子宫血管周上皮样细胞肿瘤的临床病理分析
|
摘要
目的:探讨子宫血管周上皮样细胞肿瘤(PEComa)的临床病理学特征、治疗及预后。方法:分析2例子宫血管周上皮样细胞肿瘤的临床特点、病理形态及免疫组化表现,并结合相关文献进行复习。结果:患者均为老年女性,肿瘤均位于宫体,光镜下肿瘤细胞呈上皮样或梭形,围绕血管生长,免疫组化HMB45(+)、Melan-A(-)、SMA(+),随访至今无复发或远处转移。结论:子宫血管周上皮样细胞肿瘤是一种少见的间叶性肿瘤,具有比较特征性的组织学形态及免疫组化特点,目前首选治疗为全子宫+双附件切除,肿瘤生物学行为不明确,应当定期随访。
Objective: To analyze the clinicopathologic features,treatments and prognosis of perivascular epithelioid cell tumor( PEComa). Methods: Two cases of uterine PEComa were investigated of clinical characteristics,histopathology and immunohistochemistry. Related literatures were reviewed. Results: Two patients were elderly women,the tumors were located in the uterine body. The tumor cells were epithelioid or fusiform under light microscope and grew around the blood vessels. Immunohistochemical HMB45( +),Melan-A(-),SMA( +),follow up showed no recurrence or distant metastasis. Conclusion: Uterine PEComa is a rare mesenchymal tumor with characteristic histopathological and immunohistochemical features. The preferred treatment is uterectomy and bilateral salping-oophenrectomy. It should be followed-up for its unclear biological behavior.
Objective: To analyze the clinicopathologic features,treatments and prognosis of perivascular epithelioid cell tumor( PEComa). Methods: Two cases of uterine PEComa were investigated of clinical characteristics,histopathology and immunohistochemistry. Related literatures were reviewed. Results: Two patients were elderly women,the tumors were located in the uterine body. The tumor cells were epithelioid or fusiform under light microscope and grew around the blood vessels. Immunohistochemical HMB45( +),Melan-A(-),SMA( +),follow up showed no recurrence or distant metastasis. Conclusion: Uterine PEComa is a rare mesenchymal tumor with characteristic histopathological and immunohistochemical features. The preferred treatment is uterectomy and bilateral salping-oophenrectomy. It should be followed-up for its unclear biological behavior.
引文
[1]BONETTI F,PEA M,MARTIGNONI G,et al. PEC and sugar[J]. Am J Surg Pathol,1992,16(3):307-308.
[2] ZAMBONI G,PEA M,MARTIGNONI G,et al. Clear cell“sugar”tumor of the pancreas. A novel member of the famil of lesions characterized by the presence of perivascular epithelioid cells[J]. Am J Surg Pathol,1996,20(6):722-730.
[3]VANG R,KEMPSON R L. Perivascular epithelioid cell tumor(‘PEComa’)of the uterus[J]. Am J Surg Pathol,2002,26(1):1-13.
[4] JEON I S,LEE S M. Multimodal treatment using surgery,radiotherapy,and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus[J]. J Pediatr Hemotol Oncol,2005,27(12):681-684.
[5]CELIK H,KEFELI M,CETINKAYA M,et al. Perivascular epithelioid cell tumor(PEComa)of the uterine cervix in a patient with tuberous sclerosis complex:a literature review[J]. Turk Patoloji Dergisi,2014,11:1-5.
[6]FUKUNAGA M. Perivascular epithelioid cell tumor of the uterus:report of four cases[J]. Int J Gynecol Pathol,2005,24:341-346.
[7]FOLPE A L,MENTZEL T,LEHR H A,et a1. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin:a clinicopathologic study of 26 cases and review of the literature[J]. Am J Surg Pathol,2005,29(12):1558-1575.
[8]PAN C C,JONG Y J,CHAI C Y,et al. Comparative genomic hybridization study of perivascular epithelioid cell tumor:molecular genetic evidence of perivascular epithelioid cell tumor as a distinctive neoplasm[J]. Hum Patho1,2006,37(5):606-612.
[9]SCHOOLMEESTER J K,DAO L N,SUKOV W R,et al. TFE3translocation associated perivascular epithelioid cell neoplasm(PEComa)of the gynecologic tract:morphology,immunophenotype,differential diagnosis[J]. Am J Surg Pathol,2015,39(3):394-404.
[10]刘飞飞,饶秋,张仁亚,等.伴有TFE3扩增的宫颈血管周上皮样细胞肿瘤[J].临床与实验病理学杂志,2013,29(12):1361-1363.
[11] BLEEKER J S,QUEVEDO J F,FOLPE A L.“Malignant”perivascular epithelioid cell neoplasm:risk stratification and treatment strategies[J]. Sarcoma,2012,2012(2):1-12.
[12]VANG R,KEMPSON RL. Perivascular epithelioid cell tumor(‘PEComa’)of the uterus:a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors[J]. Am J Surg Pathol,2002,26(1):1-13.
[13]BUNCH K,SUNDE J. Fertility sparing treatment of a malignant uterine perivascular epithelioid cell tumor:a case report[J]. Gynecologic Oncology Case Reports,2014,8:14-16.
[14]STARBUCK K D,DRAKE R D,BUDD G T,et al. Treatment of advanced malignant uterine perivascular epithelioid cell tumor with m TOR inhibitors:single-institution experience and review of the literature[J]. Anticancer Res,2016,36(11):6161-6164.
[15]伍健,李媛,贺玉洁.子宫体恶性血管周上皮细胞肿瘤1例并文献复习[J].临床与实验病理学杂志,2012,28(3):333-336.
