Desmoplastic small round cell tumor: A case report

被引:0
作者
Diaz, Carmen Luisa Briceno [1 ,3 ]
Scalia, Alejandra Elena Boada [1 ]
Gonzalez, William David Moreno [1 ]
Montanez, Jose Ruben Bracho [1 ]
Meza, Marco Antonio Gil [2 ]
机构
[1] Univ Los Andes, Fac Med, Merida, Venezuela
[2] Univ Los Andes, Serv Pediat & Puericultura, Merida, Venezuela
[3] Univ Los Andes, Merida, Venezuela
关键词
Desmoplastic small round cell tumor; peritoneal tumor; immunohistochemistry;
D O I
暂无
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm, with a poor prognosis, that usually affects male teenagers and young adults. The pathophysiological mechanisms are not well understood, however, the formation of a fused gene called EWSR1-WT1 has been found. Diagnosis is made by biopsy of the tumor and immunohistochemical marker tests. Treatment requires chemotherapy, radiotherapy and surgical resection. This article reports the case of a male adolescent diagnosed with this unusual pathology.
引用
收藏
页码:70 / 75
页数:6
相关论文
共 17 条
[1]  
[Anonymous], 2018, What is a rare disease?
[2]  
[Anonymous], GENETIC RARE DIS INF
[3]  
Arias LR, 2020, Rev. Colomb. Radiol., V31, P5469
[4]  
Butt SUR, 2017, J GLOB ONCOL, V3, P79, DOI 10.1200/JGO.2016.006163
[5]   The Small Round Cell Sarcomas Complexities and Desmoplastic Presentation [J].
Domanski, Henryk A. .
ACTA CYTOLOGICA, 2022, 66 (04) :279-294
[6]   Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report [J].
Duarte Ambar, Natalia Brenneken ;
de Seixas Alves, Maria Teresa ;
Lederman, Henrique Manoel ;
Abib, Simone ;
Barros Duarte, Alexandre Alberto ;
Caran, Eliana Monteiro .
JOURNAL OF MEDICAL CASE REPORTS, 2019, 13 (01)
[7]   Management of desmoplastic small round cell [J].
Hayes-Jordan, Andrea ;
LaQuaglia, Michael P. ;
Modak, Shakeel .
SEMINARS IN PEDIATRIC SURGERY, 2016, 25 (05) :299-304
[8]   Desmoplastic Small Round Cell Tumors: A review with focus on clinical management and therapeutic options [J].
Hendricks, Anne ;
Boerner, Kevin ;
Germer, Christoph-Thomas ;
Wiegering, Armin .
CANCER TREATMENT REVIEWS, 2021, 93
[9]   Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? Results of a retrospective multicentric study on 100 patients [J].
Honore, C. ;
Delhorme, J. B. ;
Nassif, E. ;
Faron, M. ;
Ferron, G. ;
Bompas, E. ;
Glehen, O. ;
Italiano, A. ;
Bertucci, F. ;
Orbach, D. ;
Pocard, M. ;
Quenet, F. ;
Blay, J. Y. ;
Carrere, S. ;
Chevreau, C. ;
Mir, O. ;
Le Cesne, A. .
SURGICAL ONCOLOGY-OXFORD, 2019, 29 :107-112
[10]   Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database [J].
Lettieri, Christina K. ;
Garcia-Filion, Pamela ;
Hingorani, Pooja .
JOURNAL OF CANCER EPIDEMIOLOGY, 2014, 2014