The use of interval-compressed chemotherapy with the addition of vincristine, irinotecan, and temozolomide for pediatric patients with newly diagnosed desmoplastic small round cell tumor.
The use of interval-compressed chemotherapy with the addition of vincristine, irinotecan, and temozolomide for pediatric patients with newly diagnosed desmoplastic small round cell tumor.
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DOI：
10.1002/pbc.28559
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发表时间：
2020-10
影响因子：
3.2
通讯作者：
Shulman DS
中科院分区：
文献类型：
--
作者：
Liu KX;Collins NB;Greenzang KA;Furutani E;Campbell K;Groves A;Mullen EA;Shusterman S;Spidle J;Marcus KJ;Weil BR;Weldon CB;Frazier AL;Janeway KA;O'Neill AF;Mack JW;DuBois SG;Shulman DS
结缔组织增生小圆细胞瘤（DSRCT）是一种罕见的侵袭性肉瘤，多发于儿童和年轻人，尽管采用了强化的多模式治疗方法，但预后不佳。根据儿童肿瘤组parst08p1，我们报告了在间隔压缩化疗中加入长春新碱、伊立替康和替莫唑胺（VIT）治疗的DSRCT患者的毒性、反应和结果。根据ARST08P1，确定了2014年至2019年期间在丹娜法伯癌症研究所和波士顿儿童医院接受治疗的所有新诊断的DSRCT儿科患者，Arm P2用VIT替代VAC周期。回顾了临床和疾病特征、治疗反应和结果的医疗记录。6例患者按上述方案治疗。诊断时的中位年龄为15.1岁（范围：3.2-16.4岁），5例患者为男性。5例患者为腹部原发肿瘤，其中1例为腹腔内转移，4例为腹外转移。两个初始周期的VIT耐受性良好，恶心、呕吐、腹泻和便秘是最常见的不良事件。在两个初始周期的VIT治疗后，总体缓解率定义为部分或完全缓解为50%。对于局部控制，所有患者均行手术切除后放疗，2例患者在手术时接受高热腹腔化疗。在迄今为止完成治疗的4名患者中，3名患者无病，中位随访时间为46.7个月。在间隔压缩化疗中添加VIT在DSRCT中是可耐受的和有效的，其活性需要进一步的研究。
Desmoplastic small round cell tumor (DSRCT) is a rare aggressive sarcoma that affects children and young adults and portends poor outcomes despite intensive multimodal treatment approaches. We reporttoxicity, response and outcomes of patients with DSRCT treated with the addition of vincristine, irinotecan, and temozolomide (VIT) to interval-compressed chemotherapy as per Children’s Oncology GroupARST08P1. All newly diagnosed pediatric patients with DSRCT treated at Dana-Farber Cancer Institute and Boston Children’s Hospital between 2014 and 2019as per ARST08P1, Arm P2 with replacement of VAC cycles with VIT, were identified.Medical records were reviewed for clinical and disease characteristics, and treatment response and outcomes. Six patients were treated as per the above regimen. Median age at diagnosis was15.1 years (range: 3.2-16.4) and five patients were male. Five patients had abdominal primary tumors, of which one had exclusivelyintra-abdominal and four hadextra-abdominal metastases. Two initial cycles of VIT were well tolerated with nausea, vomiting, diarrhea, and constipation as the most common adverse events. Overall response rate defined as partial or complete response after two initial cycles of VIT was 50%. For local control, all patients had surgical resection followed by radiotherapy, and two patients received hyperthemic intraperitoneal chemotherapy at the time of surgery. Of the four patients who have completed therapy to date, three remain disease-free with median follow-up time of 46.7 months. Theaddition of VIT to interval-compressed chemotherapy is tolerable and active in DSRCT, with activity warranting additional investigation.
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影响因子：
8.4
作者：
Eisenhauer, E. A.;Therasse, P.;Verweij, J.
通讯作者：
Verweij, J.
影响因子：
--
作者：
Frezza, Anna Maria;Benson, Charlotte;Hayward, Larry
通讯作者：
Hayward, Larry
DOI：
10.1016/j.ijrobp.2012.09.005
发表时间：
2013-01-01
影响因子：
7
作者：
Desai, Neil B.;Stein, Nicholas F.;Wolden, Suzanne L.
通讯作者：
Wolden, Suzanne L.
影响因子：
6.2
作者：
Navid, F;Santana, VM;Pappo, AS
通讯作者：
Pappo, AS
影响因子：
0.9
作者：
Bent, Melissa A.;Padilla, Benjamin E.;DuBois, Steven G.
通讯作者：
DuBois, Steven G.