促结缔组织增生性小圆细胞瘤诊疗进展

摘要/Abstract

摘要：促结缔组织增生性小圆细胞瘤（DSRCT）是一种高度恶性、预后极差的罕见恶性软组织肿瘤，主要发生于腹、盆腔，好发于青少年男性，易发生肝、肺等远处转移；病理组织学表现为小圆蓝细胞巢被促结缔组织间质包绕分隔，共表达上皮源性、神经源性及间质源性标志；分子特征为t（11；22）（p13；q12）染色体易位，EWSWT1基因融合。主要治疗手段包括根治性切除或最大限度减瘤术、高强度全身化疗、局部放疗及腹腔热灌注化疗。

关键词:诊断,治疗,促结缔组织增生性小圆细胞瘤

Abstract:Desmoplastic small round cell tumor (DSRCT) is a rare and high malignant soft tissue tumor with very poor prognosis. It usually occurs in the abdominopelvic cavity of adolescents and young males. DSRCT is prone to occur distant metastasis, mainly in the liver and lung. The histopathological manifestation is featured with nests of small round blue cells separated by desmoplastic stroma. DSRCT can coexpress epithelial, neural and mesenchymal markers. The molecular characteristic of DSRCT is the production of EWS-WT1 fusion protein via the translocation of chromosome t (11; 22) (p13; q12). Treatments of DSRCT include radical resection or cytoreductive surgery, high intensity systemic chemotherapy, local radiotherapy and hyperthermic intraperitoneal chemotherapy.

Key words:Diagnosis,Therapy,Desmoplastic small round cell tumor

陈火光，王鸿彪，林英城. 促结缔组织增生性小圆细胞瘤诊疗进展[J]. 国际肿瘤学杂志, 2018, 45(6): 379-382.

Chen Huoguang, Wang Hongbiao, Lin Yingcheng. Advances of diagnosis and therapy for desmoplastic small round cell tumor[J]. Journal of International Oncology, 2018, 45(6): 379-382.

参考文献

［1］ Lettieri CK, GarciaFilion P, Hingorani P. Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database［J］. J Cancer Epidemiol, 2014, 2014: 680126. DOI: 10.1155/2014/680126. ［2］ Bent MA, Padilla BE, Goldsby RE, et al. Clinical characteristics and outcomes of pediatric patients with desmoplastic small round cell tumor［J］. Rare Tumors, 2016, 8(1): 6145. DOI: 10.4081/rt.2016.6145. ［3］ Angarita FA, Hassan S, Cannell AJ, et al. Clinical features and outcomes of 20 patients with abdominopelvic desmoplastic small round cell tumor［J］. Eur J Surg Oncol, 2017, 43(2): 423-431. DOI: 10.1016/j.ejso.2016.08.017. ［4］ Shen XZ, Zhao JG, Wu JJ, et al. Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor［J］. World J Gastroenterol, 2014, 20(17): 5157-5164. DOI: 10.3748/wjg.v20.i17.5157. ［5］ HayesJordan A, LaQuaglia MP, Modak S. Management of desmoplastic small round cell tumor［J］. Semin Pediatr Surg, 2016, 25(5): 299304. DOI: 10.1053/j.sempedsurg.2016.09.005. ［6］ Negri T, Brich S, Bozzi F, et al. New transcriptionalbased insights into the pathogenesis of desmoplastic small round cell tumors (DSRCTs)［J］. Oncotarget, 2017, 8(20): 32492-32504. DOI: 10.18632/oncotarget.16477. ［7］ Honoré C, Atallah V, Mir O, et al. Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: is there a benefit for HIPEC after macroscopically complete cytoreductive surgery?［J］. PLoS One, 2017, 12(2): e0171639. DOI: 10.1371/journal.pone.0171639. ［8］ Zhang S, Zhang Y, Yu YH, et al. Results of multimodal treatment for desmoplastic small round cell tumor of the abdomen and pelvis［J］. Int J Clin Exp Med, 2015, 8(6): 96589666. ［9］ HayesJordan A, Green H, Lin H, et al. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) for children, adolescents, and young adults: the first 50 cases［J］. Ann Surg Oncol, 2015, 22(5): 1726-1732. DOI: 10.1245/s10434-014-4289-y. ［10］ Wong HH, Hatcher HM, Benson C, et al. Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature［J］. Clin Sarcoma Res, 2013, 3(1): 14. DOI: 10.1186/20453329-3-14. ［11］ Bulbul A, Fahy BN, Xiu J, et al. Desmoplastic small round blue cell tumor: a review of treatment and potential therapeutic genomic alterations［J］. Sarcoma, 2017, 2017: 1278268. DOI: 10.1155/2017/1278268. ［12］ HayesJordan A, Green HL, Lin H, et al. Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor［J］. Ann Surg Oncol, 2014, 21(1): 220-224. DOI: 10.1245/s10434-013-3269-y. ［13］ Atallah V, Honore C, Orbach D, et al. Role of adjuvant radiation therapy after surgery for abdominal desmoplastic small round cell tumors［J］. Int J Radiat Oncol Biol Phys, 2016, 95(4): 12441253. DOI: 10.1016/j.ijrobp.2016.02.046. ［14］ Desai NB, Stein NF, LaQuaglia MP, et al. Reduced toxicity with intensity modulated radiation therapy (IMRT) for desmoplastic small round cell tumor (DSRCT): an update on the whole abdominopelvic radiation therapy (WAPRT) experience［J］. Int J Radiat Oncol Biol Phys, 2013, 85(1): e67-72. DOI: 10.1016/j.ijrobp.2012.09.005. ［15］ Frezza AM, Benson C, Judson IR, et al. Pazopanib in advanced desmoplastic small round cell tumours: a multiinstitutional experience［J］. Clin Sarcoma Res, 2014, 4: 7. DOI: 10.1186/2045-3329-4-7. ［16］ Menegaz BA, Cuglievan B, Benson J, et al. Clinical activity of pazopanib in patients with advanced desmoplastic small round cell tumor［J］. Oncologist, 2018, 23(3): 360-366. DOI: 10.1634/theoncologist.20170408. ［17］ Italiano A, Kind M, Cioffi A, et al. Clinical activity of sunitinib in patients with advanced desmoplastic round cell tumor: a case series［J］. Target Oncol, 2013, 8(3): 211-213. DOI: 10.1007/s1152301202518. ［18］ De Sanctis R, Bertuzzi A, Bisogno G, et al. Imatinib mesylate in desmoplastic small round cell tumors［J］. Future Oncol, 2017, 13(14): 1233-1237. DOI: 10.2217/fon-2016-0305. ［19］ Tap WD, Demetri G, Barnette P, et al. Phase Ⅱ study of ganitumab, a fully human antitype1 insulinlike growth factor receptor antibody, in patients with metastatic Ewing family tumors or desmoplastic small round cell tumors［J］. J Clin Oncol, 2012, 30(15): 1849-1856. DOI: 10.1200/JCO.2011.37.2359. ［20］ Subbiah V, Brown RE, Jiang Y, et al. Morphoproteomic profiling of the mammalian target of rapamycin (mTOR) signaling pathway in desmoplastic small round cell tumor (EWS/WT1), Ewing′s sarcoma (EWS/FLI1) and Wilms′ tumor (WT1)［J］. PLoS One, 2013, 8(7): e68985. DOI: 10.1371/journal.pone.0068985. ［21］ Tarek N, HayesJordan A, Salvador L, et al. Recurrent desmoplastic small round cell tumor responding to an mTOR inhibitor containing regimen［J］. Pediatr Blood Cancer, 2018, 65(1). DOI: 10.1002/pbc.26768.

