Malignant peripheral nerve sheath tumor in a patient without neurofibromatosis 1 (NF1) : a rare case of primary lung location

Grzywa-Celińska Anna, Szmygin-Milanowska Katarzyna, Emeryk-Maksymiuk Justyna, Walczyna Maria, Palonka Michał, Krusiński Adam, Siwiec Jan. Malignant peripheral nerve sheath tumor in a patient without neurofibromatosis 1 (NF1) : a rare case of primary lung location. Journal of Education, Health and Sport. 2018;8(1):11-17. eISSN 2391-8306. DOI http://dx.doi.org/10.5281/zenodo.1134525

http://ojs.ukw.edu.pl/index.php/johs/article/view/5183

https://pbn.nauka.gov.pl/sedno-webapp/works/843691

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© The Authors 2018;

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This is an open access article licensed under the terms of the Creative Commons Attribution Non Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted, non commercial

use, distribution and reproduction in any medium, provided the work is properly cited.

The authors declare that there is no conflict of interests regarding the publication of this paper.

Received: 09.12.2017. Revised: 15.12.2017. Accepted: 02.01.2018.

Malignant peripheral nerve sheath tumor in a patient without neurofibromatosis 1 (NF1) : a rare case of primary lung location

Anna Grzywa-Celińska¹, acelin@op.pl

Katarzyna Szmygin-Milanowska¹, katarzynaszmygin@gmail.com

Justyna Emeryk-Maksymiuk², justynaemeryk@poczta.onet.pl

Maria Walczyna³, patomorfologia@umlub.pl

Michał Palonka¹, m.palonka@gmail.com

Adam Krusiński⁴, adak93@gmail.com

Jan Siwiec¹, dr.jan.siwiec@gmail.com

¹ Chair and Department of Pneumonology, Oncology and Allergology, Medical University of Lublin

² Chair of Internal Medicine and Department of Internal Medicine in Nursing, Medical University of Lublin

³ Chair and Department of Clinical Pathomorphology, Medical University of Lublin

⁴ Students’ Scientific Association, Chair and Department of Pneumonology, Oncology and Allergology, Medical University of Lublin

Key words: malignant peripheral nerve sheath tumor, lung, primary location

Abstract

We present a rare case of a patient operated due to a lung tumor, which was ultimately diagnosed as malignant peripherial nerve sheath tumor (MPNST). MPNSTs are rare tumors of soft tissue with mesenchymal origin. The World Health Organization has distinguished this group in order to unify terms in mesenchymal heterogeneous malignant tumors, e.g. neurofibrosarcomas, malignant schwannomas and malignant neurilemmoma [1]. They can occur as a result of neoplastic expansion of peripheral nerves’ branches, peripheral nerve fibers’ sheaths or Schwann cells, although many researchers believe that these tumors can derive not only from one, but several cell lines. MPNSTs are very rare in thorax, where they show aggressive pattern of growth [2] and stem from pleural cells rather than lung tissue.