Retroperitoneal infantile fibrosarcoma: Clinical, molecular, and therapeutic aspects of an unusual tumor

被引:13
|
作者
Ramphal, R
Manson, D
Viero, S
Zielenska, M
Gerstle, T
Pappo, A
机构
[1] Hosp Sick Children, Div Hematol Oncol, Dept Pediat, Toronto, ON M5G 1X8, Canada
[2] Hosp Sick Children, Dept Pediat, Div Radiol, Toronto, ON M5G 1X8, Canada
[3] Hosp Sick Children, Dept Pediat Lab Med, Div Pathol, Toronto, ON M5G 1X8, Canada
[4] Hosp Sick Children, Dept Pediat Lab Med, Div Mol Diagnost, Toronto, ON M5G 1X8, Canada
[5] Hosp Sick Children, Dept Pediat Surg, Div Gen Surg, Toronto, ON M5G 1X8, Canada
[6] Univ Toronto, Dept Lab Med & Pathobiol, Toronto, ON, Canada
关键词
chemotherapy; infantile fibrosarcoma; nodal metastases; retroperitoneal fibrosarcoma; t(12; 15) translocation;
D O I
10.1080/08880010390243077
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
The authors describe a patient with a large retroperitoneal infantile fibrosarcoma that responded well to preoperative chemotherapy, which subsequently facilitated the complete surgical resection of the mass. The patient had an unusual site of metastasis presumed to be to a regional lymph node. The histology on initial core biopsies was not classic but showed a round cell, myxoid pattern. The presence of the t(12;15)(p13;q25) and the fusion transcript ETV6-NTRK3 by RTPCR facilitated the diagnosis.
引用
收藏
页码:635 / 642
页数:8
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