M. Silva*, JC. Ribeiro, J. Romão and A. Paiva
Abstract: Orbital Apex Syndrome is a very rare clinical diagnosis presented to ENT surgeons. Intraorbital extramedullary plasmacytoma is extremely rare and there are a few cases reports in the literature. Very unusually it develops a secondary leukemic transformation to a plasma cell leukemia which is a very rare and aggressive type of acute leukemia (in our tertiary hospital, Hematology Department had previously 3 cases in the last fifteen years).
Orbital apex syndrome is a rare but potentially fatal situation rarely seen initially by ENT surgeons. We report an even rarer case of an intraorbital extramedullary plasmacytoma that caused orbital apex syndrome, initially masqueraded by a complicated pansinusitis.
An immunosuppressed 78-year-old female presented with a progressive left side reduced best corrected visual acuity, pansinusitis and orbital involvement, imaging showed opacification of all paranasal sinuses, cavernous sinus involvement and left intraorbital mass lesion. After an initial improvement with antibiotics, sudden clinical deteriotion occurred. Biopsy of the intraorbital mass showed a monoclonal lymphoid neoplasm and laboratory findings led us to the diagnosis of plasma cell leukemia.
We advocate for a multidisciplinary approach ab initio in all cases of complicated rhinosinusitis to minimize or prevent deterioration of vision and optimize clinical outcomes.
Orbital apex syndrome is a rare but potentially fatal situation rarely seen initially by ENT surgeons. We report an even rarer case of an intraorbital extramedullary plasmacytoma that caused orbital apex syndrome, initially masqueraded by a complicated pansinusitis.
An immunosuppressed 78-year-old female presented with a progressive left side reduced best corrected visual acuity, pansinusitis and orbital involvement, imaging showed opacification of all paranasal sinuses, cavernous sinus involvement and left intraorbital mass lesion. After an initial improvement with antibiotics, sudden clinical deteriotion occurred. Biopsy of the intraorbital mass showed a monoclonal lymphoid neoplasm and laboratory findings led us to the diagnosis of plasma cell leukemia.
We advocate for a multidisciplinary approach ab initio in all cases of complicated rhinosinusitis to minimize or prevent deterioration of vision and optimize clinical outcomes.
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