[1]	刘娜, 寇介丽, 杨枫, 刘桃桃, 李丹萍, 韩君蕊, 杨立洲.血清miR-106b-5p、miR-760联合低剂量螺旋CT诊断早期肺癌的临床价值[J]. 国际肿瘤学杂志, 2024, 51(6): 321-325.
[2]	钱晓涛, 石子宜, 胡格, 吴晓维.Ⅲ~ⅣA期食管鳞状细胞癌放化疗后行巩固化疗的疗效：一项真实世界临床研究[J]. 国际肿瘤学杂志, 2024, 51(6): 326-331.
[3]	杨蜜, 别俊, 张加勇, 邓佳秀, 唐组阁, 卢俊.局部晚期可切除食管癌新辅助治疗疗效及预后分析[J]. 国际肿瘤学杂志, 2024, 51(6): 332-337.
[4]	袁健, 黄燕华.Hp-IgG抗体联合血清DKK1、sB7-H3对早期胃癌的诊断价值[J]. 国际肿瘤学杂志, 2024, 51(6): 338-343.
[5]	张百红, 岳红云.新作用机制的抗肿瘤药物进展[J]. 国际肿瘤学杂志, 2024, 51(6): 354-358.
[6]	许凤琳, 吴刚.EBV在鼻咽癌肿瘤免疫微环境和免疫治疗中的研究进展[J]. 国际肿瘤学杂志, 2024, 51(6): 359-363.
[7]	王盈, 刘楠, 郭兵.抗体药物偶联物在转移性乳腺癌治疗中的研究进展[J]. 国际肿瘤学杂志, 2024, 51(6): 364-369.
[8]	张宁宁, 杨哲, 檀丽梅, 李振宁, 王迪, 魏永志.宫颈细胞DNA倍体分析联合B7-H4和PKCδ对宫颈癌的诊断价值[J]. 国际肿瘤学杂志, 2024, 51(5): 286-291.
[9]	陈琦, 徐晨阳, 王寅, 雷大鹏.高光谱成像在头颈部肿瘤诊疗中的应用现状[J]. 国际肿瘤学杂志, 2024, 51(5): 298-302.
[10]	王培鑫, 赵军, 徐世红, 姜朝阳, 王小强, 杨红娟.铁死亡相关机制在骨肉瘤中的应用进展[J]. 国际肿瘤学杂志, 2024, 51(5): 308-311.
[11]	王昆, 周中新, 臧其威.血清TGF-β1、VEGF水平对非小细胞肺癌患者单孔胸腔镜根治术后复发的预测价值[J]. 国际肿瘤学杂志, 2024, 51(4): 198-203.
[12]	萨蔷, 徐航程, 王佳玉.乳腺癌免疫治疗研究进展[J]. 国际肿瘤学杂志, 2024, 51(4): 227-234.
[13]	杨智, 陆以乔, 顾花艳, 丁佳玲, 郭贵龙.肿瘤微环境介导乳腺癌靶向治疗耐药的研究进展[J]. 国际肿瘤学杂志, 2024, 51(4): 235-238.
[14]	王子豪, 王宇, 杨鑫, 何艺, 莫兴奎, 袁涛.铁死亡在骨肉瘤中的分子机制及相关治疗的研究进展[J]. 国际肿瘤学杂志, 2024, 51(4): 239-244.
[15]	张栋岩, 王品, 魏秋亚, 邓成伍, 魏相相, 高远飞, 王琛.索凡替尼靶向联合卡培他滨和奥沙利铂治疗肝内胆管癌术后患者1例及文献复习[J]. 国际肿瘤学杂志, 2024, 51(4): 249-253.