28 yrs Lady have had complaints due to vaginal compression from 2004.  
2004-2009 multiple vaginal biopsies were taken ("cervicitis", "fibroma", "without tumor").  
2009: bilateral stents in ureters, disuria, painfull intercourse, bilateral hydronephrosis.  
MRI: tumoral masses spreading to cervix, vagina, bladder, uterus, maybe to rectum.  
After "blind" (?) administration of steroids urine-blocking symtoms are relieved.  
Any B or systemic signs of the lymphoma (LDH, etc.). Perfect general condition.  
 
HISTO/CONSULTATION CASE 2009/all biopsies 2004-2009 with the same changes: Focal infiltrates with "dermatofibroma like" or "muaring" intersticial fibrosis/sclerosis, consists of large- medium sized cells with apoptosis, irregular nuclei. Visualisation is not optimal due to sclerosis and artefacts.  
IH: CD20+; Bcl6+; Bcl2/CD10/Mum1(-); Ki67 ~50%.  
 
VIRTUAL SLIDES (APPERIO) attached (selected most informative biopsies 2008-2009). In a case of emergency routine jpg's will be placed in seconds.  
PROPOSAL: DLBCL, GCB type with prominent fibrosis/sclerosis.  
CLONALITY: pending.  
FOLLOW UP: R-CHOP/ DLBCL therapy is planning after full evaluation.  
 
SPECIAL QUESTS/COMMENTS: 1. Indolent course (gyn extranodal lymphoma?) due to sclerosis, imitating retroperitoneal fibrosis? 2. Differential with FL (age, no follicles, no FDC networks, etc?). 3. Scarse case reports and reviews on the topic of "sclerosing" or "retroperitoneal/GYN" sclerosing lymphomas.

tzankov 2009-09-18 14:25	From the slides we see I think that we can diagnose a mature sclerosing B-cell lymphoma. Considering the gender, the location (withn the pelvis), the "indolent" course and the Bcl6-positivity I would favor FL. I do not thinkt that the proliferative activity excludes FL.     Would you please add a Bcl6-stained picture and CD23 (or CD21) as well as CD30 and p63 (DD to "sclerosing primary retro-peritoneal lymphoma"/in analogy to PMBL)? FISH may bring some clarity as well, since PMBL are very seldom rearranged (BCL2 BAP).
ugnius 2009-09-21 10:37	PLease find additional Bcl6 and CD20. I will try p63. CD21/CD23/CD30 in toto negative.
hurwitz 2009-09-21 18:17	There is little doubt about the diagnosis of sclerosing B-cell lymphoma. BCL6 is an argument for FL. What about CD10, is it really negative ? A point worth mentioning is that the amount of Bcl6+ cells is much higher in the biopsy taken in 2009, than in the previous one (2008). This can be either a sampling problem, or a sign of tumor progression.
ugnius 2009-09-23 13:20	p63 negative.
ugnius 2009-09-30 10:06	PCR clonality: IgH/IgK CLONAL; TCR B/G POLYCLONAL.
tzankov 2009-09-30 10:26	The case is still classificatory difficult, the PCR results support what we morphologically diagnosed - namely a B-cell lymphoma with sclerosis. The main issue is how to call this lymphoma... some arguments (female, sclerosis, lower body part/reproperitoneum, Bcl6 expression) are in favor of follicular lymphoma (FL), some do not support the diagnosis (CD10 and Bcl2 negativity, lacking FDC), but it should be said that FL may spread outside secondary lymphoid organs and grow diffusely outside FDC and that especially FL grade 3 may be Bcl2-negative. Unfortunately due to the poor morphological quality we can not grade this case. Finally we come to Ki-67; its reactivity is very variable throughout the slide, which also supports the assumption of a possible "low grade" component.     Concluding, more of less the classification is of semantic importance since a diffusely growing FL, porobaly grade 3, should be called diffuse large B-cell lymphoma (DLBCL) and R-CHOP will not be false in FL as well (some would say is the best to be given to young patients, but this is another story). If nobody is willing to get any more material for exact diagnosis or to perform FISH, I would probably call this "B-cell lymphoma with sclerosis and moderate proliferation fraction, probably DLBCL or DLBCL transformed from FL".
ugnius 2009-10-01 10:21	Thank you one more time. Please find some jpg's attached. The block's quality is poor. The cells are deformed additionally due to prominent "muaring" sclerosis.
tzankov 2009-10-01 15:26	can you perform a Novotny silver stain?
ugnius 2009-10-01 15:31	GSPS or Gomori, if you prefer. We do not use Novotny.
tzankov 2009-10-01 16:34	gömöri
diane.c.farhi 2009-10-14 17:48	What does the word 'mauring' mean?
ugnius 2009-10-14 17:52	"Muar"- storiform stricture usually seen in dermatofibromas of the skin. An organisation of collagen fibres. In this case "fibrosis like in dermatofibromas".
hurwitz 2009-10-15 14:25	Thanks Ugnius for this extraordinary case. Thanks also for the informative discussion. The case can be closed. Please do regard Dr.Tzankov's last comment as the final diagnosis.
ugnius 2009-10-16 10:35	Appologize beeing late. Please find additional GSPS (reticulin/collagen) stain (dr.Alex request). Thank you once more time.
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