Multimodal Therapy Including Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Can Result in Long-term Disease-free Survival in Pediatric Desmoplastic Small Round Cell Tumor With Extraperitoneal Disease. J Pediatr Hematol Oncol 2021 Aug 01;43(6):228-231
Date
06/20/2020Pubmed ID
32555030DOI
10.1097/MPH.0000000000001861Scopus ID
2-s2.0-85086724925 (requires institutional sign-in at Scopus site) 1 CitationAbstract
Desmoplastic small round cell tumor is a rare sarcoma with 5-year overall survival of 15%. An 8-year-old female presented with diffuse abdominal/pelvic desmoplastic small round cell tumor including numerous liver metastasis. She underwent neoadjuvant chemotherapy followed by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Residual disease was found shortly after CRS/HIPEC which was resected, followed by whole abdomen/pelvic radiation and autologous hematopoietic cell transplant. Previous papers have reported dismal survival in patients with liver metastasis and residual disease arguing against CRS/HIPEC. Our patient remains disease-free over 6 years after completing therapy indicating long-term survival is achievable with aggressive multimodal therapy.
Author List
Xiao J, Browning MB, Boyd KP, Suchi M, Turaga KK, Firat SY, Mortland LJ, Lal DRAuthors
Selim Firat MD Professor in the Radiation Oncology department at Medical College of WisconsinDave Lal MD, MPH Chief, Professor in the Surgery department at Medical College of Wisconsin
Mariko Suchi MD, PhD Associate Professor in the Pathology department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
ChildCombined Modality Therapy
Cytoreduction Surgical Procedures
Desmoplastic Small Round Cell Tumor
Disease-Free Survival
Female
Humans
Peritoneal Neoplasms
