Ambar Natalia Brenneken Duarte, de Seixas Alves Maria Teresa, Lederman Henrique Manoel, Abib Simone, Duarte Alexandre Alberto Barros, Caran Eliana Monteiro
Instituto de Oncologia Pediátrica - UNIFESP, Sao Paulo, Brazil.
J Med Case Rep. 2019 Mar 10;13(1):77. doi: 10.1186/s13256-019-1985-z.
Desmoplastic small round cell tumor is an extremely rare and aggressive cancer that affects mainly adolescents and young adults. Despite multiple therapeutic strategies, most patients have resistant disease with very poor survival rates.
We present a case of a 10-year-old Caucasian boy with a desmoplastic small round cell tumor refractory to conventional treatment who exhibited a good response to alternative treatment. With use of irinotecan and vincristine in association with radiation therapy, a reduction of 96.9% of the dimensions of the target lesions compared with the initial image was observed.
This chemotherapy regimen, in association with radiation therapy, demonstrated efficacy for refractory desmoplastic small round cell tumor in our patient, and it is cost-effective.
促结缔组织增生性小圆细胞肿瘤是一种极其罕见且侵袭性强的癌症，主要影响青少年和年轻成年人。尽管有多种治疗策略，但大多数患者的疾病具有耐药性，生存率极低。
我们报告一例10岁的白种男孩，患有对传统治疗耐药的促结缔组织增生性小圆细胞肿瘤，对替代治疗表现出良好反应。使用伊立替康和长春新碱联合放射治疗后，与初始图像相比，目标病灶尺寸缩小了96.9%。
这种化疗方案联合放射治疗，在我们的患者中显示出对难治性促结缔组织增生性小圆细胞肿瘤有效，且具有成本效益。
