首页 > 期刊杂志 > 正文

Epithelioid Angiosarcoma of the Bladder: A Series of 9 Cases.

膀胱上皮样血管肉瘤:9例系列报道

Abstract

Primary angiosarcoma of the bladder is very rare, with approximately 30 cases reported in the literature. Those with epithelioid morphology are even rarer, with only single-case reports published. We describe the histopathologic features and clinical follow-up of 9 patients with epithelioid angiosarcoma (EA) of the bladder retrieved from our Surgical Pathology files from 1998 to 2014. Eight cases were consults. The mean age at presentation was 65 years (range, 39 to 85 y). The M:F ratio was 8:1. The clinical presentation was hematuria and bladder mass in all cases. Six patients had a history of radiotherapy to the pelvis, 5 to treat prostate cancer and 1 to treat uterine cervical cancer. The time from radiotherapy to the diagnosis of EA ranged from 6 to 15 years. The average size of the tumor was 4 cm. (range, 1 to 8 cm.). The submitting diagnoses were poorly differentiated carcinoma (n=5), high-grade invasive urothelial carcinoma (n=3), and atypical vascular proliferation (n=1). Morphologically, the tumors were composed of nests and sheets of highly atypical cells with high nuclear to cytoplasmic ratio, occasional intracytoplasmic lumens, and a hemorrhagic background. None of the cases showed any urothelial carcinoma component. Three patients showed in addition usual angiosarcoma in the resection specimen. By immunohistochemistry, 5/9 cases were positive for cytokeratins, including CK7 (n=3), AE1/AE3 (n=3), and Cam5.2 (n=1). All cases were positive for at least 1 endothelial marker, including CD31 (n=7), CD34 (n=2), FVIII (n=3), and ERG (n=2). Urothelial markers (p63 and GATA3) were consistently negative. Surgical treatment included transurethral resection of the bladder (TURB) only (n=5), TURB followed by cystoprostatectomy (n=2), TURB followed by partial cystectomy (n=1), and cystoprostatectomy only (n=1). The tumor involved the muscularis propria in 5/9 patients, the periureteric adipose tissue in 1 patient, and the prostate and seminal vesicles in 1 patient. Five of 9 patients died of disease, with a median survival of 7 months (range, 6 to 14 mo). Two patients were alive with disease at 3 and 6 months of follow-up. One patient who underwent radical cystoprostatectomy was alive with no evidence of disease 12 months after surgery. EA of the bladder is a rare malignancy that is frequently misdiagnosed as high-grade carcinoma, especially due to positive immunostaining for cytokeratins. This tumor is more frequent in older men with a history of radiotherapy to the pelvis. Morphologic features that should suggest the vascular origin of the tumor include highly atypical nuclei with interspersed erythrocytes, hemorrhagic background, and occasional intracytoplasmic lumens. Patients usually present with muscle invasive disease, and the prognosis is dismal.

摘要

膀胱原发性血管肉瘤非常罕见,文献中大约报道30例。而上皮样形态的血管肉瘤更加罕见,仅有1例个案报道。作者收集1998年至2014年期间他们外科病理档案里的9例膀胱上皮样血管肉瘤(EA),并描述其组织病理学特征和患者的临床随访情况。患者中位年龄65岁(范围从9-95岁),男:女=8:1。所有病例临床表现均为血尿和膀胱肿块。6例有骨盆放射治疗的病史(5例为治疗前列腺癌,1例为治疗宫颈癌)。从放射治疗到诊断EA的时间为6-15年。肿瘤的平均大小4cm(范围从1-8cm)。先前的诊断包括差分化癌(n=5)、高级别浸润性尿路上皮癌(n=3)、不典型血管性增生(n=1)。形态学上肿瘤由呈巢团状、片状的高度异型性细胞组成,核浆比高,偶尔可见胞浆内腔隙和出血性背景。所有病例均未见任何尿路上皮癌的成分。免疫组化5/9例细胞角蛋白阳性,包括CK7(n=3)、AE1/AE3 (n=3)和Cam5.2 (n=1);所有病例至少有1项内皮标记阳性,包括CD31(n=7)、CD34 (n=2)、FVIII (n=3)和ERG (n=2);尿路上皮标记(p63和GATA3)一致性阴性。外科治疗包括经尿道膀胱肿瘤电切除术(TURB)(n=5)、TURB后行膀胱前列腺切除术(n=2)、TURB后行部分膀胱切除术(n=1)、膀胱前列腺切除术(n=1)。5/9的患者肿瘤累及固有肌层,1例累及输尿管周脂肪组织,1例累及前列腺和精囊腺。5例患者死于该疾病,中位生存期7个月(范围6-14个月)。2例患者在随访3、6个月时带病生存。1例接受根治性膀胱前列腺切除术的患者术后随访12个月无病生存。膀胱EA是一种非常罕见的恶性肿瘤,特别因细胞角蛋白免疫染色阳性,常被误诊为高级别尿路上皮癌。常发生在有骨盆放射治疗的老年男性患者。形态学上高度异型的细胞中出现散在分布的红细胞、出血性背景及偶尔的胞浆内腔隙提示血管起源的可能性。患者通常出现肌肉侵袭性病程,预后较差。

full text

我要评论

0条评论