Browse > Article
http://dx.doi.org/10.3904/kjm.2012.83.3.378

Desmoplastic Small Round Cell Tumor Achieved Complete Remission after High-Dose Chemotherapy with Aggressive Local Treatment Modalities  

Kim, Sun-Mok (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine)
Park, Ji-Hyun (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine)
Kim, Jung-Seok (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine)
Kim, Min-Soo (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine)
Jang, Jung-Eun (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine)
Hwang, Hee-Sang (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine)
Suh, Cheol-Won (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine)
Publication Information
The Korean Journal of Medicine / v.83, no.3, 2012 , pp. 378-384 More about this Journal
Abstract
A desmoplastic small-round-cell tumor (DSRCT) is a rare, aggressive neoplasm that develops mostly in the abdominal cavity in children and young adults. We present a case of a 19-year-old male with right upper quadrant discomfort for 3 months. On abdominal computerized tomography, multiple huge and demarcated masses were found in the liver, retroperitoneal lymph nodes, and peritoneal and retroperitoneal cavities. Fine needle aspiration biopsy of the hepatic mass was performed and DSRCT was diagnosed by hematoxylin and eosin staining and immunohistochemical analysis. He was treated initially with high-dose systemic chemotherapy (alternating schedules of cyclophosphamide, vincristine, doxorubicin, ifosfamide, and etoposide), underwent two debulking surgeries and pelvic irradiation between systemic chemotherapy schedules, and achieved complete remission after the 15 months of treatment duration. We report this case to emphasize the importance of aggressive local treatment modalities as well as high-dose systemic chemotherapy for treatment of DSRCT even with initially unresectable or extensively metastatic presentation.
Keywords
Chemotherapy; Desmoplastic small-round-cell tumor; Radiotherapy; Surgery;
Citations & Related Records
연도 인용수 순위
  • Reference
1 Koniari K, Mahera H, Nikolaou M, et al. Intraabdominal desmoplastic small round cell tumor: report of a case and literature review. Int J Surg Case Rep 2011;2:293-296.   DOI   ScienceOn
2 Kolb EA, Kushner BH, Gorlick R, et al. Long-term eventfree survival after intensive chemotherapy for Ewing's family of tumors in children and young adults. J Clin Oncol 2003;21:3423-3430.   DOI   ScienceOn
3 Gerald WL, Rosai J. Case 2: desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 1989;9:177-183.   DOI
4 Kretschmar CS, Colbach C, Bhan I, Crombleholme TM. Desmoplastic small cell tumor: a report of three cases and a review of the literature. J Pediatr Hematol Oncol 1996;18: 293-298.   DOI   ScienceOn
5 Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J. Intra-abdominal desmoplastic small round-cell tumor: report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol 1991;15:499-513.   DOI   ScienceOn
6 Sawyer JR, Tryka AF, Lewis JM. A novel reciprocal chromosome translocation t(11;22)(p13;q12) in an intraabdominal desmoplastic small round-cell tumor. Am J Surg Pathol 1992;16:411-416.   DOI   ScienceOn
7 Glehen O, Kwiatkowski F, Sugarbaker PH, et al. Cytoreductive surgery combined with perioperative intraperitoneal chemotherapy for the management of peritoneal carcinomatosis from colorectal cancer: a multi-institutional study. J Clin Oncol 2004;22:3284-3292.   DOI   ScienceOn
8 Kushner BH, LaQuaglia MP, Wollner N, et al. Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol 1996; 14:1526-1531.
9 Goodman KA, Wolden SL, La Quaglia MP, Kushner BH. Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor. Int J Radiat Oncol Biol Phys 2002;54: 170-176.
10 Hayes-Jordan A, Anderson PM. The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol 2011;23:385-389.   DOI   ScienceOn