Open AccessThe addition of autologous stem cell transplantation to neoadjuvant chemotherapy, radiation, and HIPEC for patients with unresectable desmoplastic small round cell tumor: a single center case series
Author(s) -
Amir Siddiqui,
Navin Pinto,
Mark A. Applebaum,
Grace Z. Mak,
John M. Cunningham,
James L. LaBelle,
Michele L. Nassin
Publication year - 2020
Publication title -
international journal of surgery. oncology
Language(s) - English
Resource type - Journals
ISSN - 2471-3864
DOI - 10.1097/ij9.0000000000000095
Subject(s) - desmoplastic small round cell tumor , debulking , medicine , chemotherapy , melphalan , hematopoietic stem cell transplantation , transplantation , radiation therapy , autologous stem cell transplantation , surgery , oncology , cancer , ovarian cancer
Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy primarily affecting children and young adults. Although modest improvements have been gained by intensification of chemotherapy and radiation, survival of patients with DSRCT remains poor, particularly in those with unresectable or disseminated disease. We report 3 pediatric patients who were treated with a combination of therapy including chemotherapy, surgical debulking, hyperthermic intraperitoneal chemotherapy, whole abdominal irradiation, and autologous hematopoietic stem cell transplantation following busulfan and melphalan conditioning. We find that this approach is well tolerated and may offer improved survival in patients with DSRCT.