Painfull "Occipital neurofibroma" was resected for 59 yrs female with 2yrs (sic!!!) history.  
HISTO: Sinus and wedge shaped infiltrate of atypical T cells in lymph node.  
IH: CD3+ CD4+ GranzymB+ CD30+ ALK1/EMA-. Ki67 ~60-70%. The single PanCK+ cells. The block is exhausted due to low quality: LEFT material: CD23+ netwrok in FC; CD10-; CD43 and some stains from another block are pending.  
HISTORY: After bee bite (2 yrs before) the patient palpates occipital LN, soon after- oethr LN slowly grow up. Any skin damage before and nowadays is present. BLOOD and LDH: normal values; HEPATOSPLENOMEGALY: NOT; ESR 42; Cervical LN on CT scan: up to 0,6-0,8cm. Visceral nodes: normal. GI endoscopy: normal.  
BM biopsy: in the progress.  
PROPOSAL: Peripheral T lymphoma.  
QUESTION: The possibility of transformed mycosis spread to lymph node.  
WORKING/FINAL: Anaplastic large cell (T) lymphoma, ALK1(-) in lymph node.  
 
Thank you for participation.

Mueller-Hermelink 2007-11-27 21:10	Tissue distributionand phenotype would fit to an ALCL, Alk1-, although the CD 30 stain is quite weak . Differential would be nodal involvement by metastatic spread from cutaneous primary tumor. I believe that there could be some discussion whether to put it in this category or PTCL-NOS .The single keratin staining cells are inreaguing , but may be presentin l.n. sinuses . Whether these are tumor cells remains questionable.
ugnius 2007-11-27 21:21	Thank you. EMA(-). I request EBV additionally from next block. Appologies- it seems, that whole sections are flushed out in IH procedure (some stains). The quality of external blocks are low. Maybe its a reason for low reactivity for CD30...
ugnius 2007-11-27 21:22	The request about LN linkage with skin was sent.
diane.c.farhi 2007-11-27 21:42	Does this patient have a history of mycosis fungoides? If so, for how long and at what stage? Perhaps this is what you meant by linkage between skin and LN.
ugnius 2007-11-27 21:54	It was just a proposal. I hope we will get add info about this patient.
ugnius 2007-11-28 15:05	Operating surgeon do know anything about overlying skin (probably he is not dermatologist:)). It means maybe the lession is primary/nodal.
tzankov 2007-11-28 15:50	I agree with the differential of Prof. Müller-Hermelink, suggesting ALK- ALCL (T-cell) as the most proper diagnostic "drawer" for this lymphoma...     which "pancytokeratin" did you use? CK22? the panCK+ cell in the first picture is probably tumor cell, those in the 2nd and 3rd picture - probably denditic cells. considering the former, we observed single T-NHL and HL which stained positively with CK22 as well... so this is seldom, but possible.
ugnius 2007-11-28 16:26	Thank you. I'm agree that PanCK (MNF116 is I correct) is not a key. Maybe I can do something add for IH phenotype EMA-ALK1-?
tzankov 2007-11-28 17:05	yes, for example CD43
ugnius 2007-11-28 17:31	I'll be back soon with add stains. Thanx.
ugnius 2007-11-30 20:44	Please find EMA+ (30%), CD43+ (70%).
tzankov 2007-12-03 08:56	all, morphology and immunostains are in support of ALK- ALCL, but clinical history should also fit ("transformation of e.g. cutaneous T-cell lymphoma...?)
torlakovic 2007-12-12 00:34	I completely agree with Prof. Mueller-Hermelink. I do not think that panCK weak cytoplasmic reactivity adds or subtracts to this even if it would be present in much larger number of cells than in this case. Some of the CK+ cells are clearly usual CK+ dendritic cells, while some may really be tumor cells. With the level of sensitivity that your lab is operating at, you probably see some panCK in many different type of cells, in particular various tumors.
ugnius 2007-12-20 12:39	The last point: CD15+ :) Unfortunatelly ANY OTHER SIGNS of aggressive lymphoma were not found in staging procedure. I've retrieved clin data about the patient loceated in more friendly/closed to us hemato dept.
ugnius 2007-12-20 13:25	Please find some clin data (2 yrs history). I will be back soon with BM data. Thank you for attention.
ugnius 2007-12-21 19:00	BM: reactive changes (not included). Please find some NEW photos from recutted HE with beautifull panoramic view with small tumor cells and macrophageal rings... Thank you all for consults.
hurwitz 2007-12-29 21:27	Sorry to enter the discussion at this late stage. A point which has not been discussed is the focal positivity for CD15.   According to two references I found, it has been observed: {PMID:14657710}, and {PMID: 12527929}.   I wonder what is the experience of our collegues?
torlakovic 2007-12-30 01:48	CD15 positivity in ALCL is in my experience rare and I have seen only one such case (but almost all cells expressed CD15). Since this case has only focal CD15 expression, I would check if these cells are CD3-positive or not. If not, I would try to do EBER and Pax-5 to rule out focal Hodgkin phenotype superimposed on ALCL since that has been described as a rare event in other types of PTCL. Also unlikely (but to continue academic discussion, something to think about if there is any morphological difference between CD15+ and CD15- cells: Consider that small focus of metastatic carcinoma with undifferentiated morphology can be EMA+/CK+/CD15+ and you have such cells in this biopsy.
hurwitz 2008-01-16 20:39	Final diagnosis,and confirmation of the submitter's diagnosis:     Anaplastic Large Cell Lymphoma, ALK1- ;   focal positivity for CD15+     Thanks for submitting and discussing this case and raising the issue of CD15 positivity in AlCL.
ugnius 2008-02-12 11:16	The last point: it was decided to carefull "watch and wait": ANY SIGNS of systemic lymphoproliferative disease was not found. Skin lession was absent too.
ugnius 2008-03-04 11:10	The "last last" comment: PCR results: IgH polyclonal; TCR beta: POLYCLONAL; TCR gamma: MONOCLONAL.
hurwitz 2008-03-04 13:36	Thank you Ugnius, now we have the final proof for the T-cell origin of this tumor.
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