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.
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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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