Retroperitoneal Malignant Peripheral Nerve Sheath Tumour: A Rare Case Report.
Malignant nerve sheath tumours (MPNST) are rare neoplasias and retroperitoneal cases are fairly rare and clinically difficult to be detected, but they are very agressive neoplasias. MPNST are frequently seen in head, neck and upper extremities. In patients with NF1; MPNST, a poor-prognostic lesion, may result from a malignant degeneration of a former plexiform neurofibroma. It is necessary to be aware of a potential malignancy in patients diagnosed with plexiform neurofibroma. We present a 21-year-old female with a diagnosis of MPNST. The patient was admited to the hospital because of a tumour in the subcutaneous region on her left buttock. The surgeon's clinical diagnosis was lipoma. After the pathological examination of biopsy specimen, the lesion was identified as "plexiform neurofibroma" and then the patient was diagnosed with Neurofibromatosis Type 1 (NF1). Simultaneously, another mass on the retroperitoneal region was identified as malignant peripheral nerve sheath tumour (MPNST).
Faculty of Medicine, Department of Pathology, Dumlupinar University , Kutahya/Turkey .
Ayse Nur
AN
Deger
Faculty, Department of Neurosurgery, Ankara Training and Research Hospital , Ankara/Turkey .
Mehmet Akif
MA
Bayar
Faculty, Department of Pathology, Ankara Training and Research Hospital , Ankara/Turkey .
Muzaffer
M
Caydere
Faculty, Department of Neurosurgery, Kutahya Training and Research Hospital , Kutahya/Turkey .
Hakki
H
Deger
Faculty of Medicine, Department of Pathology, Erzincan University , Erzincan/Turkey .
Mahir
M
Tayfur
Neurofibromatosis Type 1(NF1); Plexiform neurofibroma; Retroperitoneal mass