Abstract
A case is described of a 19-year-old man with poor prognosis intra-abdominal desmoplastic small round cell tumor, who achieved a durable complete remission after aggressive multimodality treatment. He was diagnosed with a large peri-rectal mass and bulky metastases to the peritoneum, omentum and liver. He achieved a partial response to six courses of chemotherapy with VAC (vincristine, adriamycin, actinomycin, cyclophos- phamide) and VIP (VP-16, ifosfamide, cisplatin). This was followed by resection of the omentum and cryotherapy to six of the liver lesions. He then achieved a complete response following high-dose busulfan 16 mg/kg and melphalan 140 mg/m2 with autologous stem cell support. Finally, pelvic radiotherapy was given to consolidate the remission. Twenty-six months following his diagnosis, and 19 months after the transplant, he remains well with no clinical or radiologic evidence of recurrent disease. This case report suggests a role for autologous stem cell transplantation as part of multimodality therapy for this unusual and aggressive malignancy.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$259.00 per year
only $21.58 per issue
Buy this article
- Purchase on SpringerLink
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mazuryk, M., Paterson, A., Temple, W. et al. Benefit of aggressive multimodality therapy with autologous stem cell support for intra-abdominal desmoplastic small round cell tumor. Bone Marrow Transplant 21, 961–963 (1998). https://doi.org/10.1038/sj.bmt.1701220
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1038/sj.bmt.1701220
Keywords
This article is cited by
-
Clinical outcomes of patients with desmoplastic small round cell tumor of the peritoneum undergoing autologous HCT: a CIBMTR retrospective analysis
Bone Marrow Transplantation (2012)
-
Desmoplastic Small Round Cell Tumor-Clinicopathological Spectrum, Including Unusual Features and Immunohistochemical Analysis of 45 Tumors Diagnosed at a Tertiary Cancer Referral Centre, with Molecular Results t(11; 22) (p13; q12) (EWS-WT1) in Select Cases
Pathology & Oncology Research (2012)
-
Metastatic Desmoplastic Small Round Cell Tumor Controlled by an Anthracycline-Based Regimen: Review of the Role of Chemotherapy
Journal of Gastrointestinal Cancer (2012)
-
Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor
Bone Marrow Transplantation (2010)
-
Androgen and c-Kit receptors in desmoplastic small round cell tumors resistant to chemotherapy: novel targets for therapy
Cancer Chemotherapy and Pharmacology (2007)