[16]牟田,王建六.子宫血管周上皮样细胞肿瘤二例及文献复习[J].中国妇产科临床杂志,2010,11(1):62-64.
[17]崔恒.子宫血管周上皮样细胞肿瘤[J].中华妇产科杂志,2011,46(4):298-300.
[18]胡旦红,刘芳,于亚威.恶性子宫血管周上皮样细胞肿瘤1例[J].实用妇产科杂志,2012,28(5):375-376.
[19]李荣岗,陈兰花,黄秀芳.子宫颈恶性血管周上皮样细胞肿瘤二例[J].中华病理学杂志,2014,43(9):631-632.
[20]唐灿,粟占三,刘保安,等.子宫及阔韧带血管周上皮样细胞肿瘤6例临床病理分析[J].诊断病理学杂志,2014,21(5):286-289.
[21]宫国良,戎锡云,陈静宁,等.子宫恶性血管周上皮样细胞肿瘤3例临床病理观察[J].诊断病理学杂志,2015,22(2):74-77.
[22]张爱兵,李俊.子宫恶性具有血管周上皮样细胞分化的肿瘤1例病理分析[J].华北理工大学学报(医学版),2016,18(6):451-453.
[2] ZAMBONI G,PEA M,MARTIGNONI G,et al. Clear cell“sugar”tumor of the pancreas. A novel member of the famil of lesions characterized by the presence of perivascular epithelioid cells[J]. Am J Surg Pathol,1996,20(6):722-730.
[3]VANG R,KEMPSON R L. Perivascular epithelioid cell tumor(‘PEComa’)of the uterus[J]. Am J Surg Pathol,2002,26(1):1-13.
[4] JEON I S,LEE S M. Multimodal treatment using surgery,radiotherapy,and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus[J]. J Pediatr Hemotol Oncol,2005,27(12):681-684.
[5]CELIK H,KEFELI M,CETINKAYA M,et al. Perivascular epithelioid cell tumor(PEComa)of the uterine cervix in a patient with tuberous sclerosis complex:a literature review[J]. Turk Patoloji Dergisi,2014,11:1-5.
[6]FUKUNAGA M. Perivascular epithelioid cell tumor of the uterus:report of four cases[J]. Int J Gynecol Pathol,2005,24:341-346.
[7]FOLPE A L,MENTZEL T,LEHR H A,et a1. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin:a clinicopathologic study of 26 cases and review of the literature[J]. Am J Surg Pathol,2005,29(12):1558-1575.
[8]PAN C C,JONG Y J,CHAI C Y,et al. Comparative genomic hybridization study of perivascular epithelioid cell tumor:molecular genetic evidence of perivascular epithelioid cell tumor as a distinctive neoplasm[J]. Hum Patho1,2006,37(5):606-612.
[9]SCHOOLMEESTER J K,DAO L N,SUKOV W R,et al. TFE3translocation associated perivascular epithelioid cell neoplasm(PEComa)of the gynecologic tract:morphology,immunophenotype,differential diagnosis[J]. Am J Surg Pathol,2015,39(3):394-404.
[10]刘飞飞,饶秋,张仁亚,等.伴有TFE3扩增的宫颈血管周上皮样细胞肿瘤[J].临床与实验病理学杂志,2013,29(12):1361-1363.
[11] BLEEKER J S,QUEVEDO J F,FOLPE A L.“Malignant”perivascular epithelioid cell neoplasm:risk stratification and treatment strategies[J]. Sarcoma,2012,2012(2):1-12.
[12]VANG R,KEMPSON RL. Perivascular epithelioid cell tumor(‘PEComa’)of the uterus:a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors[J]. Am J Surg Pathol,2002,26(1):1-13.
[13]BUNCH K,SUNDE J. Fertility sparing treatment of a malignant uterine perivascular epithelioid cell tumor:a case report[J]. Gynecologic Oncology Case Reports,2014,8:14-16.
[14]STARBUCK K D,DRAKE R D,BUDD G T,et al. Treatment of advanced malignant uterine perivascular epithelioid cell tumor with m TOR inhibitors:single-institution experience and review of the literature[J]. Anticancer Res,2016,36(11):6161-6164.
[15]伍健,李媛,贺玉洁.子宫体恶性血管周上皮细胞肿瘤1例并文献复习[J].临床与实验病理学杂志,2012,28(3):333-336.
[16]牟田,王建六.子宫血管周上皮样细胞肿瘤二例及文献复习[J].中国妇产科临床杂志,2010,11(1):62-64.
[17]崔恒.子宫血管周上皮样细胞肿瘤[J].中华妇产科杂志,2011,46(4):298-300.
[18]胡旦红,刘芳,于亚威.恶性子宫血管周上皮样细胞肿瘤1例[J].实用妇产科杂志,2012,28(5):375-376.
[19]李荣岗,陈兰花,黄秀芳.子宫颈恶性血管周上皮样细胞肿瘤二例[J].中华病理学杂志,2014,43(9):631-632.
[20]唐灿,粟占三,刘保安,等.子宫及阔韧带血管周上皮样细胞肿瘤6例临床病理分析[J].诊断病理学杂志,2014,21(5):286-289.
[21]宫国良,戎锡云,陈静宁,等.子宫恶性血管周上皮样细胞肿瘤3例临床病理观察[J].诊断病理学杂志,2015,22(2):74-77.
[22]张爱兵,李俊.子宫恶性具有血管周上皮样细胞分化的肿瘤1例病理分析[J].华北理工大学学报(医学版),2016,18(6):451-453.