Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1 Patient

Sudarsono, Obdes Emputri, Iswahyudi, Indriasari, Tati Herdawati, Dyah Marianingrum

2018

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is rare and highly aggressive neoplasm. Approximately half of MPNST cases occur in association with neurofibromatosis type 1 (NF1). MPNST contribute significantly in reducing life-span of neurofibromatosis type 1 patients. The only known definitive therapy for MPNST is surgical resection with wide negative margins which may not be feasible in certain conditions involving tumor size, location, and/or metastases. Therefore, early diagnosis of MPNST is mandatory to increase the rate of successful surgical resection. We present a 36-year-old female with NF1, who had a giant MPNST on the right arm. She died six months after diagnosis in spite of a surgical resection and chemotherapy. This case report aimed to highlight the signs and symptoms of malignant peripheral nerve sheath tumor and examinations that are needed to make an early diagnosis of MPNST especially in patients with neurofibromatosis type 1.

Paper Citation

in Harvard Style

Sudarsono., Emputri O., Iswahyudi., Indriasari., Herdawati T. and Marianingrum D. (2018). Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1 Patient.In Proceedings of the 23rd Regional Conference of Dermatology - Volume 1: RCD, ISBN 978-989-758-494-7, pages 359-362. DOI: 10.5220/0008157403590362

in Bibtex Style

@conference{rcd18,
author={Sudarsono and Obdes Emputri and Iswahyudi and Indriasari and Tati Herdawati and Dyah Marianingrum},
title={Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1 Patient},
booktitle={Proceedings of the 23rd Regional Conference of Dermatology - Volume 1: RCD,},
year={2018},
pages={359-362},
publisher={SciTePress},
organization={INSTICC},
doi={10.5220/0008157403590362},
isbn={978-989-758-494-7},
}

in EndNote Style

TY - CONF

JO - Proceedings of the 23rd Regional Conference of Dermatology - Volume 1: RCD,
TI - Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1 Patient
SN - 978-989-758-494-7
AU - Sudarsono.
AU - Emputri O.
AU - Iswahyudi.
AU - Indriasari.
AU - Herdawati T.
AU - Marianingrum D.
PY - 2018
SP - 359
EP - 362
DO - 10.5220/0008157403590362