Case 701 -- A 49 year old male with headache and diplopia

Contributed by Somak Roy, MD, Ken Clark, MD, Ronald L. Hamilton, MD.


A 49-year old man presented with chief complaints of stuffy nose, worsening headache for the past 3 weeks and binocular diplopia which developed over a period of 4 days. Significant past medical history included hypertension and a biopsy performed from a right chest wall mass, approximately 1 year ago. Pathology report was not available at the time of presentation. General physical examination was unremarkable and neurological examination revealed signs of bilateral sixth nerve palsy.


A contrast enhanced computed tomography (CECT) of the head and maxillofacial sinuses revealed a very large irregular mass, 5.8 x 5.8 x 4.5 cm, which demonstrated fairly homogeneous enhancement centered about the clivus, sella and sphenoid sinus areas. There was complete obliteration of the sphenoid sinus with near complete erosion of the anterior clivus, and sella. The mass also invaded into the posterior superior portion of the nasal cavity, posterior portion of the right maxillary and posterior ethmoid sinuses, multiple portions of the sphenoid bone and petrous apexes. (RAD 1, 2 and 3). Serum ACTH, TSH and prolactin levels were found to be within normal physiological limits.


Smear preparation were cellular with large cluster of cells with few vessels running across and scattered single cells in the background (Image 1). Medium power shows slightly enlarged cells with somewhat plasmacytoid appearance, moderate amount of eosinophilic cytoplasm and finely clumped nuclear chromatin (Image 2). Touch preparation were paucicellular, however on closer examination revealed scattered cells with eccentrically placed nucleus, finely clumped chromatin, moderate amount of eosinophilic cytoplasm with peri-nuclear hoff in few cells. (Image 3).


Paraffin sections from the mass demonstrated fragments of respiratory mucosa and cellular tumor comprised of slightly enlarged cells with eccentrically placed nucleus, coarse clumped chromatin, scant to moderate eosinophilic cytoplasm with occasional cells demonstrating a peri-nuclear hoff (Images 4 and 5). A reticulin stain demonstrated scattered thin strands of reticulin fibers (Image 6). Immunohistochemical stain for synaptophysin (Image 7) and prolactin (Image 8) failed to demonstrate pituitary tissue. Tumor cells showed strong and diffuse cytoplasmic staining for CD138 (Image 9) and Kappa (Image 10). Lambda staining highlighted few scattered plasma cells (Image 11).


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