Metastatic malignant peripheral nerve sheath tumor. A diagnostic surprise

https://doi.org/10.4081/monaldi.2021.1658

Authors

  • Muniza Bai Department of Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India. https://orcid.org/0000-0001-9186-1380
  • Vishnukanth Govindaraj | vishnu1429@yahoo.com Department of Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India. https://orcid.org/0000-0002-3854-2388
  • Abhishek Singh Chauhan Department of Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.
  • Bheemanati Hanuman Srinivas Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.
  • Jinkala Sree Rekha Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.
  • Vijayarangam Narenchandra Department of Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.

Abstract

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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References

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Published
2021-05-06
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Section
Pneumology - Case Reports
Conflict of interest statement
lung secondaries
Supporting Agencies
none
Keywords:
Malignant peripheral nerve sheath tumours, neurofibromatosis, soft tissue sarcomas, cardiac metastasis, recurrence.
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How 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.