Primary epithelioid angiosarcoma of the bladder: clinicopathological analysis of a case and review of literature

doi:10.16150/j.1671-2870.2022.06.09

Abstract

Abstract:

Objective: To investigate the clinicopathological characteristics, diagnosis, differential diagnosis, biological behavior, prognosis, and treatment options of patients with primary epithelioid angiosarcoma(EA) of the bladder. Methods: The clinical manifestations, histological features, immunohistochemistry results and follow-up of a case of primary EA of the bladder were analyzed：Relevant literature from 1998 up to 2021 were searched on PubMed， and 17 cases with relatively complete clinical and follow-up data reported in the literature were summarized. Results: Primary EA of the bladder is rare, males tend to occur (male: female, 15∶3), the median age at presentation was 65 years (range, 32-85 years), and 8 patients (8/18 cases)(44.4%) had a clear history of radiotherapy to the pelvis. Most of them had local recurrence and distant metastasis within a short period of time. During follow-up 12 of the 18 cases (66.7%) died of disease, with a median survival of 6 months (range, 5 weeks to 19 months), 6 of 18 cases (33.3%) survived with or without disease, the follow-up time ranged from 3 months to 32 months. Histologically, the tumors were composed of sheets and sinusoid of highly atypical epithelioid cells. Tumor cells were pleomorphic with amphophilic cytoplasm. Mitotic activity was high and pathological mitotic could be seen. Intracytoplasmic lumens or vacuoles were present with occasional red blood cell within the vacuole. A hemorrhagic background was present. Immunostaining was positive for Vimentin, CD31, CD34, ERG, Fli-1 and CK-P, with partly positivity for CK7, Ki-67 hot spot about 40%. Conclusions: Bladder primary EA is rare. Some patients have a history of pelvis radiotherapy and are highly malignant. The therapentic effect and prognosis are not satisfied. Radical surgery combined with radiotherapy and chemotherapy and other treatment options may prolong survival. Because the immunohistochemical staining of cytokeratin is positive, it is easy to be misdiagnosed as high-grade cancer. It needs to be differentiated from post-radiotherapy sarcoma, sarcomatoid carcinoma, pseudoangiosarcomatoid urothelial carcinoma, epithelioid hemangioendothelioma, et al.

Key words: Bladder, Epithelioid angiosarcoma, Post-radiotherapy sarcoma, Anlotinib

CLC Number:

R541.7

HE Yanyan, LI Fengzhu. Primary epithelioid angiosarcoma of the bladder: clinicopathological analysis of a case and review of literature[J]. Journal of Diagnostics Concepts & Practice, 2022, 21(06): 719-725.

Figures/Tables 6

病例号	作者，年份	年龄（岁）	性别	首发症状	手术方式	部位	免疫组化	肿瘤史	放疗史	放疗至 EA时间（y）	联合治疗方式	随访（月）
1	Engel, 1998^[5]	47	男	血尿，耻骨上及左侧腹股沟区疼痛	根治	膀胱三角区	不详	无	无	无	放化疗	32，带病生存
2	Seethala, 2006^[6]	66	男	血尿	根治	膀胱左后壁、顶壁	CD31⁺CD34⁺ CK^-	不详	有	4	化疗	19，带病生存
3	Kulaga, 2007^[7]	83	女	血尿	电切	膀胱后外侧壁	Vimentin⁺CD31⁺ FⅧ^-CD34^-	子宫内膜癌	有	14	无	3，死亡
4	Warne, 2011^[8]	32	女	血尿，左侧腹痛	电切	后壁	CD31⁺FⅧ⁺ ULEX⁺CK⁺	无	无	无	放化疗	19，死亡
5	Abbasov, 2011^[4]	51	男	血尿	电切+根治	右前壁、侧壁	CK⁺FⅧ⁺CD31⁺ Vimentin⁺CD34^-	无	无	无	无	5周，死亡
6	Matoso, 2015^[3]	73	女	血尿	电切+部分切除	顶壁	CD31⁺FⅧ⁺	宫颈鳞癌，恶性间皮瘤	有	10	无	6，死亡
7	-	77	男	血尿	电切	膀胱颈	CD31⁺CD34⁺FⅧ^-	前列腺癌	有	9	无	14，死亡
8	-	71	男	血尿	电切+根治	膀胱颈	CD31⁺FⅧ⁺	前列腺癌	有	10	无	7，死亡
9	-	85	男	血尿	电切	右侧壁	CD31⁺CD34⁺FⅧ⁺	前列腺癌	有	15	无	6，死亡
10	-	39	男	血尿	电切+根治	后壁	CD31⁺	无	无	无	无	13，死亡
11	-	64	男	血尿	电切+根治+盆腔淋巴结清扫	膀胱颈+ 左侧壁	CD31⁺ERG⁺	前列腺癌	有	6	无	12，无病生存
12	-	43	男	血尿	电切	左侧壁	CD31⁺FⅧ⁺ERG⁺ CD34^-	无	无	无	无	6，带病生存
13	-	73	男	血尿	电切	左侧尿道口	CD31⁺ERG⁺	无	不详	不详	无	3，死亡
14	-	64	男	血尿	电切	膀胱颈	CD34⁺ERG⁺	前列腺癌	有	15		3，带病生存
15	Gerbaud, 2016^[9]	72	男	血尿，急性尿潴留	电切+根治	左侧壁	Vimentin⁺CD31⁺ERG⁺ Fli-1⁺CD34^-CK^-	无	无	无	放疗	5，死亡
16	Wang, 2016^[10]	79	男	血尿，刺激性下尿路症状	电切+根治	三角区+ 前列腺	CD31⁺CD34+ERG⁺ Fli-1⁺CK部分⁺	前列腺癌	有	6	无	20，无病生存
17	NizamA, 2018^[11]	57	男	血尿	部分切除	肾盂、输尿管、膀胱	CD31⁺FⅧ⁺Fli-1⁺CK⁺ CD34灶阳性 ERG灶阳性	无	无	无	无	3，死亡
18	本例	56	男	血尿	电切	左后壁	CD31+CD34+ERG+ Fli-1⁺Vimentin⁺CK⁺	无	无	无	安罗替尼	9，死亡

