肺促纤维增生性小圆细胞肿瘤1例并文献复习

促纤维增生性小圆细胞肿瘤(desmoplatic small round cell tumor,DSRCT)是一种恶性程度较高的肿瘤,多见于腹腔及盆腔,其它一些部位如胸膜、睾丸、颅内、肝、卵巢等亦有报道[1 ].1989年,GERALD和ROSAIL报道了第一例发生于腹腔内的促纤维组织增生性小细胞肿瘤,并于1991 年...     

促纤维增生性小圆细胞肿瘤1例报道及文献复习

目的：探讨促纤维增生性小圆细胞肿瘤的诊断及鉴别诊断。方法：通过HE、免疫组化染色观察1例腹腔内促纤维增生性小圆细胞肿瘤,并复习文献。结果：肿瘤组织由小圆形瘤细胞巢及周围硬化性纤维间质所组成。免疫组化染色显示有上皮性、间叶性及神经性标记物共同表达。结论：促纤维增生性小圆细胞肿瘤是一种特殊的高度恶性肿瘤。     

促纤维增生性小圆细胞肿瘤一例

患者女,50岁,腹部包块、腹胀伴渐进性黄疸6个月。患者既往曾患肺结核、腹腔及肠系膜淋巴结结核。体格检查:体温36.8℃,全身中度黄染。全腹触及巨大包块,质硬,边界不清。白细胞7.84×109,淋巴细胞15%,中性粒细胞79.4%,血红蛋白101g/L,血沉65mm/10min。丙氨酸氨基转移酶(ALT)72.3U/L,总胆红素50mmol/L,直接胆红素47.5mmol/L,碱性磷酸酶(ALP)659U/L,r谷氨酰转肽酶(r GT)623.1U/L,甲胎蛋白(AFP)、癌胚抗原(CEA)、CA125、CA153、CA199均为阴性。B超及放射影像学检查提示:腹腔巨大包块,腹盆腔积液,左胸腔积液。腹腔穿刺细胞学检查诊断…     

腹腔促纤维组织增生性小圆细胞肿瘤1例并文献复习

腹腔促纤维组织增生性小圆细胞肿瘤(Intraab-dominal desmoplastic small round cell tumor,IAD-SRCT)是一种近年来才有报道的极少见、高度恶性肿瘤。自Gerald1991年报道第1例以来至今全世界报道仅130余例[1],国内仅有个例报道[2-5]。本文将报道我院首例IADSRCT,并对国内外相关     

促纤维增生性小圆细胞肿瘤一例

患儿女 ,6岁。腹痛 ,Ｂ超发现上腹部肿块 1周 ,于 2 0 0 0年 8月入院。术中见腹腔巨大肿块 ,约 16ｃｍ× 14ｃｍ× 10ｃｍ大小 ,肿瘤与横结肠系膜、胰腺、大网膜及十二指肠粘连 ,切除肿块、大网膜及部分肠管。病理检查 :肿物 15ｃｍ× 13ｃｍ× 10ｃｍ大小 ,表面结节状 ,包膜不完整 ,切面灰黄色 ,质地不均 ,未见出血、坏死。镜下观察 :肿瘤细胞呈小圆形 ,胞质少 ,核深染 ,核分裂易见 ,细胞排列呈大小、形状不一的巢团状 ,其间有丰富的纤维间质。肿瘤细胞团境界清楚 ,巢团周边细胞可见栅栏样排列及小管状结构 ,并有菊形团形成 ,部分较大细胞…     

横结肠促纤维组织增生性小圆细胞肿瘤1例

促纤维组织增生性小圆细胞肿瘤(Desmoplastic small round cell tumor,DSRCT)是一种近年来鲜有报道的极少见的高度恶性肿瘤。自1991年由Gerald报道第1例以来迄今为止全球报道仅数百例^[1],国内也仅见个例报道。本文将报道我院首例DSRCT,以期与临床医师分享对此病的认识。     

