Long‐term survivor of relapsed stage IV malignant rhabdoid tumor of the kidney

Abstract

The prognosis for metastatic malignant rhabdoid tumor (MRT) is poor, and metastatic (stage IV) MRT was resistant to conventional treatment, with less than 20% of cure rate. Moreover, there have been no reports of patients who have survived relapsed stage IV MRT. Here we report a long-term survivor of relapsed MRT with lung metastasis at diagnosis. He was diagnosed as MRT of the kidney at 5-month-old. After resection of the renal tumor, he was treated with ICE (ifosfamide, carboplatin, and etoposide), total abdominal irradiation 10.8 Gy and high-dose chemotherapy using thiotepa and melphalan. Six months after initial treatment, a relapse in the lung was detected, and he received chemotherapy including doxorubicin/pirarubicin for 78 weeks. He is alive at five years of follow up, without any evidence of disease. Our report suggests the important role of anthracycline in treatment of MRT.

High dose alkylator therapy for extracranial malignant rhabdoid tumors in children

“…In our series, there was no progression/relapse 7 months after diagnosis. None of our patients who progressed survived even though there have been isolated reports of survival after relapse [4,8]. Age is an important prognostic factor in MRT with younger age associated with worse prognosis [5].…”

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

“…In fact, tumor specimens of most of our Extra-cranial MRT is treated with multimodal therapies, including chemotherapy, surgery, radiotherapy, and HDCT/ ASCR, however, no standard treatment has been established to date. Anthracycline and actinomycine D have been shown to be important chemotherapeutic agents for MRT [13,14]. Also, alternating courses of the combination of vincristine, doxorubicin, and cyclophosphamide (VDCy) and the combination of ifosfamide, carboplatin, and etoposide (ICbE) have been suggested to be effective in metastatic MRT [15,16].…”

“…In literature, complete resection of solitary lung nodules, whole-lung irradiation, and alternative courses of ICbE and VDCy chemotherapy has been suggested as possible strategies for lung metastases [14,15,20].…”

Malignant rhabdoid tumors of soft tissues in children. Literature review

“…Показано, что на выживаемость влияет использование высоких доз алкилирующих агентов, в том числе антрациклино-вых антибиотиков и актиномицина D [9,80]. Также отмечено влияние альтернирующих курсов терапии в комбинации с такими препаратами, как винкри-стин, циклофосфамид, доксорубицин (VDC) и ифос-фамид, карбоплатин, этопозид (ICE) на метастати-ческую форму заболевания [81,82].…”