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Hematological malignancy (2664)
Hematological malignancynew
Subtitle: Frontal and ethmoidal sinus infiltration
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semir
2006-11-25 13:06
INCTR - EBMWG Hematopathology Online
69-years-old Bosnian woman was admitted two weeks ago at the Department of Neurosurgery, Clinical Center Sarajevo, Bosnia and Herzegovina because of the tumor mass that rapidly had developed within several months and infiltrated her frontal and ethmoidal sinuses (radiology scan revealed osteolytic mass, not shown here). Intraoperativelly, the surgeon removed well-vascularized, infiltrative tumor mass that invaded not only both sinuses but also dura.  
Microscopically, the tumor was composed of large, polygonal cells with typical oncocytic appearance. The tumor cells are arranged in follicles, trabeculae and solid areas. The nuclei are oval and round with prominent nucleoli. The mitotic index is quite low (<4 mitoses/10 Hpf). Focally, the tumor cells contained intranuclear hyaline inclusions. The tumor infiltrated the adjacent bone structures.  
Immunohistochemistry: CD56 (-), CD138 (-/+), Chromogranin (-), Synaptophtysin (+/-), Cytokeratin AE1-AE3 (+/-), IgG (+), Plasma cell (+).  
The patient has Bence-Jones proteins in her urine. (related to tumor?)  
Differential diagnosis: plasmacytoma, oncocytic carcinoma or pituitary adenoma?  
 
imported on 2006-11-25 13:06:14 originally owned by semir
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hurwitz
2006-11-26 23:00
Dear Semir, I assume you wanted to create a referral from the histopathology group. If you agree I will ask the webmasters to delete this case and create a new referral.
kurt
2006-11-27 11:47
Just as a note: A "referral" is a link to your original case in the histopathology forum. Thus, you don't have to upload a case twice and members from the hematology forum can access the original case in the histopathology forum.  
 
When uploading cases to several group it will become confusing to keep track of different comments
torlakovic
2006-11-27 19:41
Dear Semir,  
Please do kappa and lambda, CD79a, as well as MUM1. I do not know what do you use as "plasma cell marker" (VS38c?, can be positive in epithelial tumors just like CD138), but CD138 was not really convincing and at best, even if considered weakly positive, it is not a specific marker to support plasma cell diff. Are those intranuclear hyaline incusions or macronucleoli? Hard to tell from images. Other than BJ in urine, what was found by serum electrophoresis? Is your IgG really positive or negative? From the image, it seems that the largest cells with plasmacytoid features are not staining. Extramedullary plasmacytoma of head and neck region may rarely cause extensive dectruction of the bone and extend intracranially. If you cannot demonstrate unequivocal plasma cell phenotype, you may consider other "plasmacytoid" tumors including metastatic hepatocellular ca, and many others, but would not go there before you do other plasma cell markers.  
aorazi
2006-11-27 19:52
I would favor a pituitary adenoma or perhaps other epithelial tumor with similar morphology (e.g. metastatic renal cell carcinoma). Agree with Ermina, immunohistology need to be completed. Please add CD45, S-100 and a better keratin cocktail with at least CAM5.2 in it. In addition specific markers for pituitary adenoma strongly suggested. In conclusion, I think this is pitutitary adenoma. The "aberrant" reactivity with VS38 and CD138 does not bother me at all (since it seen very often).
semir
2006-11-28 21:33
I have just added additonal immuno we performed. Lambda was positive, kappa (-/+) whereas Ki-67 and CD79a were negative. p53 expression was very low (3-4% positive cells).
anpo
2006-11-28 22:01
I agree with the previous comments - the pictures do not favor plasma cell tumor. Staining for lambda seem to be strong extracellularly not in cells. CD138 is weak and negative CD79a also suggests other than plasma cell origin. I would suggest to perform a bone marrow biopsy - if the patient has BJ protein in the urin she may have two diseases.
Mueller-Hermelink
2006-12-01 16:44
I agree with the previous comments. this is probably an endocrine tumor , especially all the comments on specificty of markers as discussed by Dr. Torlakovic would fit with my experience.The synaptophysin reaction seems particularly specific. Just two further remarks : Concerning the light chain reaction ,it should be correlated with the light chain in the BJ protein. The staining is all extracellular and not specific for the tumor cells. Furthermore, in the differential diagnosis to pituary adenoma would be a paraganlioma , especially if the keratin is considered not specific and probably negative. What is the S 100 stain for sustentacular cells?
SergeyN
2006-12-02 15:31
I, too, would say that lambda doesn't stain cells themselves. If you use DAKO polyclonal antibodies, backgroung lambda staining is often stronger, by the way. Sometimes the things become clearer if the staining is repeated with more diluted antibodies. And I join others with the question if B-J proteins are lambda, too.  
 
Well, B-J proteins should be explained in any case. Range of differential diagnoses is narrow and includes plasma cell dyscrasias (MGUS/myeloma/plasmacytoma), B-CLL/immunocytoma and amyloidosis (paraneoplastic included). Standard check-up for all three seems to be indicated to exclude the faint possibility of a second tumour.
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Last modified: 2006-11-25 13:06:14