促纤维组织增生性小圆细胞肿瘤三例并文献复习

目的 探讨促纤维组织增生性小圆细胞肿瘤(DSRCT)的临床特点、诊断及化疗方法.方法 回顾性分析3例晚期DSRCT患者的临床资料,并复习相关文献.结果 3例患者均为男性,年龄分别为15、20和25岁,均经病理结合免疫组织化学检查确诊.患者均无法手术,采用伊立替康联合顺铂(IP)方案全身化疗,2例达部分缓解,1例疾病稳定,症状均明显改善,不良反应可耐受.结论 DSRCT临床罕见,是常见于青少年、起病隐匿及预后极差的腹腔恶性肿瘤,临床上只能通过病理检查明确诊断.DSRCT患者使用IP方案治疗有效,耐受性好,可作为有效治疗选择之一.     

促纤维增生性小圆细胞肿瘤的诊断和治疗

摘 要：促纤维增生性小圆细胞肿瘤（desmoplastic small round cell tumor,DSRCT）是一种极罕见的、高度恶性软组织肉瘤,它起源未定,好发于青少年男性的盆腹腔。临床表现无特异性,诊断依赖于临床病理特征、免疫表型和遗传分子学改变,特征性的t（11,22）（p13;q12）染色体异位产生EWS-WT1融合基因,可通过RT-PCR、FISH、Southern blot技术检测这一融合基因来确诊。该疾病预后极差,中位生存期17~25个月。DSRCT最优的治疗目前尚无共识,最大限度地减瘤手术、化疗、放疗、腹腔热灌注化疗等多学科综合治疗虽在一定程度上延长生存期,但总体疗效欠佳。靶向治疗、免疫治疗等新兴治疗措施尚需进一步研究探索。     

促纤维增生性小圆细胞肿瘤的诊断和治疗

促纤维增生性小圆细胞肿瘤(Desmoplastic small round cell tumor，DSRCT)是一种少见的、恶性程度极高的软组织肿瘤类型，由Geraid等于1991年首次报道。该病具有鲜明的临床及病理组织学特点，但是由于报道比较少，还不为广大临床医生所熟悉，容易漏诊和误诊。我们曾收治多例本病患者，希望通过本课题的综述，向大家介绍该病诊治的现状和发展趋势。     

促结缔组织增生性小圆细胞肿瘤1例

促结缔组织增生性小圆细胞肿瘤国外有较多报道^{[1, 2]}, 国内见少数报道^[3]。     

肝脏促结缔组织增生性小圆细胞肿瘤(1例报告并复习文献)

目的　探讨促结缔组织增生性小圆细胞肿瘤 (DSRCT)的临床特点、诊断及治疗。方法　回顾分析 1例原发于肝脏的促结缔组织增生性小圆细胞肿瘤的诊治经过及近期随访 ,并结合复习文献。结果　本例病理获正确诊断 ,肿瘤原发部位及所浸润器官联合切除并辅以化疗 ,随访 12个月效果良好。结论　充分认识和掌握 DSRCT的特点是临床诊断的关键 ;彻底切除肿瘤并辅以化疗可获良好效果。     

Paratesticular desmoplastic small round cell tumor: case report and review of the literature.

Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm mainly affecting young males and typically located in the abdomen. Prognosis is generally very poor. We report a rare case of paratesticular DSRCT in a 17-year-old boy, presenting with an isolated left scrotal mass. The patient had an excellent outcome after complete surgical resection of the tumor and adjuvant multi-agent chemotherapy. DSRCT should be included in the differential diagnosis of small round cell tumors of the paratesticular region in adolescents and young adults. Tumor resection and chemotherapy may be beneficial for these patients. Our experience and a review of the literature suggest that DSRCT located in the paratesticular region may have a better prognosis than its more frequent abdominal counterpart.     

