https://doi.org/10.4081/monaldi.2021.1658
Metastatic malignant peripheral nerve sheath tumor. A diagnostic surprise
Submitted: October 29, 2020
Accepted: January 17, 2021
Published: May 6, 2021
Accepted: January 17, 2021
Abstract Views: 1221
PDF: 651
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
Malignant peripheral nerve sheath tumours (MPNSTs) are rare soft tissue tumors that arise from pre-existing plexiform neurofibromas or within a normal peripheral nerve. They are aggressive tumors with high rates of recurrence and distant metastases, the most common sites of metastasis being the lung followed by bone.
A 46 year old gentleman presented with breathlessness and chest pain three years after post amputation of left thumb for an ulcerative growth. CECT thorax showed a left upper lobe mass with pleural and pericardial effusion. Within a month of presentation he worsened and succumbed to the disease. Antemortem biopsy of the left hand ulcerative growth showed features suggestive of malignant peripheral nerve sheath tumour- epithelioid variant and post mortem liver and lung biopsy showed metastasis of MPNST. The diagnosis was a malignant peripheral nerve sheath tumor with lung, liver and cardiac metastasis.
This case report aims to highlight the importance of upfront aggressive multimodality local therapy for achieving local disease control in patients presenting with localised MPNST and regular follow up for early detection of relapse and metastasis.
Yoon J, Lee H, Chun J, et al. Huge intrathoracic malignant peripheral nerve sheath tumor in an adolescent with neurofibromatosis type 1. Case Rep Pediatr 2014;2014:951252.
DOI: https://doi.org/10.1155/2014/951252
Penumadu P, Shanmugasundaram S, Sachdeva A, Srinivas B. Malignant peripheral nerve sheath tumor of the thorax: Unusual location of a rare tumor. J Cancer Res Ther 2018;14:1145-8.
DOI: https://doi.org/10.4103/0973-1482.203595
Kitamura M, Wada N, Nagata S, et al. Malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart: a case report. Diagn Pathol 2010;5:2.
DOI: https://doi.org/10.1186/1746-1596-5-2
Kleihues P, Louis D, Scheithauer B, et al. The WHO Classification of Tumors of the Nervous System. J Neuropathol Exp Neurol 2002;61:215-25. discussion 226-9.
DOI: https://doi.org/10.1093/jnen/61.3.215
Stucky C, Johnson K, Gray R, et al. Malignant peripheral nerve sheath tumors (MPNST): The Mayo Clinic Experience. Ann Surg Oncol 2011;19:878-5.
DOI: https://doi.org/10.1245/s10434-011-1978-7
Grobmyer S, Reith J, Shahlaee A, et al. Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations. J Surg Oncol 2008;97:340-9.
DOI: https://doi.org/10.1002/jso.20971
Ducatman B, Scheithauer B, Piepgras D, et al. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 1986;57:2006-21.
DOI: https://doi.org/10.1002/1097-0142(19860515)57:10<2006::AID-CNCR2820571022>3.0.CO;2-6
Wise J, Cryer J, Belasco J, et al. Management of head and neck plexiform neurofibromas in pediatric patients with neurofibromatosis type 1. Arch Otolaryngol Head Neck Surg 2005;131:712-8.
DOI: https://doi.org/10.1001/archotol.131.8.712
Jo V, Fletcher C. Epithelioid malignant peripheral nerve sheath tumor. Am. J. Surg. Pathol. 2015;39:673-2.
DOI: https://doi.org/10.1097/PAS.0000000000000379
Wang T, Yin H, Han S, et al. Malignant peripheral nerve sheath tumor (MPNST) in the spine: a retrospective analysis of clinical and molecular prognostic factors. J Neurooncol 2015;122:349-5.
DOI: https://doi.org/10.1007/s11060-015-1721-5
Kamran S, Shinagare A, Holler Howard S, et al. Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management. Radiol Oncol 2013;47:230-8.
DOI: https://doi.org/10.2478/raon-2013-0047
Goldblum JR, Weiss SW, Folpe AL. Enzinger and Weiss’s Soft Tissue Tumors. 6th ed. Philadelphia: Elsevier Saunders; 2014. p. 855‑79.
Zou C, Smith K, Liu J, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg 2009;249:1014-22.
