Epithelioid Angiosarcoma of the Bladder A Series of 9 Cases
Epithelioid Angiosarcoma of the Bladder A Series of 9 Cases
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DOI：
10.1097/pas.0000000000000444
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发表时间：
2015-10-01
影响因子：
5.6
通讯作者：
Epstein, Jonathan I.
中科院分区：
文献类型：
--
作者：
Matoso, Andres;Epstein, Jonathan I.
原发性膀胱血管肉瘤非常罕见，文献报道了大约 30 例。具有上皮样形态的则更为罕见，仅发表过单例报告。我们描述了 1998 年至 2014 年从我们的外科病理学档案中检索到的 9 例膀胱上皮样血管肉瘤 (EA) 患者的组织病理学特征和临床随访。其中 8 例进行了会诊。就诊时的平均年龄为 65 岁（范围为 39 至 85 岁）。 M:F比率为8:1。所有病例的临床表现均为血尿和膀胱肿块。 6 名患者有骨盆放射治疗史，其中 5 名患者接受过前列腺癌治疗，1 名患者接受过宫颈癌治疗。从放疗到诊断EA的时间为6至15年。肿瘤的平均大小为4厘米。 （范围：1 至 8 厘米）。提交的诊断为低分化癌（n=5）、高级别浸润性尿路上皮癌（n=3）和非典型血管增生（n=1）。在形态学上，肿瘤由高度非典型细胞的巢和片组成，具有高核质比、偶尔有胞质内腔和出血背景。所有病例均未显示出任何尿路上皮癌成分。三名患者在切除标本中还显示出常见的血管肉瘤。免疫组化显示，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 1 种内皮标志物呈阳性，包括 CD31 (n=7)、CD34 (n=2)、FVIII (n=3) 和 ERG (n=2)。尿路上皮标志物（p63 和 GATA3）始终呈阴性。手术治疗包括仅经尿道膀胱切除术 (TURB) (n=5)、TURB 后行膀胱前列腺切除术 (n=2)、TURB 后行膀胱部分切除术 (n=1) 和仅膀胱前列腺切除术 (n=1)。 5/9患者肿瘤累及固有肌层，1例累及输尿管周围脂肪组织，1例累及前列腺和精囊。 9 名患者中有 5 名死于疾病，中位生存期为 7 个月（范围为 6 至 14 个月）。随访 3 个月和 6 个月时，两名患者仍存活。一名接受根治性膀胱前列腺切除术的患者在术后 12 个月仍存活，没有任何疾病证据。膀胱 EA 是一种罕见的恶性肿瘤，经常被误诊为高级别癌，特别是由于细胞角蛋白免疫染色呈阳性。这种肿瘤在有骨盆放射治疗史的老年男性中更为常见。提示肿瘤血管起源的形态学特征包括高度非典型的细胞核，散布着红细胞、出血背景和偶尔的胞质内腔。患者通常表现为肌肉侵袭性疾病，预后不佳。
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.