Malignant peripheral nerve sheath tumours and neurofibromatosis 1: A case series and recommendations for care
SA Journal of Oncology
| Field | Value | |
| Title | Malignant peripheral nerve sheath tumours and neurofibromatosis 1: A case series and recommendations for care | |
| Creator | Burger, Henriette Bezuidenhout, Heidre Sher-Locketz, Candice Baatjes, Karin van Wyk, Jacques Bonthuys, Anita | |
| Description | Background: 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. | |
| Publisher | AOSIS | |
| Date | 2018-08-20 | |
| Identifier | 10.4102/sajo.v2i0.46 | |
| Source | South African Journal of Oncology; Vol 2 (2018); 8 pages 2523-0646 2518-8704 | |
| Language | eng | |
| Relation |
The following web links (URLs) may trigger a file download or direct you to an alternative webpage to gain access to a publication file format of the published article:
https://sajo.org.za/index.php/sajo/article/view/46/133
https://sajo.org.za/index.php/sajo/article/view/46/132
https://sajo.org.za/index.php/sajo/article/view/46/134
https://sajo.org.za/index.php/sajo/article/view/46/131
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