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CC BY-NC-ND 4.0 · World J Nucl Med 2019; 18(03): 296-300
DOI: 10.4103/wjnm.WJNM_54_18
Case Report

Malignant peripheral nerve sheath tumor of thigh with sphenoid and brain metastases: Extremely rare occurrence with dismal prognosis despite significant response to palliative chemoradiotherapy

Authors

  • Abhishek Purkayastha

    Department of Radiation Oncology, Command Hospital (Southern Command), Pune, Maharashtra, India

  • Sankalp Singh

    Department of Radiation Oncology, Command Hospital (Southern Command), Pune, Maharashtra, India

  • Amul Kapoor

    1   Department of Medical Oncology, Command Hospital (Southern Command), Pune, Maharashtra, India

  • Jasvinder Kaur Bhatia

    2   Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India

  • Niharika Bisht

    Department of Radiation Oncology, Command Hospital (Southern Command), Pune, Maharashtra, India

  • Azhar Husain

    3   Department of Nuclear Medicine, Command Hospital (Southern Command), Pune, Maharashtra, India
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Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a neurogenic tumor arising from peripheral nerves or nerve sheaths. MPNSTs are highly aggressive sarcomas mainly associated with neurofibromatosis type-1 (NF-1) with high rates of local recurrence and distant metastasis carrying a dismal prognosis. Lung is the most common metastatic site. Bone metastasis although documented in the literature is still very rare, while dissemination to brain without the involvement of lungs and that too in a non-NF-1 case is extremely unusual. A 48-year-old female was diagnosed with a case of non-NF-1 MPNST left thigh with bone metastases including sphenoid. Despite showing complete resolution of skeletal and primary lesions postpalliative chemoradiotherapy, she developed brain metastases and succumbed to her disease. This case is discussed to highlight an unusual scenario we encountered, the clinical course of the disease with its management, and overall poor prognosis. To the best of our knowledge, this may be the earliest case of MPNST with sphenoid metastases detected by 18-fluorodeoxyglucose positron-emission computed tomography scan and a sporadic case of brain metastases reported in the world literature.

Keywords

18-Fluorodeoxyglucose positron-emission computed tomography scan - brain - chemoradiotherapy - malignant peripheral nerve sheath tumor - metastases - sphenoid

Financial support and sponsorship

Nil.




Publikationsverlauf

Eingereicht: 20. Mai 2018

Angenommen: 12. Juni 2018

Artikel online veröffentlicht:
22. April 2022

© 2019. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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