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Mediastinal tumor (2291)
Mediastinal tumornew
Subtitle: LDHD, NSHL(syn) med.DLBCL?
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Tiina
2006-09-05 13:09
INCTR - EBMWG Hematopathology Online
34y.female. During two month fatigue, fever, dyspnoe, cough, pain in the left part of thorax.  
Objective: enlargment of lymph nodes in left supraclavicular, neck and axillar regions.  
CT: Nodular tumor, predominantly in upper part of mediastinum(8,5 x 12,5 x 11,0), with continual spread to supraclavicular and neck regions. Involvement of left pumonary hilus, compression of blood vessels and bronhci, bilateral hilar lymph node enlargement and fluidothorax (cyt: eosinophilia, no ly-ma elements).  
Blood values normal, sligth lymphopenia.  
Clinical chemistry normal;  
HIV neg; EBV is not performed.  
Supraclavicular lymphnode(2,2x1,3)investigated:  
Ln is obliterated with nodules and sheets of large,LCA/ALK/CK- and partly CD20/30/15/EMA+, pleomorphic,highly atypical neoplastic lacunar cells, admixed with variable amount of irregularly arranged fibrotic bands and blood vessels.  
Clonality investigations(PCR)are performed, results are not available up to present.
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ugnius
2006-09-05 14:08
Congratulations, dr.Tiinam, with the first presentation here.  
Proposal: cHL NS (II), sincytial variant
ugnius
2006-09-05 14:14
Question: has anybody have diagnosed LD cHL nowadays?
Tiina
2006-09-05 15:09
Thank you for comment. I think that most of them are NHL of anaplastic or pleomorphic morfology or lyphocyte depleted variants of NSHL. I don´t have enough experience, but may be some immuunosupressed pt-s(HIV, treatment, etc.).
ugnius
2006-09-05 15:30
The last our similar case was No 87348 (mediastinal tumor) in this group. Maybe BOB.1 and Oct2 would be of value in such cases(we have not these Ab yet in our armor)...
Tiina
2006-09-05 15:35
We don´t have them.
nurija
2006-09-05 19:12
I agree fully with your diagnosis (including morphological and immunohistochemical features).  
However, the fixation does not seem to work properly.  
 
nurija
2006-09-05 19:14
Actually, I agree fully with with Micug's diagnosis (including morphological and immunohistochemical features).  
Mueller-Hermelink
2006-09-05 23:21
Although I think that the discussion is very good I have still another opinion coming from the fact that there is a very highly proliferating blastic CD20 positive but CD30 negative component and a low CD3 positive background . Therefore I believe that this is a composite primary Large B Cell Lymphoma of the mediastinum with composite foci of Hodgkin's Disease or at least including Hodgkin's Cells. The biologic vicinity of HD and PMBL is proven by gene expression and these composite or gray zone lymphomas are not so rare in my consultation.Our Clinicians are treating these cases like PMBL including Involved fiels radiotherapy by polychemotherapy, since the larger tumor cells are CD 2o + and the patienbts may benefit from R- based CHOP or equivalent regimens.
Mueller-Hermelink
2006-09-05 23:27
I forgot to mention that coexpression of CD 30 is a regular finding in PMBL and that the diagnosis might be further proven by a positive CD23 stain.EMA may be rarely positive in HD cells but also in DLBCL and is not really helpful in the differential diagnosis.
Tiina
2006-09-06 09:13
Thank you for comment. That was the first impression I got, looking this case first time. That is why I odered also B-clonaliti investigations, unfortunately we can get results with delay. FLOW cytometry from lymph node cell-suspension did´t reveal B-cell clonality(we have to mention that we can prove clonality by FC very rarely in cases DLBCL).
anpo
2006-09-06 10:35
I agree with Prof.Müller-Hermelink that the first choice would be Primary Mediastinal DLCB. I would not make a diagnosis of Hodgkin in this case, although there are "grey zone cases" (see Am J Surg Pathol. 2005 Nov;29(11):1411-21).
hurwitz
2006-09-06 15:03
I as well would primarily opt for a PMBL, perhaps with composite HL. However, the few cases of composite PMBL and HL, I have seen, both parts were completely seperated, with areas of pure PMBL and others of HL, in the present case both components seem to be intermingled. I would like to ask if some one can comment on this.  
If you do not nimd a suggestion regarding immunohistochemical technique, your hematoxillin stain is rather week, a stronger nuclear staining will allow to appreciate the nuclear structure of the positive cells in order to recognize the type of the positive cell.
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Last modified: 2006-09-05 13:09:49