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内脏血管内皮肉瘤是罕见结构,占内脏肿瘤不到1%,原发于脾脏者国内仅见一例报告。临床主要表现为左上腹痛,脾肿大,贫血等非特异性症状。病灶有多发性和多脏器的特点,可复发及转移,但病程于良性,平均生存期49个月,5年存活率为30%。10年存活率为5%。手术前常不能确诊。组织病理学一般可分为分化程度较高型和未分化型。肿瘤细胞释放相关Ⅷ因子是其生物学特征,所以相关Ⅷ因子抗原反应(FⅧ-RAG)及荆豆凝集素有诊断及鉴别诊断价值。电镜及影像学检查可提高确诊率。主要治疗是手术切除,而单纯放疗和化疗效果较差。 相似文献
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血管肉瘤(angiosarcoma)也称恶性血管内皮瘤,是由血管内皮细胞或向血管内皮细胞方向分化的间叶细胞发生的恶性软组织肿瘤,占所有软组织肉瘤的1%~2%[1]。血管肉瘤可累及全身多个器官,通常发生在皮肤、深层软组织[2],也可累及头颈部、乳房、脾脏和肝脏、肺部和胸膜等部位[3-5]。原发性胸膜血管肉瘤非常罕见,目前相关文献病例数报道较少,且部分患者资料不全。原发性胸膜血管肉瘤的主要症状为呼吸困难、胸闷及胸痛[6],但其临床表现缺乏特异性,容易导致漏诊、误诊。本文就胸膜血管肉瘤的特点及目前对该疾病的认识进行分析和总结。 相似文献
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目的 分析11例上皮样肉瘤(ES)患者病灶病理检查结果.方法 11例上皮样肉瘤患者,采用肉眼观察及HE染色、免疫组化染色镜下观察手术切除的病灶.结果 5例为经典型上皮样肉瘤(CES),6例为近端型上皮样肉瘤(PES).肉眼观ES组织呈不规则的结节状病变,部分表面溃疡,切面灰白灰黄色,实性,质中,偶可见黏液变.光镜下见结节状或弥漫性分布的瘤细胞,呈上皮样和梭形细胞形态,细胞核大,核深染,部分核空泡状,可见核仁及核分裂象,胞质嗜酸性,常伴有坏死及横纹肌样细胞.免疫组化染色肿瘤细胞均表达Vimentin、EMA和CKpan,偶可见CK19、CD34和GATA3表达,均存在INI1/SMARCB1表达缺失,Ki-67增殖指数5%~80%.肿瘤细胞不表达S-100、desmin、SMA、Myogenin、MyoD1、DOG-1、CD31、CR、HMB-45、CD117.所有患者均行肿瘤扩大切除术,部分患者叠加化疗及放疗.9例患者获得随访,2例失访.6例出现复发或转移,3例患者死亡.结论 ES肉眼观以灰白色,实性,质中结节为主;光镜下结节状或弥漫性分布的上皮样细胞为诊断此病的重要线索,部分细胞核变大、空泡状染色质,出现坏死及横纹肌样细胞等;肿瘤细胞表达Vimentin、EMA、CKpan,Ki-67一般较高,INI1/SMARCB1表达缺失. 相似文献
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原发性心脏血管肉瘤(primary cardiac angio-sarcomas,PCAS)极为罕见,主要特征为高度侵袭性、早期转移以及预后不良.该病的临床表现缺乏特异性,早期诊断较为困难,影像学检查如超声、CT、MRI等对疾病诊断至关重要.本文报道1例PCAS并结合文献对其进行影像学诊断分析. 相似文献
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目的探讨胃肠道间质瘤(GIST)的临床病理以及免疫组织化学特征。方法回顾性分析72例GIST患者的临床资料。结果 72例GIST患者的肿瘤部位为:25例(34.7%)胃部,24例(33.3%)小肠,23例(31.9%)其他部位。镜下显示主型为梭形细胞68例(94.4%),主型为上皮样细胞1例(1.4%),混合细胞型3例(4.2%)。免疫组化检查显示,71例(98.6%)CD117阳性,52例(72.2%)CD34阳性,22例(30.6%)SMA阳性,1例(1.4%)Desmin阳性,6例(8.3%)S-100阳性。结论 GIST的发病部位较多,组织形态学表现复杂,联合应用免疫组化检查有助于临床诊断与鉴别。 相似文献
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Rotondano G Salerno R Cipolletta F Bianco MA De Gregorio A Miele R Prisco A Garofano ML Cipolletta L 《World journal of gastroenterology : WJG》2007,13(12):1877-1878
We report an unusual case of primary amyloidosis of the stomach in a patient complaining of dyspeptic symptoms. The diagnosis was confirmed histologically and other gastrointestinal site or systemic involvement was ruled out, Uncharacteristic dyspeptic symptoms may hide this rare metabolic disease. 相似文献
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Kai-Feng Yang Yong Li De-Long Wang Jun-Wu Yang Sen-Yan Wu Wei-Dong Xiao 《World journal of gastroenterology : WJG》2016,22(12):3506-3510
Primary splenic angiosarcoma(PSA) is an unusual and highly malignant vascular tumour with a high rate of metastatic. Moreover, the research on prognosis of the disease is poor. The epidemiology, etiology, clinical diagnosis and treatment of the disease remain challenging, because case reports of the disease are few in number. In accordance with other malignant tumors, PSA is very aggressive, and the majority of patients in which this disease is found are at an advanced stage. Almost all patients die within 12 mo of diagnosis irrespective of treatment. We report here a woman who had complained of upper bellyache and anorexia for 10 d. Magnetic resonance imaging showed enlargement of the spleen with multiple heterogeneous masses in the lower pole of the spleen. A hand-assisted laparoscopic splenectomy was performed which allowed histopathologic diagnosis. The patient was diagnosed with PSA and liver metastasis, and succumbed to the disease 35 d after surgery. The literature was finished combined with the clinical features, diagnosis and management of PSA. 相似文献
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Kacar S Kuran S Temucin T Odemis B Karadeniz N Sasmaz N 《World journal of gastroenterology : WJG》2007,13(9):1460-1465
Angiolipoma is a rare vascular variant of the benign lipomatous tumors and is generally seen in subcutaneous tissues. We report a 70-year-old female with abdominal distension not related to rectal small polypoid mass with peduncule described as angiolipoma by histologically, and review the literature. 相似文献
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Colombo P Rahal D Grizzi F Quagliuolo V Roncalli M 《World journal of gastroenterology : WJG》2005,11(33):5226-5228
Intra-abdominal fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor (GIST). This report highlights the fact that benign IAF can be misdiagnosed as a malignant GIST localized in the mesentery or arising from the intestinal wall. Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor STI571 (imatinib mesylate) has greatly changed the clinical approach to intra-abdominal stromal spindle cell tumors. 相似文献
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Cronkhite-Canada综合征(Cronkhite-Canada syndrome,CCS)非常罕见,病因不明,以胃肠道多发性息肉和外胚层三联征两大症候群为主.主要表现为慢性腹泻、腹部不适、毛发脱落、色素沉着、指(趾)甲萎缩等.本文报道CCS 1例,通过病史及内镜检查结果并结合相关文献对该病进行分析讨论,探讨CCS的临床特征,提高对该病的认识. 相似文献