DOI: https://doi.org/10.1097/SLA.0b013e3181a77e9a
Treglia G, Taralli S, Bertagna F, et al. Usefulness of whole-body fluorine-18-fluorodeoxyglucose positron emission tomography in patients with neurofibromatosis type 1: A systematic review. Radiol Res Pract 2012;2012:431029.
DOI: https://doi.org/10.1155/2012/431029
Ethics Approval
lung secondariesSupporting Agencies
noneHow to Cite
Bai, Muniza, Vishnukanth Govindaraj, Abhishek Singh Chauhan, Bheemanati Hanuman Srinivas, Jinkala Sree Rekha, and Vijayarangam Narenchandra. 2021. “Metastatic Malignant Peripheral Nerve Sheath Tumor. A Diagnostic Surprise”. Monaldi Archives for Chest Disease 91 (2). https://doi.org/10.4081/monaldi.2021.1658.
Copyright (c) 2021 The Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.
Similar Articles
- Savvas Omorphos, Christophoros Kotoulas, Jarrod James Homer, Kenneth Edward McLaughlin, Delayed repair of esophageal perforation due to transoesophageal echocardiography , Monaldi Archives for Chest Disease: Vol. 68 No. 4 (2007): Cardiac series
- Margaret Cupples, Neil Heron, What to do after cardiac rehabilitation programs: the role of the general practitioner in cardiovascular prevention , Monaldi Archives for Chest Disease: Vol. 86 No. 1-2 (2016): Cardiac series
- Sabino Scardi, Carmine Mazzone, Paolo Umari, The challenge of improving evidence-based therapy adherence in the secondary prevention of coronary artery disease: the next frontier of cardiac rehabilitation , Monaldi Archives for Chest Disease: Vol. 72 No. 2 (2009): Cardiac series
- A. Chroneou, N. Zias, A.V. Gonzalez, J.F. Beamis Jr, Tracheobronchopathia Osteochondroplastica. An underrecognized entity? , Monaldi Archives for Chest Disease: Vol. 69 No. 2 (2008): Pulmonary series
- Gianaugusto Slavich, Paola Mapelli, Rudy Fregolent, Massimo Slavich, Duilio Tuniz, Non ST ergometric variables in the diabetic patient and their prognostic significance , Monaldi Archives for Chest Disease: Vol. 74 No. 1 (2010): Cardiac series
- Giovanni Luca Botto, Carlo Piemontese, Giovanni Russo, Rhythm-control vs rate-control in the elderly: When to do it and which drug to prefer? , Monaldi Archives for Chest Disease: Vol. 88 No. 2 (2018)
- Matteo Ruzzolini, Gaetano Luca Panetta, Antonietta Evangelista, Carlo Peraldo Neja, Paolo Azzolini, Usefulness of transthoracic echocardiogram in management of cardiac involvement in large B-Cell lymphoma: a case report , Monaldi Archives for Chest Disease: Vol. 82 No. 4 (2014): Cardiac series
- Teresa Strisciuglio, Gennaro Galasso, Dario Leosco, Roberta De Rosa, Giuseppe Di Gioia, Valentina Parisi, Stefania De Luca, Tullio Niglio, Chiara De Biase, Rossella Luciano, Giuseppe Rengo, Bruno Trimarco, Federico Piscione, Adipokines and coronary artery disease , Monaldi Archives for Chest Disease: Vol. 78 No. 3 (2012): Cardiac series
- Antonello D’Andrea, Raffaella Scarafile, Ilaria Ferrara, Maria Luisa De Rimini, Pietro Muto, Maria Giovanna Russo, Raffaele Calabrò, An unusual case of apical hypertrophic cardiomyopathy in a transplanted heart , Monaldi Archives for Chest Disease: Vol. 74 No. 4 (2010): Cardiac series
- Tommaso Diaco, Geremia Milanesi, Daniela Zaniboni, Massimo Gritti, Gianna Zavatteri, Marco Claus, Francesco Velo, Proposal of resources optimization in the hospital treatment of heart failure by an increased utilization of cardiac rehabilitation , Monaldi Archives for Chest Disease: Vol. 66 No. 4 (2006): Cardiac series
<< < 13 14 15 16 17 18 19 20 21 22 > >>
You may also start an advanced similarity search for this article.
