Desmoplastic small round-cell tumor: Prolonged progression-free survival with aggressive multimodality therapy Journal Article


Authors: Kushner, B. H.; LaQuaglia, M. P.; Wollner, N.; Meyers, P. A.; Lindsley, K. L.; Ghavimi, F.; Merchant, T. E.; Boulad, F.; Cheung, N. K. V.; Bonilla, M. A.; Crouch, G.; Kelleher, J. F. Jr; Steinherz, P. G.; Gerald, W. L.
Article Title: Desmoplastic small round-cell tumor: Prolonged progression-free survival with aggressive multimodality therapy
Abstract: Purpose: To test intensive alkylator-based therapy in desmoplastic small round-cell tumor (DSRCT). Patients and Methods: Patients received the P6 protocol, which has seven courses of chemotherapy. Courses 1, 2, 3, and 6 included cyclophosphamide 4,200 mg/m2, doxorubicin 75 mg/m2, and vincristine (HD-CAV). Courses 4, 5, and 7 consisted of ifosfamide 9 g/m2 and etoposide 500 mg/m2 for previously untreated patients, or ifosfamide 12 g/m2 and etoposide 1,000 mg/m2 for previously treated patients. Courses started after neutrophil counts reached 500/μL and platelet counts reached 100,000/μL. Tumor resection was attempted. Post-P6 treatment options included radiotherapy and a myeloablative regimen of thiotepa (900 mg/m2) plus carboplatin (1,500 mg/m2), with stem-cell rescue. Results: Ten previously untreated and two previously treated patients have completed therapy. The male-to-female ratio was 11:1. Ages were 7 to 22 years (median, 14). The largest masses were infradiaphragmatic (n = 11) or intrathoracic (n = 1). Other findings included serosal implants (n = 11), regional lymph node invasion (n = 8), ascites or pleural effusion (n = 7), and metastases to liver (n = 5), lungs (n = 4), distant lymph nodes (n = 3), spleen(n = 2), and skeleton (n = 2). Tumors uniformly responded to HD-CAV, but there were no complete pathologic responses. One patient died at 1 month from tumor- related Budd-Chiari syndrome. Of seven patients who achieved a complete remission (CR), five remain in CR 9, 12, 13, 33, and 38 months from the start of P6, one patient died of infection at 12 months (autopsy-confirmed CR), and one patient relapsed 4 months off therapy. Of four patients who achieved a partial remission (PR), one remains progression-free at 34 months and three developed progressive disease. Five patients received local radiotherapy: three were not assessable for response, but in two patients, antitumor effect was evident. Four patients received thiotepa/carboplatin: two were in CR and remain so, and two patients had measurable disease that did not respond. Conclusion: For control of DSRCT, our experience supports intensive use of HD-CAV, aggressive surgery to resect visible disease, radiotherapy to high- risk sites, and myeloablative chemotherapy with stem-cell rescue in selected cases.
Keywords: adolescent; adult; cancer survival; child; clinical article; school child; survival analysis; clinical trial; doxorubicin; cancer combination chemotherapy; multimodality cancer therapy; cancer radiotherapy; combined modality therapy; prospective studies; tumor localization; carboplatin; etoposide; antineoplastic combined chemotherapy protocols; cyclophosphamide; vincristine; ifosfamide; thiotepa; gene rearrangement; pelvis tumor; abdominal mass; tumor classification; intravenous drug administration; abdominal neoplasms; humans; human; male; female; priority journal; article
Journal Title: Journal of Clinical Oncology
Volume: 14
Issue: 5
ISSN: 0732-183X
Publisher: American Society of Clinical Oncology  
Date Published: 1996-05-01
Start Page: 1526
End Page: 1531
Language: English
PUBMED: 8622067
PROVIDER: scopus
DOI: 10.1200/JCO.1996.14.5.1526
DOI/URL:
Notes: Article -- Export Date: 22 November 2017 -- Source: Scopus
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MSK Authors
  1. Brian Kushner
    190 Kushner
  2. Farid Boulad
    251 Boulad
  3. Nai-Kong Cheung
    439 Cheung
  4. William L Gerald
    367 Gerald
  5. Peter G Steinherz
    164 Steinherz
  6. Paul Meyers
    244 Meyers
  7. Fereshteh   Ghavimi
    14 Ghavimi