Abstract
BACKGROUND.
Extracranial malignant rhabdoid tumor (MRT) is a rare, aggressive, pediatric malignancy with a historically poor outcome. Recent efforts to intensify treatment for MRT have resulted in isolated reports of longβterm survival.
METHODS.
The authors conducted a retrospective review of consecutive patients with MRT at Childrens Hospital Los Angeles over the 20 years from 1983 to 2003.
RESULTS.
Fourteen children were diagnosed with MRT over the 20βyear study period. The median age at presentation was 22.5 months (range, 0.5β108 months). Five patients had renal primary tumors, and 9 patients had extrarenal tumors. Eleven of 14 patients had stage III or IV disease at diagnosis. Five patients (35.7%) were longβterm survivors. The time to disease progression was rapid (mean, 3.6 months). There were no recurrences or deaths beyond 10 months after diagnosis. All survivors received multimodal therapy, including both chemotherapy and surgery with or without radiation. In addition, 2 patients received highβdose chemotherapy with hematopoietic stem cell rescue (HSCT) after neoadjuvant chemotherapy and local tumor control. Both of those patients were longβterm survivors. There were no survivors after disease recurrence or progression.
CONCLUSIONS.
Patients with localized disease and complete surgical resection were most likely to survive longβterm. Consolidation with HSCT may benefit selected patients with advanced disease stage. International collaboration and further understanding of the biology of this disease is necessary to improve the survival of children with MRT. Cancer 2007. Β© 2007 American Cancer Society.