De novo entstandener maligner peripherer Nervenscheidentumor der Brust – Fallbericht

Abstract

BACKGROUND: Sarcomas of the breast are rare, representing less than 1% of malignant breast tumours. Malignant peripheral nerve sheath tumour (MPNSTs) is the malignant counterpart to benign soft tissue tumours such as neurofibromas and schwannomas. It is the most common sarcoma arising in the setting of von Recklinghausen's disease. METHODS: We report a de novo malignant peripheral nerve sheath tumour of the breast in 18-year-old patient. To the best of our knowledge, this is the first reported case of sporadic MPNST occurring in the breast in the absence of neurofibromatosis. RESULTS: Immunohistochemical examination is essential to establish the diagnosis and helpful in excluding other lesions in the differential diagnosis. CONCLUSIONS: The surgeon should approach these tumours according to the established guidelines for soft tissue sarcoma surgery.