原发性脾上皮样血管肉瘤临床病理观察
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摘要
目的探讨脾上皮样血管肉瘤(SEAS)的临床病理特点、免疫组化、诊断及鉴别诊断。方法回顾性分析1例脾上皮样血管肉瘤的临床表现、组织学形态特征及免疫组化,并复习相关文献。结果正常的脾组织被增生的上皮样肿瘤细胞所代替,并且向周围脾组织浸润生长,瘤细胞呈圆形、卵圆形,胞质丰富,细胞核呈空泡状,可见大核仁,核分裂易见(>20个/50HPF)。肿瘤细胞排列呈巢片状,局灶可呈乳头状或不规则裂隙状排列,裂隙内见红细胞,提示血管来源。肿瘤细胞CD31、CK、vimentin、ERG(+),CgA及Syn散在(+),Ki-67阳性指数30%;CD34、S-100、EMA、FⅧ、CK7、CK20、CD30、CD68、Lys、ALK、FIL-1、D2-40和p63均为(-)。患者于脾切除术后1个月死亡。结论原发于脾的上皮样血管肉瘤是起源于血管的恶性肿瘤,临床主要表现为急腹症,预后较差;诊断主要依赖于组织学形态及免疫组化。
Objective To analyze the clinical pathological characteristics, diagnosis and differential diagnosis of splenic epithelioid angiosaroma(SEAS). Methods One case of SEAS was collected, the clinical and pathological features were investigated with review of the related literatures. Results The normal spleen cells had been replaced by atypical epithelioid cells that infiltrated into the surrounding tissue. The epithelioid cells were large, oval or round, and had abundant cytoplasm, large vesicular nuclei, prominent nucleoli, and frequent mitosis. The tumor cells showed a diffuse growth pattern and were arranged in sheets. Occasionally, they also showed vascular cavity formation and papillary structure and erythrocytes were found in the vascular cavity, suggesting a vascular tumor origin. Tumor cells were positive for CD31, CK and vimentin, focal positive for CgA and Syn; the Ki-67 proliferation index was about 30%. The tumor cells were negative for S-100, CD34, EMA, FⅧ, CK7, CK20, CD30, CD68, Lys, ALK and P63. The patient died a month after the laparotomy. Conclusions The SEAS is a malignant tumor derived from blood vessels with the clinical manifestations of acute abdomen symptom; the prognosis is extremely poor; the diagnosis of SEAS is mainly based on histological characteristics and immunohistochemical staining.
Objective To analyze the clinical pathological characteristics, diagnosis and differential diagnosis of splenic epithelioid angiosaroma(SEAS). Methods One case of SEAS was collected, the clinical and pathological features were investigated with review of the related literatures. Results The normal spleen cells had been replaced by atypical epithelioid cells that infiltrated into the surrounding tissue. The epithelioid cells were large, oval or round, and had abundant cytoplasm, large vesicular nuclei, prominent nucleoli, and frequent mitosis. The tumor cells showed a diffuse growth pattern and were arranged in sheets. Occasionally, they also showed vascular cavity formation and papillary structure and erythrocytes were found in the vascular cavity, suggesting a vascular tumor origin. Tumor cells were positive for CD31, CK and vimentin, focal positive for CgA and Syn; the Ki-67 proliferation index was about 30%. The tumor cells were negative for S-100, CD34, EMA, FⅧ, CK7, CK20, CD30, CD68, Lys, ALK and P63. The patient died a month after the laparotomy. Conclusions The SEAS is a malignant tumor derived from blood vessels with the clinical manifestations of acute abdomen symptom; the prognosis is extremely poor; the diagnosis of SEAS is mainly based on histological characteristics and immunohistochemical staining.
引文
[1] Perez-Atayde AR, Achenbach H, Lack EE. High-grade epithelioid angiosarcoma of the scalp: an immunohistochemical and ultrastructural study [J]. Am J Dermatopathol, 1986, 8(5): 411-418.
[2] Wu J, Li X, Liu X. Epithelioid angiosarcoma: a clinicopathological study of 16 Chinese cases [J]. Int J Clin Exp Pathol, 2015, 8(4): 3901-3909.
[3] Padaki P, Hutton R, Amer T, et al. A rare case of primary epithelioid angiosarcoma of the ureter [J]. Ann R Coll Surg Engl, 2016, 98(8): e184-e185.
[4] Rotellini M, Vezzosi V, Bianchi S. Epithelioid angiosarcoma of the thyroid: report of a case from an italian non-alpine area and review of the literature [J]. Endocr Pathol, 2015, 26(2): 152-156.
[5] Neuhauser TS, Derringer GA, Thompson LD, et al. Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases [J]. Mod Pathol, 2000, 13(9): 978-987.
[6] 杨景,柳凤轩. 脾上皮样血管肉瘤1例[J].癌症,2001,20(9):1008.
[7] 蔡萍,丁仕义. 脾上皮样血管肉瘤一例[J].中华放射学杂志,2002,36(2):190-191.
[8] Abdallah RA, Abdou AG, Asaad NY, et al. Primary epithelioid angiosarcoma of spleen: a case report and review of literature[J]. J Clin Diagn Res, 2016, 10(1): D5-D7.
