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Recurrent orbital malignant peripheral nerve sheath tumor 18 years after initial resection
A D Morton1, V M Elner, B Frueh
1Department of Ophthalmology, University of Michigan, Ann Arbor 48105, USA.
Ophthalmic Plastic and Reconstructive Surgery
|January 16, 1998
Summary
This study reports the youngest patient diagnosed with malignant peripheral nerve sheath tumor (MPNST), an aggressive orbital neoplasm. The patient achieved long-term survival after treatment, offering hope for rare cancer cases.
Area of Science:
- Ophthalmology
- Neuropathology
- Oncology
Background:
- Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive orbital neoplasms.
- These tumors typically affect adults and spread along nerves, often leading to a poor prognosis with a 5-year survival rate.
- Orbital MPNSTs commonly originate from sensory branches of the trigeminal nerve.
Observation:
- A 23-year-old male presented with rapid, posttraumatic left orbital proptosis and pain.
- Histopathological comparison of current tumor biopsy with specimens from an incomplete excision 18 years prior confirmed recurrent malignant PNS tumor.
- The patient's initial tumor presentation was 18 years before the current diagnosis.
Findings:
- The patient was diagnosed with malignant peripheral nerve sheath tumor at an unusually young age.
- Histopathology confirmed the tumor as malignant PNS tumor, consistent with a previous diagnosis from 18 years prior.
- The patient remains alive without recurrence 8 months post-orbital exenteration.
Implications:
- This case represents the youngest documented occurrence of an orbital malignant PNS tumor.
- The patient's survival marks the longest documented survival for an orbital malignant PNS tumor.
- Findings suggest potential for long-term survival in select orbital MPNST cases with aggressive management.