Desmoplastic small round cell tumour successfully treated with caffeine-assisted chemotherapy: a case report and review of the literature

Desmoplastic small round cell tumour (DSRCT) is a rare tumour, usually arising in the abdominal cavity. DSRCT remains an aggressive malignancy, with a poor prognosis despite multi-modality treatments. In the published literature, there has been no patient who lived for three years or more without surgical excision. This report describes a case of DSRCT arising from the brachial plexus and successfully treated with caffeine-assisted chemotherapy. A 29-year-old male presented with pain and numbness in his left forearm. Radiological findings were suggestive of malignant tumour. Histology, immunohistochemical stain and fluorescence in situ hybridisation (FISH) results confirmed the diagnosis of DSRCT. He underwent caffeine-potentiated chemotherapy and the tumour disappeared. The tumour was not removed surgically as it was intertwined in the brachial plexus. Four years after the initial diagnosis, no local relapse and no distant metastases have been observed. Therefore, it is concluded that caffeine-assisted chemotherapy should be one of the treatment options for DSRCT.     

Intra-abdominal desmoplastic small round cell tumor in childhood: case report and review of the literature

Desmoplastic small round cell tumor (DSRCT) correspond to a recent clinicopathological entity, individualized in 1989 into the group of tumors with small round cells. This pathology puts ethiopathogenic, diagnostic, therapeutic and prognosis problems. Indeed, the ethiopathogenic is still unknown, diagnosis is asserted only by immuno-histochimic and cytogenetic study because of the big number of differential diagnoses and the anatomopathologic polymorphism. Its treatment is not well codified and its outcome remains dark in spite of therapeutic progress. The objective of this work is to report a personal observation of a DSRCT and to proceed to a review of the literature to clarify the epidemiological, clinical, paraclinical and therapeutic aspects of this rare tumor.     

良性多囊性腹膜间皮瘤1例及文献复习

目的:探讨良性多囊性腹膜间皮瘤（benign multicystic peritoneal mesothelioma,BMPM）临床病理特征、诊断及治疗,提高对该病的认识。方法:回顾性分析我院2019年1月收治的1例BMPM患者的临床资料并复习相关文献。结果:该例患者女性,48岁,主因“反复右下腹疼痛5个月”入院,在全麻下行腹腔镜检查+中转开腹:肿瘤细胞减灭术+腹腔热灌注化疗（cytoreducrive surgery+hyperthermic intraperitoneal chemotherapy,CRS+HIPEC）。术后病理报告示:良性囊性腹膜间皮瘤,免疫组化结果:Calretinin（+）,MC（+）,WT-1（+）,CK（+）,Vimentin（+）,CK5/6（+）,CEA（-）,CK7（+）,CK20（-）,PAX-8（+）,ER（-）,PR（-）。结论:BMPM临床发病率较低,无典型临床表现且缺乏特异性检查,术前诊断较困难,确诊依靠术后病理及免疫组化结果。CRS+HIPEC是目前首选有效的治疗方法,预后良好,复发率较高但恶变率低。     

胰腺恶性孤立性纤维性肿瘤1例并文献复习

目的:讨论孤立性纤维性肿瘤（solitary fibrous tumor,SFT）的临床特征、病理学表现、影像学表现、诊断方法、鉴别诊断、治疗及预后,以提高临床医生对此病的认识及诊治水平。方法:报道1例术后肿瘤2次复发的胰腺恶性SFT的临床病理资料并文献复习。结果:患者女性,59岁,以“体检发现胰尾部肿物10天”为主诉首次入院,入院后诊断为胰体部占位,行胰体尾切除、脾切除术,术后石蜡病理提示胰体尾交界性梭形细胞肿瘤,考虑孤立性纤维性肿瘤。术后5月余复查,肿瘤复发于左上腹网膜囊内,再次手术切除复发肿物,术后石蜡病理提示交界性或低度恶性孤立性纤维性肿瘤。距二次术后4月余复查,肿瘤再次复发于左上腹网膜囊内,再次予手术切除肿物,术后石蜡病理提示交界性或低度恶性孤立性纤维性肿瘤。结论:胰腺恶性SFT病例极其罕见,术后复发者更是少之又少,确诊主要依靠病理组织形态学及免疫组化标记,并与其他相关性疾病加以鉴别,治疗方法以手术完全切除肿物为主,注意定期复查及远期随访。