Retroperitoneal desmoplastic small round cell tumor: pediatric patient treated with multimodal therapy.
A desmoplastic small round cell tumor (DSRCT) is a rare, aggressive mesenchymal neoplasm. Although a DSRCT can develop at various sites, the intra-abdominal site is the most common location. These tumors are found most commonly among young adolescents and the prognosis is extremely poor. Multimodal treatment with surgery, chemotherapy and radiotherapy is very important for these rare cases, and this treatment can improve patient survival. In this report, we describe the case of an 8-year-old boy diagnosed with DSRCT located in the retroperitoneal space. The patient has undergone surgical resection and adjuvant chemoradiation therapy, and is currently alive without disease recurrence.
Kim
Yong Seok
YS
Department of Surgery, College of Medicine, Chung-Ang University, 224-1, Heuk Seok-Dong, Dongjak-Ku, Seoul 156-755, South Korea.
Cha
Seong Jae
SJ
Choi
Yoo Shin
YS
Kim
Beom Gyu
BG
Park
Sung Jun
SJ
Chang
In Taik
IT
D018288:Carcinoma, Small Cell / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D002648:Child; D003131:Combined Modality Therapy; D006801:Humans; D008297:Male; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*