ArticleTranslational pediatrics2021
An analysis of the diagnosis, clinical characteristics, treatment, and survival outcomes of 36 extracranial malignant rhabdoid tumor patients.
Article in Translational pediatrics, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers, 1 of them a synthesis that pooled it.
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Who cites it
6 citing papers in PubMed, 1 synthesis or guideline pooled it, 7 citations in OpenAlex.
- Extracranial malignant rhabdoid tumors in children: high mortality even with the help of an aggressive clinical approach.European journal of pediatrics · 2024Pooled it
- Article
- Case Report: Multidisciplinary management and nursing care for a preterm infant with rare extrarenal malignant rhabdoid tumor: a clinical case study.Frontiers in pediatrics · 2025Article
- Case Report: Pathological confirmation and aggressive postoperative recurrence of WHO grade III rhabdoid meningioma.Frontiers in medicine · 2025Article
- Malignant Rhabdoid Tumor of the Kidney in a Child With Xeroderma Pigmentosum: Incidence or Coincidence?Cureus · 2022Article
- Molecular Heterogeneity in Pediatric Malignant Rhabdoid Tumors in Patients With Multi-Organ Involvement.Frontiers in oncology · 2022Article
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Authors and funding
15 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundExtracranial malignant Rhabdoid tumors (eMRTs) are rare but aggressive lesions in young children. This work aimed to review and analyze the diagnosis, clinical characteristics, treatment, and survival of eMRTs so as to summarize experience for future therapy.
methodsA total of 36 eMRT cases were treated between January 2008 and August 2019 according to Shanghai Children's Medical Center (SCMC) multimodal protocol of mixed surgery, radiation and chemotherapy involving vincristine, carboplatin, doxorubicin, etoposide, cyclophosphamide and ifosfamide. We collected information including: age at diagnosis, tumor location, disease stage, therapy, outcomes, etc. Overall survival (OS) and event free survival (EFS) were calculated and risk factors for survival were analyzed.
resultsThe patients had a median age of 1.80 years at diagnosis (range, 1.4 m-13.42 years), and were followed up for 9.17 months in median (range, 4 d-11.14 y). A total of 16 patients achieved complete remission (CR), and 7 survived without reoccurrence till December 2019. The 3-year EFS was 17.4% (95% CI: 11.0-23.8%) with a 3-year OS of 23.4% (95% CI: 15.8-31.0%). Recurrence was found only in children younger than the median age (1.80 y). Localized staging (Log Rank P=0.039 for OS and P=0.021) and older age (Log Rank P=0.016 for OS and P=0.002 for EFS) were associated with improved outcome. Younger age (Cox regression, OS, OR =2.610, 95% CI: 1.147-5.937, P=0.022; EFS, OR =3.401, 95% CI: 1.495-7.752, P=0.004) were independent risk factors for death and recurrence.
conclusionsThose eMRTs treated according to SCMC protocol turned out to have poor outcomes. Higher staging at diagnosis and reoccurrence in younger patients remain major threats to the prognosis.
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