Cancer Res Treat.  2015 Oct;47(4):889-896. 10.4143/crt.2013.176.

Extra-cranial Malignant Rhabdoid Tumor in Children: A Single Institute Experience

• Hong CR
• Kang HJ
• Ju HY
• Lee JW
• Kim H
• Park SH
• Kim IH
• Park KD
• Shin HY

• KMID: 2403409
• DOI: http://doi.org/10.4143/crt.2013.176

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.

Keyword

Rhabdoid tumor; Kidney neoplasms; Soft tissue neoplasms; Neoplasm metastasis

MeSH Terms

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
Carboplatin
Etoposide
Melphalan

Figure

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  Fig. 1. (A) Kaplan-Meier analysis of the overall survival of the total study population. (B) Kaplan-Meier analysis of the event-free survival of the total study population.

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  Fig. 2. (A) Kaplan-Meier analysis of the overall survival of the recipients of high dose chemotherapy and autologous stem cell rescue (HDCT/ASCR). (B) Kaplan-Meier analysis of the event-free survival of the recipients of HDCT/ASCR.

Reference

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