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CASE REPORT
Radical resection and improvised surgical reconstruction for a rare malignant triton tumour of intercostal nerve in a patient with neurofibromatosis type 1
  1. Ikram Ulhaq Chaudhry1,
  2. Ahsan Iqbal Cheema2,
  3. Hadi Mutari3,
  4. Samir S Amr4
  1. 1Department of Chest Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
  2. 2Division of Thoracic Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
  3. 3Department of Thoracic Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia
  4. 4Department of Pathology and Laboratory Medicine, King Fahad Specialist Hospital, Dammam, Saudi Arabia
  1. Correspondence to Ikram Ulhaq Chaudhry, drihc{at}hotmail.co.uk

Summary

A man aged 28 years, with neurofibromatosis type 1, presented with abdominal pain and visible right lower chest swelling. He had cutaneous neurofibromas and several café-au-lait spots. CT scan of the chest and abdomen revealed a giant mass most likely originating from the right 7th intercostal nerve, extending downwards into the abdomen causing massive hepatic compression. A Trucut biopsy showed malignant peripheral nerve sheath tumour. Radical surgical resection with wide margins was performed. The chest wall and diaphragm were reconstructed with improvised surgical technique with excellent results. Final histopathology report revealed a triton tumour of the intercostal nerve.

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Footnotes

  • Contributors IUC, operating surgeon, performed the procedure and wrote the abstract and manuscript. AIC is the assistant surgeon and searched for the references. HM wrote the part of the discussion and helped in croping the pictures. SSA, pathologist, provided the histology report and slides.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.