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
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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
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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.
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