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Related Experiment Videos

Multiple cervical and intrathoracic plexoid neurofibromas. Case report.

C Zisis1, A Dountsis, E Tzamourani

  • 1Department of Thoracic Surgery, Athens Medical Center, Greece. dkakats@cc.uoa.gr

The Journal of Cardiovascular Surgery
|March 5, 2004
PubMed
Summary

This case study highlights a rare instance of vagus nerve neurofibromas in a patient with von Recklinghausen

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Area of Science:

  • Neuro-oncology
  • Surgical Oncology
  • Genetics

Background:

  • Neurofibromatosis type 1 (von Recklinghausen's disease) is a genetic disorder characterized by tumor formation on nerve sheaths.
  • Vagus nerve neurofibromas are rare, particularly when presenting with significant symptoms like chronic pain and numbness.

Observation:

  • A 29-year-old male with known neurofibromatosis type 1 presented with a decade of paroxysmal right upper hand pain and numbness.
  • Cervical and mediastinal MRI revealed multiple large tumors originating from the vagus nerves bilaterally.
  • Surgical resection of the right-sided tumors was successfully performed via posterolateral thoracotomy.

Findings:

  • Histopathological examination confirmed the diagnosis of plexoid neurofibromas.
  • The patient experienced symptom resolution following surgical intervention.

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  • The contralateral (left-sided) tumor is currently under surveillance per patient preference.

    • Implications:

    • This case underscores the importance of considering rare neurofibroma presentations in patients with neurofibromatosis type 1.
    • Successful surgical management can lead to significant symptom improvement.
    • Highlights the need for individualized treatment strategies, including surveillance for unresected tumors.