Malignant peripheral nerve sheath tumours and neurofibromatosis 1 : a case series and recommendations for care

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dc.contributor.authorBurger, Henrietteen_ZA
dc.contributor.authorBezuidenhout, Heidreen_ZA
dc.contributor.authorSher-Locketz, Candiceen_ZA
dc.contributor.authorBaatjes, Karinen_ZA
dc.contributor.authorVan Wyk, Jacquesen_ZA
dc.contributor.authorBonthuys, Anitaen_ZA
dc.date.accessioned2020-05-17T05:24:13Z
dc.date.available2020-05-17T05:24:13Z
dc.date.issued2018-08-20
dc.descriptionCITATION: Burger, H. et al. 2018. Malignant peripheral nerve sheath tumours and neurofibromatosis 1 : a case series and recommendations for care. South African Journal of Oncology, 2:a46, doi:10.4102/sajo.v2i0.46.
dc.descriptionThe original publication is available at https://sajo.org.za
dc.description.abstractBackground: The incidence of malignant peripheral nerve sheath tumours (MPNST) in patients with neurofibromatosis 1 (NF1) is significantly higher than that of the general population. NF1-associated MPNST occur at a younger age and carry a worse prognosis than sporadic MPNST. Aim: This case series describes four cases of MPNST in patients with NF1. Setting: The study was performed in a public academic hospital in the Western Cape province of South Africa. Method: Demographics, disease status, histopathology, treatment and outcome data were collected retrospectively from medical charts and through review of histological slides. Results: The median age was 36.5 years. All tumours were > 5 cm at presentation and located on the trunk. One patient presented with metastatic disease. There was a mean delay of 3.5 months from presentation to initiation of treatment. Three patients underwent wide excision, with one receiving adjuvant chemotherapy and radiotherapy. At a median follow-up of 20 months from histological diagnosis only one patient was alive in clinical remission. Two patients had succumbed to progressive disease at 8 and 16 months from diagnosis and one patient with terminal metastatic disease was lost to follow-up. Conclusion: In this series the patients presented with advanced, often unresectable lesions for which single modality therapy was not curative. An adult NF1 health surveillance guideline for resource-constrained environments could lead to early diagnosis and treatment of MPNST and other complications in NF1 patients.en_ZA
dc.description.urihttps://sajo.org.za/index.php/sajo/article/view/46
dc.description.versionPublisher's version
dc.format.extent8 pages
dc.identifier.citationBurger, H. et al. 2018. Malignant peripheral nerve sheath tumours and neurofibromatosis 1 : a case series and recommendations for care. South African Journal of Oncology, 2:a46, doi:10.4102/sajo.v2i0.46.
dc.identifier.issn2523-0646 (online)
dc.identifier.issn2518-8704 (print)
dc.identifier.otherdoi:10.4102/sajo.v2i0.46
dc.identifier.urihttp://hdl.handle.net/10019.1/108570
dc.language.isoen_ZAen_ZA
dc.publisherAOSIS
dc.rights.holderAuthors retain copyright
dc.subjectNeurofibromatosisen_ZA
dc.titleMalignant peripheral nerve sheath tumours and neurofibromatosis 1 : a case series and recommendations for careen_ZA
dc.typeArticleen_ZA
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