恶性肾血管周上皮样细胞肿瘤1例报告并文献复习
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摘要
目的:探讨恶性肾血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)的临床病理学特征、诊断以及治疗方式。方法 :报道1例PEComa患者的临床诊断及治疗结果,分析其组织形态学特点,并复习相关文献,归纳该肿瘤的诊断和治疗策略。结果 :1例21岁女性患者因发现左肾肿物就诊,接受左肾切除术治疗。术后病理提示PEComa。2年后CT检查发现肺部及骨骼转移,遂予以阿帕替尼和重组人内皮抑制素联合化疗。尽管经积极治疗,但是肿瘤仍进展。患者在确诊45个月后死于呼吸衰竭。文献复习发现,PEComa缺乏典型的临床症状,免疫组织化学染色标志物如人类黑素瘤特异性单抗45(human melanoma black 45,HMB45)、黑素瘤抗原(melanoma antigen,Melan-A)和平滑肌抗体(smooth muscle actin,SMA)对于PEComa诊断具有关键作用。结论 :PEComa是一种罕见肿瘤,应提高对其认识和诊疗水平,并在术后长期密切随访。
Objective: To investigate the clinicopathological characteristics, diagnosis, and treatment of malignant perivascular epithelioid cell tumor(PEComa) in kidney.Methods: The cinical diagnosis and treatment outcome of a case of PEComa were reported. The morphological characteristics of renal PEComa were analyzed. The PEComa-related literatures were reviewed, and the diagnosis and treatment strategies of PEComa were summarized.Results: A 21-year-old female patient with a solid mass in the left kidney underwent nephrectomy. The pathological examination revealed PEComa. After 2 years, the computed tomography(CT) scan showed several masses in the lung and bone, which were speculated to be metastases from the kidney lesion. The patient received chemotherapy with recombinant human endostatin and Apatinib. Despite active treatment, the tumor was still progressing, and the patient died of respiratory failure 45 months after the original diagnosis. Literature reviews showed that PEComa patients had not typical clinical symptoms, and the positive immunohistochemical results of human melanoma black-45(HMB45), melanoma antigen(Melan-A), and smooth muscle actin(SMA) were the key features in the diagnosis of PEComa.Conclusion: PEComa is a kind of rare tumor. The diagnosis and treatment of this disease should be intensified, and the long-term close follow-up is necessary.
Objective: To investigate the clinicopathological characteristics, diagnosis, and treatment of malignant perivascular epithelioid cell tumor(PEComa) in kidney.Methods: The cinical diagnosis and treatment outcome of a case of PEComa were reported. The morphological characteristics of renal PEComa were analyzed. The PEComa-related literatures were reviewed, and the diagnosis and treatment strategies of PEComa were summarized.Results: A 21-year-old female patient with a solid mass in the left kidney underwent nephrectomy. The pathological examination revealed PEComa. After 2 years, the computed tomography(CT) scan showed several masses in the lung and bone, which were speculated to be metastases from the kidney lesion. The patient received chemotherapy with recombinant human endostatin and Apatinib. Despite active treatment, the tumor was still progressing, and the patient died of respiratory failure 45 months after the original diagnosis. Literature reviews showed that PEComa patients had not typical clinical symptoms, and the positive immunohistochemical results of human melanoma black-45(HMB45), melanoma antigen(Melan-A), and smooth muscle actin(SMA) were the key features in the diagnosis of PEComa.Conclusion: PEComa is a kind of rare tumor. The diagnosis and treatment of this disease should be intensified, and the long-term close follow-up is necessary.
引文
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[4]SALUJA K,THOMAS K,ZHANG S,et al.Malignant perivascular epithelioid cell tumor of the oropharynx with strong TFE3expression mimicking alveolar soft part sarcoma:A case report and review of the literature[J].Hum Pathol,2018,76(1):149-155.
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[7]张俊,胡林昆,张学锋,等.肾脏血管周上皮细胞肿瘤临床分析[J].浙江临床医学,2016,18(3):553-554.
[8]FADARE O.Uterine PEComa:Appraisal of a controversial and increasingly reported mesenchymal neoplasm[J].Int Semin Surg Oncol,2008,5:7.
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[11]PARK SJ,HAN DK,BAEK HJ,et al.Perivascular epithelioid cell tumor(PEComa)of the ascending colon:the implication of IFN-α2b treatment[J].Korean J Pediatr,2010,53(11):975-978.
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[13]TYNSKI Z,CHIANG W,BARRETT A.An inguinal perivascular epithelioid cell tumor metastatic to the orbit[J].Case Rep Pathol,2018,2018:5749421.
[14]FOLKMAN J.Tumor angiogenesis:therapeutic implications[J].N Engl J Med,1971,285(21):1182-1186.
[15]LI J,ZHAO X,CHEN L,et al.Safety and pharmacokinetics of novel selective vascular endothelial growth factor receptor-2 inhibitor YN968D1 in patients with advanced malignancies[J].BMCCancer,2010,10:529.
[16]傅敬忠,徐冠军,李金高,等.甲磺酸阿帕替尼治疗晚期恶性肿瘤的疗效观察[J].实用癌症杂志,2018,33(2):343-345.
[17]桂秀娟,赵建夫,林绍强,等.阿帕替尼治疗转移性肾细胞癌1例[J].肿瘤学杂志,2018,24(4):409-410.
[18]王金万,孙燕,刘永煜,等.重组人血管内皮抑素联合NP方案治疗晚期NSCLC随机、双盲、对照、多中心Ⅲ期临床研究[J].中国肿瘤杂志,2005,8(4):283-290.
[19]牛牛,李宝兰,刘朝阳,等.重组人内皮抑制素联合贝伐珠单抗体内抑瘤作用的效果及分析[J].中国肺癌杂志,2013,16(2):61-66.
