Evolution Of An Orbital Apex Tumor Over A Decade
Ugurlu, S., & BARTley, G. B. (2004). Evolution of an orbital apex tumor over a decade. Ophthalmic plastic and reconstructive surgery, 20(1), 75–77. https://doi.org/10.1097/01.IOP.0000105740.40210.66
View Original Source →Abstract
We report the evolution of an orbital apex tumor that underwent spontaneous regression, followed several years later by rapid growth and loss of vision. A turkey farmer had a right apical lesion, initially thought to be an optic nerve meningioma. The mass spontaneously regressed over 2 years, with marked improvement of vision. Eight years later, the patient presented with loss of light perception and severe proptosis of the right eye from a massive orbital tumor that extended in the cavernous sinus. An eyelid-sparing orbital exenteration was performed. The pathologic diagnosis was diffuse large cell lymphoma. This case demonstrates that non-Hodgkin lymphoma of the orbital apex may resolve spontaneously or it may enlarge rapidly, degenerating into more aggressive forms.
Case Details
Disease Location
Right orbital apex frontal lobe
Personal Characteristics
76 -year-old male farmer was examined 10 years earlier for proptosis of the right eye
Clinical Characteristics
Presented with a recurred proptosis of the right eye with loss of light perception original proptosis presentation 10 years earlier yielded a visual acuity of counting fingers od and 20/20 os. Exophthalmometry was 29mm od and 24mm os. There was swelling of the right optic disc with mild atrophy. Original MRI found an ill-defined mass within the right orbital apex that extended through the superior orbital fissure in the right cavernous sinus. Separate areas of enhancement along the planum sphenoidal and right frontal region were noticed. The lesion was thought to be compatible with a right optic nerve sheath meningioma. The patient was regularly followed up clinical findings at two follow-ups with 6 months intervals were unchanged 2 years later after original presentation showed significant improvement. Due to resolution of initial findings, meningioma was less likely and inflammatory cause was considered. There were no follow-ups for 8 years after the 8 year period the patient presented with the severe proptosis that developed during the previous 4-6 weeks. Visual acuity was no light perception od and 20/20 os. Exophthalmometry was estimated to be 40mm od. MRI showed a massive orbital tumor that extended through the superior orbital fissure in the right cavernous sinus the eye was removed and specimens were taken eyelid-sparing exenteration was performed pathologic sections showed CD20+ and CD45+ large b-cell lymphoma patient was treated with chop, no radiotherapy, and remains tumor-free during the last follow-up 9 months later
Remission Characteristics
2 years after original presentation, the right eye showed significant improvement. Visual acuity was 20/50, and proptosis had regressed 4mm. MRI showed the degree of enhancement at the orbital apex was less and the abnormal enhancement along the planum sphenoidale and inner table of the right frontal lobe had completely resolved after treatment, patient remains tumor free
Treatment & Mechanisms
Proposed Remission Mechanisms
Growth factors or cytokines, induction of differentiation, hormonal factors, elimination of a carcinogen, tumor necrosis, or angiogenesis inhibition, psychological mechanisms, apoptosis, or epigenetic mechanisms. Decreases in immunosuppressants or after h. Pylori eradication no specific mechanism proposed for this particular case
Clinical Treatment
Eye removal 6 cycles of chop (cyclophosphamide, doxorubicin, vincristine, prednisone)
Non-Clinical Treatment
None reported mentioned