References 14

[1]	王坚. 血管肉瘤. 软组织肿瘤病理学.2版. 北京: 人民卫生出版社, 2017:819-857.
	Wang J. Angiosarcoma[M]//Wang J, Zhu X Z. Pathology of soft tissue tumors. 2nd Ed, Beijing: People′s Health Publishing House, 2017:819-857.
[2]	Humphrey P A, Moch H, Cubilla A L, et al. Tumors of the urinary tract[M]// WHO Classification of Tumors of the Urinary System and Male Gential Organs. Lyon: IARC Press, 2016:122-127.
[3]	Matoso A, Epstein J I. Epithelioid Angiosarcoma of the Bladder: A Series of 9 Cases[J]. Am J Surg Pathol, 2015, 39(10):1377-1382. doi: 10.1097/PAS.0000000000000444 pmid: 25929352
[4]	Abbasov B, Munguia G, Mazal P R, et al. Epithelioid angiosarcoma of the bladder: report of a new case with immunohistochemical profile and review of the literature[J]. Pathology, 2011, 43(3):290-293. doi: 10.1097/PAT.0b013e328344e2fb URL
[5]	Engel J D, Kuzel T M, Moceanu M C, et al. Angiosarcoma of the bladder: a review[J]. Urology, 1998, 52(5):778-784. pmid: 9801098
[6]	Seethala R R, Gomez J A, Vakar-Lopez F. Primary angiosarcoma of the bladder[J]. Arch Pathol Lab Med, 2006, 130(10):1543-1547. doi: 10.5858/2006-130-1543-PAOTB pmid: 17090199
[7]	Kulaga A, Yilmaz A, Wilkin R P, et al. Epithelioid angiosarcoma of the bladder after irradiation for endometrioid adenocarcinoma[J]. Virchows Arch, 2007, 450(2):245-246. pmid: 17149614
[8]	Warne R R, Ong J S, Snowball B, et al. Primary angiosarcoma of the bladder in a young female[J]. BMJ Case Rep, 2011, 2011:bcr1120103484.
[9]	Gerbaud F, Ingels A, Ferlicot S, et al. Angiosarcoma of the bladder: review of the literature and discussion about a clinical case[J]. Urol Case Rep, 2017, 13:97-100. doi: 10.1016/j.eucr.2016.12.007 pmid: 28480169
[10]	Wang G, Black P C, Skinnider B F, et al. Post-radiation epithelioid angiosarcoma of the urinary bladder and prostate[J]. Can Urol Assoc J, 2016, 10(5-6):E197-E200.
[11]	Nizam A, Paquette E L, Wang B G, et al. Epithelioid angiosarcoma of the bladder: a case report and review of the Literature[J]. Clin Genitourin Cancer, 2018, 16(6):e1091-e1095. doi: 10.1016/j.clgc.2018.07.010 URL
[12]	聂秀. 尿路上皮癌的组织学变异[M]//聂秀, 黄邦杏. 膀胱活检病理解读. 2版. 北京: 人民卫生出版社, 2012:131-135.
	Nie X. Biopsy interpretation of the bladder.2nd ed. Beijing: People′s Health Publishing House, 2012:131-135.
[13]	Pazona J F, Gupta R, Wysock J, et al. Angiosarcoma of bladder: long-term survival after multimodal therapy[J]. Urology, 2007, 69(3):575,e9-e10.
[14]	祝冰晶, 罗虎, 唐春兰. 安罗替尼治疗原发性胸膜及心包血管肉瘤1例[J]. 肿瘤预防与治疗, 2020, 33(9):813-816.
	Zhu B J, Luo H, Tang C L. Treatment of primary pleural and pericardial angiosarcoma with arotinib: a case report[J]. J Cancer Control Treat, 2020, 33(9):813-816.