ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2007
Sarcomas in children with neurofibromatosis type 1-poor prognosis despite aggressive combined therapy in four patients treated in a single oncological institution.
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers, 2 of them syntheses that pooled it.
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4 citing papers in PubMed, 2 syntheses or guidelines pooled it, 18 citations in OpenAlex.
- Autologous hematopoietic stem cell transplantation following high dose chemotherapy for non-rhabdomyosarcoma soft tissue sarcomas.The Cochrane database of systematic reviews · 2013Pooled it
- High-dose chemotherapy followed by autologous stem cell transplantation for metastatic rhabdomyosarcoma--a systematic review.PloS one · 2011Pooled it
- Retroperitoneal malignant triton tumor in an infant: a case report and literature review.Translational pediatrics · 2020Article
- A Comprehensive Review of Pediatric Tumors and Associated Cancer Predisposition Syndromes.Journal of genetic counseling · 2017Review
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12 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectsPatients with neurofibromatosis type 1 (NF1) are predisposed to developing soft tissue sarcomas (STS). MATERIALS AND
methodsWe report on four cases of STS diagnosed in locally advanced, unresectable stages in children with NF1 (three girls, one boy; age = 8 months-14 years). All patients received protocols for STS: Cooperative Weichteilsarkomstudie 91, 96 and 2002. One patient with limb rhabdomyosarcoma entered complete remission but developed late metastatic relapse and died of progression despite complete excision and autologous bone marrow transplantation. The other patient with bladder rhabdomyosarcoma died of neutropenia-related sepsis without remission. Patients with malignant peripheral nerve sheet tumour and malignant triton tumour located in the pelvis did not respond to therapy. One of them died of disease progression, while the other is disease-free 6 years post-therapy after mutilating tumour resection.
conclusionSTS in NF1 seem to display poor prognosis in spite of combined therapy; thus, children with NF1 should remain under detailed control of the oncologist.
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