Matoso Andres, Epstein Jonathan I
Departments of *Pathology †Oncology ‡Urology, The Johns Hopkins Hospital, Baltimore, MD.
Am J Surg Pathol. 2015 Oct;39(10):1377-82. doi: 10.1097/PAS.0000000000000444.
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.
膀胱原发性血管肉瘤非常罕见，文献报道约30例。具有上皮样形态的病例更为罕见，仅有个案报道发表。我们描述了1998年至2014年从我们的外科病理档案中检索出的9例膀胱上皮样血管肉瘤（EA）患者的组织病理学特征及临床随访情况。其中8例为会诊病例。患者就诊时的平均年龄为65岁（范围39至85岁）。男女比例为8:1。所有病例的临床表现均为血尿和膀胱肿块。6例患者有盆腔放疗史，5例用于治疗前列腺癌，1例用于治疗子宫颈癌。从放疗至EA诊断的时间为6至15年。肿瘤平均大小为4 cm（范围1至8 cm）。送检诊断为低分化癌（5例）、高级别浸润性尿路上皮癌（3例）和非典型血管增生（1例）。形态学上，肿瘤由巢状和片状高度非典型细胞组成，核质比高，偶见胞质内管腔，背景为出血性。所有病例均未显示任何尿路上皮癌成分。3例患者的切除标本中还显示有普通型血管肉瘤。免疫组化结果显示，9例中有5例细胞角蛋白阳性，包括CK7（3例）、AE1/AE3（3例）和Cam5.2（1例）。所有病例至少有一种内皮标志物阳性，包括CD31（7例）、CD34（2例）、FVIII（3例）和ERG（2例）。尿路上皮标志物（p63和GATA3）始终为阴性。手术治疗包括单纯经尿道膀胱肿瘤切除术（TURB）（5例）、TURB后行膀胱前列腺切除术（2例）、TURB后行部分膀胱切除术（1例）和单纯膀胱前列腺切除术（1例）。9例患者中，5例肿瘤侵犯固有肌层，1例侵犯输尿管周围脂肪组织，1例侵犯前列腺和精囊。9例患者中有5例死于疾病，中位生存期为7个月（范围6至14个月）。2例患者在随访3个月和6个月时仍有疾病存活。1例行根治性膀胱前列腺切除术的患者术后12个月无疾病存活。膀胱EA是一种罕见的恶性肿瘤，常被误诊为高级别癌，尤其是由于细胞角蛋白免疫染色阳性。该肿瘤在有盆腔放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核伴散在红细胞、出血性背景和偶见胞质内管腔。患者通常表现为肌层浸润性疾病，预后不佳。
