PATHOLOGY & ONCOLOGY RESEARCHVol. 9 No. 3, 2003

 Report

Malignant Peripheral Nerve Sheath Tumors Associated With Neurofibromatosis Type 1

Report of 4 cases

Bilge BILGIC1, Lora Esberk ATES1, Misten DEMIRYONT1, Harzem OZGER2, Yavuz DIZDAR3

1Department of Pathology, Istanbul University, Istanbul Medicine Faculty, Istanbul, Turkey
2Department of Orthopaedic Surgery, Istanbul University, Istanbul Medicine Faculty, Istanbul, Turkey
3Institute of Oncology, Department of Radiation Oncology, Istanbul University, Istanbul Medicine Faculty, Istanbul, Turkey

 

We report 4 cases of malignant peripheral nerve sheath tumors (MPNST) with neurofibromatosis type 1 (NF1). Mean age was 29.5. Two of them had a family history. Three of them were male. All of them had enlarging mass and pain in the background of neurofibromas. Locations were popliteal, thigh and forearm. The masses were greater than 5 cm in diameter in each case. In two cases the mass was showing continuity with a nerve. One patient had a nonossifying fibroma as well as a MPNST. Wide excision and radiotherapy were applied to three of the patients. One of them did not take any therapy after surgical resection. Two of the patients died of lung metastases after a mean period of 12.5 months. In a majority of NF1 patients MPNST emerges from a preexisting neurofibroma. The patients with NF1 are at greatest risk for developing sarcomas, so they should be followed closely. Pathology & Oncology Research, Vol 9, Nr 3, 201-205, 2003

Key words: Neurofibromatosis; malignant peripheral nerve sheath tumours; sarcoma; nonossifying fibroma


Received: Jun 12, 2003; accepted: Sep 5, 2003
Correspondence: Lora Esberk ATES, Department of Pathology, Istanbul University, Istanbul Medicine Faculty, Capa Istanbul 34390, Turkey; Tel: +90 212 6351171, Fax: +90 212 6311367; E-mail: loraates@hotmail.com

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