Rhabdoid tumors in children: Prognostic factors in 70 patients diagnosed in Germany

Department of Pediatric Hematology and Oncology, University Hospital, D-66421 Homburg/Saar, Germany.
Oncology Reports (Impact Factor: 2.3). 04/2008; 19(3):819-23. DOI: 10.3892/or.19.3.819
Source: PubMed


We retrospectively analysed the data of 70 patients (41 boys and 29 girls) with Rhabdoid tumors (RT) regardless of localisation, recorded in the German Childhood Cancer Registry (GCCR) from 1984 to 1999. The primary tumor was located in the kidney in 32 cases, in the central nervous system (CNS) in 13 cases and in the soft tissue in 25 cases. Variables examined were tumor stage, sex, age at diagnosis, surgical radicality, radiotherapy and chemotherapeutic regimens. Metastatic disease at diagnosis was observed in 18 of the 70 individuals. Outcome of this group was very poor with a 5-year overall survival of 11%. There were no differences in survival between males and females, or younger and older children. Chemotherapeutic regimens were mainly given according to the primary site of the tumor. Radiotherapy was given in 28 of the 70 patients with a mean dose of 35 Gray, though this did not improve the outcome. Overall survival of the whole cohort was 27% at 5 years and there was no significant difference in prognosis regarding the different locations of the tumor (kidney 24%, soft tissue 30%, CNS 29%). In conclusion, RT in infants and children has a dismal prognosis, independent from localisation. The presence of metastasis at diagnosis seems to be the only prognostic factor of outcome.

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    • "In the International Society of Pediatric Oncology, only 2.8% of the RTK patients had CNS involvement because CNS evaluation was not a mandatory diagnostic procedure14). Reinhard et al.10) reported that only 1 patient had synchronous brain lesion among 70 patients of rhabdoid tumor (32 RTK patients). However, these studies only reported either CNS involvement or brain lesion, and failed to evaluate the rate of solitary spinal metastasis. "
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    ABSTRACT: Rhabdoid tumor of the kidney (RTK) is a rare malignancy in infancy. Central nervous system involvement in RTK is already known. However, solitary spinal metastasis in RTK has been hardly reported. The authors report a case of metastatic RTK to spine causing paraplegia in an 8-month-old girl. Since the patient was young, the diagnosis of spine metastasis was delayed until paraplegia was seen after radical nephrectomy. Thorough neurological examination should be performed for early diagnosis of spinal metastasis in young patients with RTK. If there are any abnormal signs in neurologic examination, magnetic resonance images of brain and spine are recommended.
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    • "Tomlinson et al. demonstrated a strong correlation of increasing survival with increasing age at diagnosis for patients with renal malignant rhabdoid tumors which is similar to what we have found for ER/EC MRT [86]. Similar to our study, a German review of 70 pediatric cases of malignant rhabdoid tumor of varying locations showed no improvement in outcome with radiotherapy [88]. "
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    ABSTRACT: Purpose. Malignant rhabdoid tumor (MRT) is an uncommon tumor that rarely occurs outside of renal and central nervous system (CNS) sites. Data from the literature were compiled to determine prognostic factors, including both demographic and treatment variables of malignant rhabdoid tumor, focusing on those tumors arising in extra-renal, extra-CNS (ER/EC MRT) sites. Patients and Methods. A systematic review and meta-analysis was performed by extracting demographic, treatment, and survival follow up on 167 cases of primary ER/EC MRT identified in the literature. Results. No survival differences were observed between those treated with or without radiation, or with or without chemotherapy. A Cox regression of overall survival revealed several independent prognostic factors. Surgical excision had a 74% (P = 0.0003) improvement in survival. Actinomycin had a 73% (P = 0.093) improvement in survival. Older age was associated with improved survival. The four-year survival, by Kaplan-Meier estimates, comparing patients less than two years old versus older than two at diagnosis was 11% versus 35%, respectively (P = 0.0001, Log-Rank). Conclusion. ER/EC MRT is a rare, soft-tissue tumor with a poor prognosis most commonly occurring in children. Surgical resection, treatment with actinomycin, and older age at diagnosis are all associated with improved survival.
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