Abstract | BACKGROUND: With nearly 450 cases reported since 1991, desmoplastic small round cell tumor (DSRCT) is a rare abdominal tumor typically arising in adolescent and young adult white men. With no large series described, the best therapeutic strategy remains unclear. METHODS: All consecutive patients treated in our tertiary care center between January 1991 and December 2013 for a DSRCT were retrospectively studied. RESULTS: Thirty-eight patients with a median age of 27 years (range 13-57 years) were identified; 71 % were men. At the time of diagnosis, 47.4 % patients had extraperitoneal metastases (EPM): 78 % were located in the liver and 11 % were located in the lungs. Fourteen patients (37 %) were treated exclusively with systemic chemotherapy, with a median survival of 21.1 months. Twenty-three patients underwent surgery, 12 (52 %) experienced complete removal of all macroscopic disease, 5 (21.7 %) received additional intraperitoneal chemotherapy, and 7 (30 %) received postoperative whole abdominopelvic radiotherapy (WAP RT). With a median follow-up of 59.9 months, the median survival was 37.7 months, and the median disease-free survival was 15.5 months. The factors predictive of 3-year overall survival were the absence of EPM, complete surgical resection, postoperative WAP RT, and postoperative chemotherapy. The intraperitoneal chemotherapy had no impact on overall survival. CONCLUSIONS:
|
Authors | Charles Honoré, Koceila Amroun, Laurence Vilcot, Olivier Mir, Julien Domont, Philippe Terrier, Axel Le Cesne, Cecile Le Péchoux, Sylvie Bonvalot |
Journal | Annals of surgical oncology
(Ann Surg Oncol)
Vol. 22
Issue 4
Pg. 1073-9
(Apr 2015)
ISSN: 1534-4681 [Electronic] United States |
PMID | 25300608
(Publication Type: Journal Article)
|
Topics |
- Abdominal Neoplasms
(mortality, pathology, therapy)
- Adolescent
- Adult
- Antineoplastic Combined Chemotherapy Protocols
(therapeutic use)
- Combined Modality Therapy
- Desmoplastic Small Round Cell Tumor
(mortality, pathology, therapy)
- Female
- Follow-Up Studies
- Humans
- Hypothermia, Induced
- Liver Neoplasms
(mortality, secondary, therapy)
- Lung Neoplasms
(mortality, secondary, therapy)
- Lymphatic Metastasis
- Male
- Middle Aged
- Neoplasm Recurrence, Local
(mortality, pathology, therapy)
- Neoplasm Staging
- Peritoneal Neoplasms
(mortality, secondary, therapy)
- Prognosis
- Retrospective Studies
- Survival Rate
- Tertiary Care Centers
- Young Adult
|