位于环状软骨板后上方,似有包膜,表面喉黏膜光滑,肿物向下达环状软骨下缘,向对侧已越过中线,向前未达会厌。行全喉切除术加双侧颈部清扫。[第一段]     

Embryonal botryoid rhabdomyosarcoma of the bladder

A 10-year-old black child presented with bloody urine and urine retention due to a bladder tumor. Cystoprostetatectomy led to the diagnosis of embryonal botryoid rhabdomyosarcoma. At gross examination, tumor presented a grape-shaped form. Microscopically, the specimen presented undifferentiated cells in a myxoid stroma and subepithelial cambium. The tumor was desmin and MyoD positive. The child died before chemotherapy could be completed.