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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
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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
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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