Results of multimodal treatment for desmoplastic small round cell tumors Journal Article
| Authors: | Lal, D. R.; Su, W. T.; Wolden, S. L.; Loh, K. C.; Modak, S.; La Quaglia, M. P. |
| Article Title: | Results of multimodal treatment for desmoplastic small round cell tumors |
| Abstract: | Desmoplastic small round cell tumors (DSRCTs) are rare aggressive neoplasms that frequently present with large symptomatic intraabdominal masses. We examined the effects of multimodal therapy including induction chemotherapy, aggressive surgical debulking, and external beam radiotherapy on patients with DSRCT. Institutional Review Board permission was obtained. Sixty-six patients were diagnosed by histology, immunohistochemistry, and or cytogenetics as having DSRCT at our institution from July 1, 1972, to July 1, 2003. Data were collected on patient demographics, presenting symptoms, tumor location and extent, treatment regimen, and overall survival. A majority of patients were male (91%), Caucasian (85%), and with a median age of 19 (7-58) years old at diagnosis. The most common presenting complaint was an intraabdominal mass (64%). In 63 patients (96%), the primary tumor was located in the abdomen or pelvis. Thirty-three (50%) had positive lymph nodes and 27 (41%) had distant parenchymal metastases at diagnosis. Overall, 3- and 5-year survivals were 44% and 15%, respectively. Twenty-nine of these patients (44%) underwent induction chemotherapy (P6), surgical debulking, and radiotherapy. Three-year survival was 55% in those receiving chemotherapy, surgery, and radiotherapy vs 27% when all 3 modalities were not used (P <. 02). Gross tumor resection was highly significant in prolonging overall survival; 3-year survival was 58% in patients treated with gross tumor resection compared to no survivors past 3 years in the nonresection cohort (P <. 000 01). Ten patients (15%) have no evidence of disease with a median follow-up of 2.4 years (range, 0.4-11.2 years). Multimodal therapy results in improved survival in patients with DSRCT. Aggressive surgical resection of these extensive intraabdominal neoplasms correlates with improved patient outcome. © 2005 Elsevier Inc. All rights reserved. |
| Keywords: | immunohistochemistry; survival; cancer chemotherapy; cancer survival; controlled study; human tissue; cancer surgery; major clinical study; clinical trial; cancer localization; cisplatin; doxorubicin; multimodality cancer therapy; cancer radiotherapy; chemotherapy; topotecan; follow up; lymph node metastasis; antineoplastic agent; demography; carboplatin; controlled clinical trial; etoposide; cohort analysis; cytogenetics; cyclophosphamide; vincristine; stem cell transplantation; histology; ifosfamide; thiotepa; irinotecan; symptom; surgery; outcomes research; radiation therapy; resection; abdominal mass; drug dose regimen; pelvis cancer; caucasian; clinical examination; desmoplastic small round cell tumor; autologous bone marrow transplantation; parenchyma; abdominal cancer; beam therapy |
| Journal Title: | Journal of Pediatric Surgery |
| Volume: | 40 |
| Issue: | 1 |
| ISSN: | 0022-3468 |
| Publisher: | W.B. Saunders Co-Elsevier Inc. |
| Date Published: | 2005-01-01 |
| Start Page: | 251 |
| End Page: | 255 |
| Language: | English |
| DOI: | 10.1016/j.jpedsurg.2004.09.046 |
| PROVIDER: | scopus |
| PUBMED: | 15868593 |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 60" - "Export Date: 24 October 2012" - "CODEN: JPDSA" - "Source: Scopus" |
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