Extra-cranial Malignant Rhabdoid Tumor in Children: A Single Institute Experience.
- Author:
Che Ry HONG
1
;
Hyoung Jin KANG
;
Hee Young JU
;
Ji Won LEE
;
Hyery KIM
;
Sung Hye PARK
;
Il Han KIM
;
Kyung Duk PARK
;
Hee Young SHIN
Author Information
- Publication Type:Original Article
- Keywords: Rhabdoid tumor; Kidney neoplasms; Soft tissue neoplasms; Neoplasm metastasis
- MeSH: Carboplatin; Child*; Cohort Studies; Diagnosis; Disease-Free Survival; Drug Therapy; Etoposide; Follow-Up Studies; Humans; Kidney Neoplasms; Medical Records; Melphalan; Mortality; Neoplasm Metastasis; Radiotherapy; Recurrence; Retrospective Studies; Rhabdoid Tumor*; Seoul; Soft Tissue Neoplasms; Stem Cells
- From:Cancer Research and Treatment 2015;47(4):889-896
- CountryRepublic of Korea
- Language:English
- Abstract: PURPOSE: Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that affects young children. Due to its extreme rarity, most of the available data are based on retrospective case series. To add to the current knowledge of this disease, we reviewed the patients treated for extra-cranial MRT in our institute. MATERIALS AND METHODS: A retrospective medical record review was conducted on children treated for pathologically confirmed extra-cranial MRT at Seoul National University Children's Hospital between January 2003 and May 2013. RESULTS: Eleven patients (7 boys, 4 girls) were diagnosed with extra-cranial MRT at a median age of 9 months old. INI1 staining was important in the pathological confirmation. Six patients (55%) had renal MRT and five (45%) had soft tissue MRT. Five patients (45%) had metastases at diagnosis. All patients underwent chemotherapy, eight patients (73%) underwent surgery, six patients (55%) received therapeutic radiotherapy, and four patients (36%) underwent high dose chemotherapy with autologous stem cell rescue (HDCT/ASCR) with melphalan, etoposide, and carboplatin. Five patients (45%) died of disease following progression (n=3) or relapse (n=2), however, there was no treatment related mortality. The overall survival of the cohort was 53.0% and the event-free survival was 54.5% with a median follow-up duration of 17.8 months (range, 2.3 to 112.3 months). CONCLUSION: Extra-cranial MRT is still a highly aggressive tumor in young children. However, the improved survival of our cohort is promising and HDCT/ASCR with melphalan, etoposide, and carboplatin may be a promising treatment option.
