目的 探讨原发性肾脏血管肉瘤的临床、病理学特点及其鉴别诊断和预后评估。方法 在光学显微镜下观察3例肾脏血管肉瘤组织的病理形态,行免疫组织化学（免疫组化）染色检测,并结合文献进行分析讨论。结果 镜下观察,可见肿瘤边界不清,形成不规则的互相吻合的血管腔隙,内衬异型增生的内皮细胞,其中2例伴有上皮样特征。免疫组化检测结果显示,3例肿瘤细胞均为波形蛋白(+)、CD31（+）、CD34（+）,例2同时存在少量肿瘤细胞EMA（+）,例3同时存在肿瘤细胞AE1/AE3（+）、CK8/18(+),而例1肿瘤细胞上皮性标记AE1/AE3、EMA均为阴性。结论 原发性肾脏血管肉瘤罕见,术前影像学检查缺乏特异性征像,故组织病理及免疫组化检测对患者的最终确诊至关重要。目前较为肯定的治疗方法是外科手术完整切除。
Objective: To study the clinnicopathological features, differential diagnosis and prognosis of primary renal angiosarcoma. Methods: Tumor specimens from 3 cases of primary renal angiosarcoma were observed under light microscope. Immunohistochemistry examination was performed and relevant literatures were reviewed. Results: The lesions didn't have a clear boundary between tumor and normal tissue, and multiple irregular anastomosing vascular spaces or channels which were lined by discrete and large endothelial cells with variable degrees of nuclear atypia were observed. Two cases had epithelioid characteristics. Immunohistochemical study showed that tumor cells were positive for vimentin, CD31, CD34. Tumor cells of Case 2 were also partially positive for EMA. Tumor cells of Case 3 were strongly and diffusely positive for AE1/AE3, CK8/18. Tumor cells of Case 1 were negative for AE1/AE3, EMA. Conclusions: Primary renal angiosarcoma is extremely rare, and imaging examination does not allow for specific diagnosis of the tumor. Histopathology and immunohistochemistry are of great importance for diagnosis of the tumor. Whole intact tumor resection is the most effective treatment for primary renal angiosarcoma.
[1] Prince CL.Primary angioendothelioma of the kidney: report of a case and brief review[J]. J Urol,1942,47:787.
[2] Celebi F, Pilanci KN, Saglam S, et al.Primary renal angiosarcoma with progressive clinical course despite surgical and adjuvant treatment: A case report[J]. Oncol Lett,2015,9(4):1937-1939.
[3] Nanus DM, Kelsen D, Clark DG.Radiation-induced angiosarcoma[J]. Cancer,1987,60(4):777-779.
[4] Jennings TA, Peterson L, Axiotis CA, et al.Angiosarcoma associated with foreign body material. A report of three cases[J]. Cancer,1988,62(11):2436-2444.
[5] Akkad T, Tsankov A, Pelzer A, et al.Early diagnosis and straight forward surgery of an asymptomatic primary angiosarcoma of the kidney led to long-term survival[J]. Int J Urol,2006,13(8):1112-1114.
[6] Chaabouni A, Rebai N, Chabchoub K, et al.Primary renal angiosarcoma: Case report and literature review[J]. Can Urol Assoc J,2013,7(5-6):E430-E432.
[7] Kern SB, Gott L, Faulkner J 2nd. Occurrence of primary renal angiosarcoma in brothers[J]. Arch Pathol Lab Med,1995,119(1):75-78.
[8] Zenico T, Saccomanni M, Salomone U, et al.Primary renal angiosarcoma: case report and review of world literature[J]. Tumori,2011,97(4):e6-e9.
[9] Papadimitriou VD, Stamatiou KN, Takos DM, et al.Angiosarcoma of kidney: a case report and review of literature[J]. Urol J,2009,6(3):223-225.
[10] Omiyale AO.Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases[J]. Transl Androl Urol,2015,4(4):464-473.
[11] 崔华娟, 赖日权. 脉管肿瘤和瘤样病变[M]// 韩家安,赖日权. 软组织肿瘤病理学. 北京: 科学出版社,2015:276.
[12] Terris D, Plaine L, Steinfeld A.Renal angiosarcoma[J]. Am J Kidney Dis,1986,8(2):131-133.
[13] Mordkin RM, Dahut WL, Lynch JH.Renal angiosarcoma: a rare primary genitourinary malignancy[J]. South Med J,1997,90(11):1159-1160.
[14] Martínez-Piñeiro L, López-Ferrer P, Picazo ML, Martínez-Piñeiro JA.et al.Primary renal angiosarcoma. Case report and review of the literature[J]. Scand J Urol Nephrol,1995,29(1):103-108.
[15] Azzariti A, Porcelli L, Mangia A, et al.Irradiation-induced angiosarcoma and anti-angiogenic therapy: a therapeutic hope?[J]. Exp Cell Res,2014,321(2):240-247.
[16] Sabharwal S, John NT, Kumar RM, et al.Primary renal angiosarcoma[J]. Indian J Urol,2013,29(2):145-147.
[17] Leggio L.Primary angiosarcoma of the kidney: size is the main prognostic factor[J]. Int J Urol,2007,14(8):777-778.
[18] Leggio L, Addolorato G, Abenavoli L, et al.Primary renal angiosarcoma: a rare malignancy. A case report and review of the literature[J]. Urol Oncol,2006,24(4):307-312.
[19] Nguyen T, Auquier MA, Renard C, et al.Hemoptysis and spontaneous rupture of a primary renal angiosarcoma: a case report[J]. J Radiol,2010,91(12 Pt 1):1313-1317.
[20] Zhang HM, Yan Y, Luo M, et al.Primary angiosarcoma of the kidney: case analysis and literature review[J]. Int J Clin Exp Pathol,2014,7(7):3555-3562.
