60 yrs female underwent tumor resection from duodeno-jejunal junction muscular wall.  
MACRO: 14 cm small intestine with circumscribed, lobated, broght borwn 6,5x3x6 cm tumor.  
HISTO: Biphasic tumor in muscularis propria, well circumscribed, with lobation/spetation with small infiltrative nidus closed to main mass. 1 zone: Castlemann like areas with follicles with FDC whorls and interfollicular atypical spindle cell spread; 2 zone- more aggressive, epithelioid solid and spindled cell tumor. Mitoses ~6/10 HPF. In tumor periphery (subserosa), sinuses still present (lymph node spread).  
Original diagnosis: Malignant mesenchymal tumor, most probably GIST with high malignant potential): CD117 (-)(sic!), CD34/ASMACTIN/DESMIN/S-100(-); VIMENTIN (+).  
REVIEW AND EXPANDED IH in 2009 under request of GIST registry as CD117 negative GIST.  
IH SUMMARY: VIMENTIN+, CD68+ (epithelioid), CD23-/+ (single cells in epithelioid), CD21+, PanCK(-), EMA+. ALK1/DESMIN/CD34/CD117/CD20/CD3/EBV LMP1/AsmACTIN(-). Ki67 highest in epithelioid cell component ~ 40%.  
VIRTUAL SLIDES: Pending.  
PROPOSAL: Jejunal folicullar dendritic cell sarcoma (with epithelioid (high grade) and Castlemann/IMFT like (low grade) areas).  
 
SPECIAL QUEST: Castlemann lymphadenopathy like tumor component with interfollicular interstitial tumor cell population, histo and IH different (CD68-/+; CD21/CD23/S100-) from main epithelioid tumor mass (CD68+; CD21+; CD23-/+; S100-): unclassifiable dendritic cell component? FDC component IMFT like?.  
 
Series of these 2 cases are dedicated to Basel hematopathology team. It's pleasure be with you on the web.  
 
 

tzankov 2009-11-16 12:49	Compared to the former case, this one is in my opinion much more complicated. Could you add a CD5, Cyclin D1 and Kappa/Lambda stained slides since I can not understand why there are Castleman-like changes here and I fear overseeing a lymphoma? Would you like to show us the expression of CD21 in the spindle cell component? Thank you!
ugnius 2009-11-16 14:53	Thanx. CD21 added. DLBCL will be uploaded in minutes...
dirnhofer 2009-11-17 10:30	in my opinion, the diagnostic clue is the epitheloid tumor (probably fdc sarcome, myelosarcoma.....) the cd-like reaction in the vicinity (ln?) is probably reractive (but lets see the stains recommended by alex.     the former case is a fdc sarcoma
ugnius 2009-11-17 10:47	Thanx. The point is, that: 1) Castlemann like zone is located around tumor closed to MUCOSA (so it cannot be a lymph node); 2) In part, in subserosal location we can imagine, that the lession BEGINS/INVOLVE the mesentery nodes. But it just hypothesis.
ugnius 2009-11-21 18:32	Please find attached: 1) additional virtual slides; 2) CyclinD1+ in epithelioid part photo; 3) Bcl2+ infiltrative FDCs and Bcl2- epithelioid part of the tumor; 4) EMA dot like+ in epithelioid component.   It seems that diagnosis fit "FDC sarcoma with IMFT-like low grade component in jejunum".
ugnius 2009-11-21 18:32	Please find some case reports attached.
leoncini 2009-12-22 12:46	there is not doubt of Castleman like lesion Do you stain for HHV8, less clear is the epitheloid component   I cannot give any judgement on these images I think in this case I would like to see the original sections
ugnius 2010-05-24 15:09	The case was sent to external consultation to dr.A.Tzankov and dr.S.Dirnhofer (Basel, Swiss). Additional IH markers in the tumor: DOG1(-), CD43(-).   THE FINAL DIAGNOSIS: JEJUNAL FOLLICULAR DENDRITIC CELL SARCOMA. The case was presented in 7th Baltic Hematology congress in tartu, Estonia.   Thank for fruitfull discussion!
tzankov 2010-05-25 06:54	The case can be closed now.     The final diagnosis is     JEJUNAL FOLLICULAR DENDRITIC CELL SARCOMA     Thanks to Ugnius for submitting this interesting case and to all the participants for their comments.
Kamau 2012-07-05 10:49	Very helpful especially the well chosen and prioritized immunohistochemical stains. I have learnt a lot
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