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Malignant peripheral sheath tumour of the neck mimicking a thyroid tumour. A case report and a literature review from a reference centre for endocrine surgery
Affiliations- Department for General, Gastroenterological and Endocrine Surgery, Medical University of Wroclaw, Poland
- Department for Vascular, General and Transplant Surgery, Medical University of Wroclaw, Poland
open access
Abstract
Malignant peripheral sheath tumors (MPNST) are rare soft tissue sarcomas originating from peripheral sheath cells. The main risk factor is neurofibromatosis type 1 (NF-1). The localization in the head and neck region is uncommon (up to 15% of all cases of MPNST) making the diagnosis difficult due to anatomical and clinical reasons. We present a case of 45-years-old female with the NF-1- linked MPNST localized in the thyroid region with clinical manifestation as a left thyroid tumour. The patient underwent surgery and adjuvant radiation therapy, remaining disease-free for 14 months after the treatment.
Abstract
Malignant peripheral sheath tumors (MPNST) are rare soft tissue sarcomas originating from peripheral sheath cells. The main risk factor is neurofibromatosis type 1 (NF-1). The localization in the head and neck region is uncommon (up to 15% of all cases of MPNST) making the diagnosis difficult due to anatomical and clinical reasons. We present a case of 45-years-old female with the NF-1- linked MPNST localized in the thyroid region with clinical manifestation as a left thyroid tumour. The patient underwent surgery and adjuvant radiation therapy, remaining disease-free for 14 months after the treatment.
Keywords
malignant peripheral sheath tumour, head and neck tumours, thyroid tumours, neurofibromatosis type 1 (von Recklinghausen disease), soft tissue sarcoma
About this articleTitle
Malignant peripheral sheath tumour of the neck mimicking a thyroid tumour. A case report and a literature review from a reference centre for endocrine surgery
Journal
Chirurgia Polska (Polish Surgery)
Issue
Pages
34-38
Published online
2017-06-13
Page views
1502
Article views/downloads
771
Bibliographic record
Chirurgia Polska 2016;18(1-2):34-38.
Keywords
malignant peripheral sheath tumour
head and neck tumours
thyroid tumours
neurofibromatosis type 1 (von Recklinghausen disease)
soft tissue sarcoma
Authors
Waldemar Balcerzak
Andrzej T. Dorobisz
Piotr Czopnik
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