Sir,
Extramedullary plasmacytomas (EMPs) are localized tumors composed of monoclonal plasma
cells in an extraskeletal area constituting 3% of all plasma cell neoplasms. A 75-year-old
male presented with diplopia on horizontal gaze and headache for 1 month. His medical
history was unremarkable. He was a smoker for 35 years (pack-year: 105). He had complete
ptosis; dilated pupil (right 5 mm and left 3 mm); absent direct and indirect light
reflexes, and limitation of adduction, depression, and elevation in the right eye.
Fundus and rest of the systemic examination was normal. He had mild normocytic normochromic
anemia, with high erythrocyte sedimentation rate. Biochemical parameters showed mild
renal failure (serum creatinine – 1.6 mg%), reversed albumin/globulin ratio, and hypercalcemia.
Magnetic resonance imaging of the brain showed a lesion in the sphenoid sinus measuring
4.2 cm × 3.4 cm × 3 cm extending into the sellar and suprasellar regions and clivus
bone, which was heterogeneously hyperintense on T2 and isointense on T1 with contrast
enhancement [Figure 1a] and [b]. The lesion was extending to the bilateral cavernous sinus encasing the cavernous
portion of the right internal carotid artery for approximately 210° [Figure 1c]
[d]
[e]. Multiple focal lesions in the skull vault were seen. Skull X-ray showed multiple
lytic lesions [Figure 1f]. A transnasal biopsy of the lesion showed plasmacytoma [Figure 1g] and [h]. Further investigations for multiple myeloma showed a monoclonal gammopathy on serum
protein electrophoresis, Bence Jones protein in the urine, and 42% plasma cells in
bone marrow study. A diagnosis of multiple myeloma stage with EMD was made, and the
patient was started on chemotherapy with dexamethasone, cyclophosphamide, and bortezomib.
When followed up after 6 months, the patient had no headache or diplopia and had radiological
improvement (reduction in the size of the lesion). EMP generally displays a destructive
course. EMP can be localized in one region or occur as a component of a systemic plasma
cell dyscrasia. EMP associated with multiple myeloma has a bad prognosis when compared
to solitary EMP. Sphenoid sinus is an extremely rare localization for an EMP.[1] Sphenoid sinus lesions have multiple benign and neoplastic causes.[2] Hence, it is important for clinicians to be aware of such unusual presentations
of a common disease. This case highlights the need to consider EMP and coexisting
multiple myeloma in patients with sphenoid sinus lesions.
Figure 1: Magnetic resonance imaging brain showing a lesion in the sphenoid sinus
measuring 4.2 cm × 3.4 cm × 3 cm extending into the sellar and suprasellar regions
and clivus bone, which was isointense on T1 (a) and hyperintense on T2 (b) with contrast
enhancement. The lesion was extending to the bilateral cavernous sinus encasing the
cavernous portion of the right internal carotid artery for 210° (c). Susceptibility
images showing blooming (d). Coronal T2 image showing the lesion (e). Skull X-ray
showing lytic lesions (f). Hematoxylin and eosin staining showing neoplastic plasma
cells (g and h)
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