[20]李永强,胡晓桦.唑来磷酸治疗癌性骨痛的临床分析[J].广西医学,2015,27(9):1353-1355.
[21]何斌,蔡雅霜,张志刚,等.双磷酸盐类药物用于肿瘤辅助治疗的临床研究进展[J].中国新药与临床杂志,2018,37(4):185-192.
[22]SHAO C,TIAN G,HUANG Y,et al.Thymosin alpha-1-transformed Bifidobacterium promotes T cell proliferation and maturation in mice by oral administration[J].Int Immunnopharmacol,2013,15(3):646-653.
[23]YANG X,QIAN F,HE HY,et al.Effect of thymosin alpha-1 on subpopulations of Th1,Th2,Th17,and regulatory T cells(Tregs)in vitro[J].Braz J Med Biol Res,2012,45(1):25-32.
[24]LAO X,LIU M,ZHENG H.A tumorpenetrating peptide modification enhances the antitumor activity of thymosin alpha1[J].PLo S One,2013,8(8):e72242.
[2]FOLPE AL,KWIATKOWSKI DJ.Perivascular epithelioid cell neoplasms:pathology and pathogenesis[J].Hum Pathol,2010,41(1):1-15.
[3]SADIGH S,SHAH P,WEBER K,et al.Primary malignant perivascular epithelioid cell neoplasm(PEComa)of the bone mimicking granular cell tumor in core biopsy:A case report and literature review[J].Oncol Lett,2018,15(3):2946-2952.
[4]SALUJA K,THOMAS K,ZHANG S,et al.Malignant perivascular epithelioid cell tumor of the oropharynx with strong TFE3expression mimicking alveolar soft part sarcoma:A case report and review of the literature[J].Hum Pathol,2018,76(1):149-155.
[5]HAIGES D,KURZ P,LAAFF H,et al.Malignant PEComa[J].J Cutan Pathol,2018,45(1):84-89.
[6]PATRA S,VIJ M,KOTA V,et al.Pigmented perivascular epithelioid cell tumor of the liver:Report of a rare case with brief review of literature[J].J Cancer Res Ther,2013,9(2):305-307.
[7]张俊,胡林昆,张学锋,等.肾脏血管周上皮细胞肿瘤临床分析[J].浙江临床医学,2016,18(3):553-554.
[8]FADARE O.Uterine PEComa:Appraisal of a controversial and increasingly reported mesenchymal neoplasm[J].Int Semin Surg Oncol,2008,5:7.
[9]FOLPE AL,MENTZEL T,LEHR HA,et al.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.
[10]RIGB Y H,YU W,SCHMIDT M H,et al.Lack of response of a metastatic renal perivascular epithelial cell tumor(PEComa)to successive courses of DTIC based-therapy and imatinib mesylate[J].Pediatr Blood cancer,2005,45(2):202-206.
[11]PARK SJ,HAN DK,BAEK HJ,et al.Perivascular epithelioid cell tumor(PEComa)of the ascending colon:the implication of IFN-α2b treatment[J].Korean J Pediatr,2010,53(11):975-978.
[12]BISSLER JJ,MCCORMACK FX,YOUNGLR,et al.Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis[J].N Engl JMed,2008,358(2):140-151.
[13]TYNSKI Z,CHIANG W,BARRETT A.An inguinal perivascular epithelioid cell tumor metastatic to the orbit[J].Case Rep Pathol,2018,2018:5749421.
[14]FOLKMAN J.Tumor angiogenesis:therapeutic implications[J].N Engl J Med,1971,285(21):1182-1186.
[15]LI J,ZHAO X,CHEN L,et al.Safety and pharmacokinetics of novel selective vascular endothelial growth factor receptor-2 inhibitor YN968D1 in patients with advanced malignancies[J].BMCCancer,2010,10:529.
[16]傅敬忠,徐冠军,李金高,等.甲磺酸阿帕替尼治疗晚期恶性肿瘤的疗效观察[J].实用癌症杂志,2018,33(2):343-345.
[17]桂秀娟,赵建夫,林绍强,等.阿帕替尼治疗转移性肾细胞癌1例[J].肿瘤学杂志,2018,24(4):409-410.
[18]王金万,孙燕,刘永煜,等.重组人血管内皮抑素联合NP方案治疗晚期NSCLC随机、双盲、对照、多中心Ⅲ期临床研究[J].中国肿瘤杂志,2005,8(4):283-290.
[19]牛牛,李宝兰,刘朝阳,等.重组人内皮抑制素联合贝伐珠单抗体内抑瘤作用的效果及分析[J].中国肺癌杂志,2013,16(2):61-66.
[20]李永强,胡晓桦.唑来磷酸治疗癌性骨痛的临床分析[J].广西医学,2015,27(9):1353-1355.
[21]何斌,蔡雅霜,张志刚,等.双磷酸盐类药物用于肿瘤辅助治疗的临床研究进展[J].中国新药与临床杂志,2018,37(4):185-192.
[22]SHAO C,TIAN G,HUANG Y,et al.Thymosin alpha-1-transformed Bifidobacterium promotes T cell proliferation and maturation in mice by oral administration[J].Int Immunnopharmacol,2013,15(3):646-653.
[23]YANG X,QIAN F,HE HY,et al.Effect of thymosin alpha-1 on subpopulations of Th1,Th2,Th17,and regulatory T cells(Tregs)in vitro[J].Braz J Med Biol Res,2012,45(1):25-32.
[24]LAO X,LIU M,ZHENG H.A tumorpenetrating peptide modification enhances the antitumor activity of thymosin alpha1[J].PLo S One,2013,8(8):e72242.