[9] Cokelaere K, Vanvuchelen J, Michielsen P, et al. Epithelioid angiosarcoma of the splenic capsule. Report of a case reiterating the concept of inert foreign body tumorigenesis[J]. Virchows Arch, 2001, 438(4): 398-403.
[10] Keymeulen K, Dillemans B. Epitheloid angiosarcoma of the splenic capsula as a result of foreign body tumorigenesis: a case report [J]. Acta Chir Belg, 2004, 104(2): 217-220.
[11] Brǎtilǎ E, Ionescu OM, Berceanu C, et al. Vaginal epithelioid angiosarcoma: a rare case [J]. Rom J Morphol Embryol, 2016, 57(3): 1131-1135.
[12] Yang Z, Tao H, Ye Z, et al. Multicentric epithelioid angiosarcoma of bone [J]. Orthopedics, 2012, 35(8): e1293-e1296.
[13] 闫慧明,张明明,贾木彩,等. 小肠上皮样血管肉瘤伴出血穿孔1例报告[J]. 中国实用外科杂志,2013, 33(1):85-86.
[14] Collini P, Barisella M, Renne SL, et al. Epithelioid angiosarcoma of the thyroid gland without distant metastases at diagnosis: report of six cases with a long follow-up [J]. Virchows Arch. 2016, 469(2): 223-232.
[15] Frontario SC, Goldenberg-Sandau A, Roy D, et al. Primary splenic angiosarcoma presenting as idiopathic thrombocytopenic purpura: a case report and review of the literature [J]. Case Rep Surg, 2016, 2016: 4173060.
[16] Chen G, Li M, Wu D, et al. Primary splenic angiosarcoma in a 2.5-year-old boy with hepatic metastasis [J]. Pediatr Surg Int, 2012, 28(11): 1147-1150.
[17] Hsu JT, Ueng SH, Hwang TL, et al. Primary angiosarcoma of the spleen in a child with long-term survival [J]. Pediatr Surg Int, 2007, 23(8): 807-810.
[2] Wu J, Li X, Liu X. Epithelioid angiosarcoma: a clinicopathological study of 16 Chinese cases [J]. Int J Clin Exp Pathol, 2015, 8(4): 3901-3909.
[3] Padaki P, Hutton R, Amer T, et al. A rare case of primary epithelioid angiosarcoma of the ureter [J]. Ann R Coll Surg Engl, 2016, 98(8): e184-e185.
[4] Rotellini M, Vezzosi V, Bianchi S. Epithelioid angiosarcoma of the thyroid: report of a case from an italian non-alpine area and review of the literature [J]. Endocr Pathol, 2015, 26(2): 152-156.
[5] Neuhauser TS, Derringer GA, Thompson LD, et al. Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases [J]. Mod Pathol, 2000, 13(9): 978-987.
[6] 杨景,柳凤轩. 脾上皮样血管肉瘤1例[J].癌症,2001,20(9):1008.
[7] 蔡萍,丁仕义. 脾上皮样血管肉瘤一例[J].中华放射学杂志,2002,36(2):190-191.
[8] Abdallah RA, Abdou AG, Asaad NY, et al. Primary epithelioid angiosarcoma of spleen: a case report and review of literature[J]. J Clin Diagn Res, 2016, 10(1): D5-D7.
[9] Cokelaere K, Vanvuchelen J, Michielsen P, et al. Epithelioid angiosarcoma of the splenic capsule. Report of a case reiterating the concept of inert foreign body tumorigenesis[J]. Virchows Arch, 2001, 438(4): 398-403.
[10] Keymeulen K, Dillemans B. Epitheloid angiosarcoma of the splenic capsula as a result of foreign body tumorigenesis: a case report [J]. Acta Chir Belg, 2004, 104(2): 217-220.
[11] Brǎtilǎ E, Ionescu OM, Berceanu C, et al. Vaginal epithelioid angiosarcoma: a rare case [J]. Rom J Morphol Embryol, 2016, 57(3): 1131-1135.
[12] Yang Z, Tao H, Ye Z, et al. Multicentric epithelioid angiosarcoma of bone [J]. Orthopedics, 2012, 35(8): e1293-e1296.
[13] 闫慧明,张明明,贾木彩,等. 小肠上皮样血管肉瘤伴出血穿孔1例报告[J]. 中国实用外科杂志,2013, 33(1):85-86.
[14] Collini P, Barisella M, Renne SL, et al. Epithelioid angiosarcoma of the thyroid gland without distant metastases at diagnosis: report of six cases with a long follow-up [J]. Virchows Arch. 2016, 469(2): 223-232.
[15] Frontario SC, Goldenberg-Sandau A, Roy D, et al. Primary splenic angiosarcoma presenting as idiopathic thrombocytopenic purpura: a case report and review of the literature [J]. Case Rep Surg, 2016, 2016: 4173060.
[16] Chen G, Li M, Wu D, et al. Primary splenic angiosarcoma in a 2.5-year-old boy with hepatic metastasis [J]. Pediatr Surg Int, 2012, 28(11): 1147-1150.
[17] Hsu JT, Ueng SH, Hwang TL, et al. Primary angiosarcoma of the spleen in a child with long-term survival [J]. Pediatr Surg Int, 2007, 23(8): 807-810.