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ONE MORE LOOK TO THE OLD CASE (4970)
ONE MORE LOOK TO THE OLD CASEnew
Subtitle: B08-5053
Type:
DERMA
Sender:
ugnius
2008-03-09 15:08
INCTR - EBMWG Hematopathology Online
This case is linked to HISTOPATHOLOGY group. Please take a look once more- some new stains and thoughts about this "Eo reach T cell proliferation with high Ki67 activity and high density of vessels".  
EXPANDED IH: The infiltrate: CD3+ CD2+ CD5+ CD7(sic!)(-), TIA1+, GranzymB/Perforin-/+, CD30-/+, Ki67 prolif. activity up to 40%; CD8/CD4 ~2/1; CD23+ FDC network is absent. EBV LMP1 is negative.  
IN PROGRESS: CD21, CD68, S100, CD1a, CD117.  
HISTO FEATURES: Coagulative slit like necrosis; Slight nodularity and angiocentrism with slightly pleomorphic spindle cell proliferation; mitotic activity and nuclear irregularities in T population even in medium cellular zones; PROMINENT eosinophilic infiltration.  
 
WORKING/QUESTION: ATYPICAL T PROLIFERATION: peripheral T cell lymphoma, cytotoxic immunophenotype?  
Thank you for collaboration.
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tzankov
2008-03-10 10:57
did you perform your additional stains on the originla slides or is this a new (lymph node?) biopsy? is there a clinical progression?  
 
on the original slide, the prolifeartive process extends deep inbetween muscle fibres, so that consider it highly suspicious of malignancy, but the exact classification is still not possible. dendritic neoplasms as well as T-cell lymphoma are the two differentials that should be first excluded... further unusal forms of Hodgkin lymphoma, Langehans cell histocytosis.  
 
ALK? EMA? CD45?
ugnius
2008-03-10 11:05
I've just noticed this case on iPath:) and decide rethink this case due to high proliferative activity, prominent irregularities of T cells, focal nodular architecture, slikt like necrosis, infiltrations of the muscle. Typical RS or H giant cells are absent. It's strange, that well visible spindle (FDC?) cells were still CD23-. So working diagnosis: suspition of peripheral T NHL, NOS.
Mueller-Hermelink
2008-03-11 18:26
The most important question in this case ( which is propably at the oral side of the cheek) concerns socalled traumatic granuloma RIGA-FEHDE, which is a awful mimick of PTCL of cytotoxic type and may be even clonal by PCR of TCR-Gamma.The localized aspect , central necrosis and some " differentiation" to small cells in the periphery , the high macrophage content are arguments for this diagnosis , which we try to exclude first in a biopsy from the oral cavity before diagnosing PTCL.
tzankov
2008-03-12 08:13
I think the idea of Riga-Fede's disease excellent, though most cases have been observed in childern, but I had the impression that this entity is now more and more considered lymphoproliferative, somethink like LyPap at special site... isn't it?  
See also:  
Alobeid B, Pan LX, Milligan L, Budel L, Frizzera G. Eosinophil-rich CD30+ lymphoproliferative disorder of the oral mucosa. A form of "traumatic eosinophilic granuloma". Am J Clin Pathol. 2004 Jan;121(1):43-50.  
Segura S, Romero D, Mascaró JM Jr, Colomo L, Ferrando J, Estrach T. Eosinophilic ulcer of the oral mucosa: another histological simulator of CD30+ lymphoproliferative disorders. Br J Dermatol. 2006 Aug;155(2):460-3.
ugnius
2008-03-12 09:01
Thank you a lot for ar idea and links. I will try to check TCR additionally and ask about DEFINITE location.
kunze
2008-03-12 16:06
Fede-Riga's ulcer is better compatible with the central necrosis and a focal angiocentric infiltration pattern than my previous assumption of a granuloma faciale. I expect the location in the oral mucosa can be confirmed.
torlakovic
2008-03-19 21:52
The lesion appears to have features of Riga-Fede's disease, but it should be considered that the site of presentation, the age of the patient, and lack of usual history of trauma are not typical for this rare entity. I have consulted our oral pathologist (Dr. Maria Copete) who suggested to use the term "atypical eosinophilic ulceration" ("atypical histiocytic granuloma"). As we expect this lesion to regress, clinical follow up will be critical to rule out a T-cell lymphoproliferative disease.  
However, I think that CD20 is not sufficient to disclose all B-cells and would suggest to use CD79a, Pax-5, and CD138 before you rule out a B-cell lesion. In oral mucosa, even B-cell lesions may be associated with large number of eosinophils.
ugnius
2008-03-20 14:43
Thank you for idea and for recommendations. The patient will be recruited for further evaluation and PCR clonality analysis. The lession was removed from inside ("lipoma like at first glance"). The history of trauma is unknown- it will be checked out.
ugnius
2008-04-03 10:31
NOTE: PCR reveals TCR OLIGOCLONALITY. The patient will be sent to the hemato dept for evaluation.  
Really it's a first similar case in my practice. Thank you for your well done job.  
IH: CD79a, Pax-5+ in the same population CD20+ cells (not digitalized).
ugnius
2008-04-07 20:12
PCR results: FULL BLOWN CLONALITY ON TCR BETA/GAMMA. The patient is admitted for evaluation in hemato dept.
ugnius
2008-04-21 18:17
Please find some accessory photos for this case. Thank you for attention and help once more.
schulze
2008-04-22 21:12
Sorry, but I do not understand, to which "old case" the present case is related to, where the biopsy was taken from, and what are the history of the patient and the clinical findings?  
The dense infiltrate of large clusters of "histiocytic" CD1a and S100 positive cells without dendritic morphology and many eosinophils let me think of Langerhans cell histiocytosis. However, clonality of TCR beta/gamma seems to speak against this diagnosis.
ugnius
2008-04-23 08:22
This case at first was presented in histopathology forum. At the second look it was not resolved completelly. So it was enreached with add photos and PCR was performed. Any previous history was known (trauma or smth else). The patient was directed to hemato dept., but here he refuses an evaluation at all. So follow up and further investigetion are questionable. Appologies for missunderstanding.
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Last modified: 2008-03-09 15:08:40