STORY: 56 yrs old Lady (russian citizen) underwent full hysterectomy with adnexa due to clinical diagnosis "ca ovariorum, ascites, hydrothorax, mts ad hepar". Main symptoms were: ascites, hydrothorax, dyspnoea. CA 125: 412. Gastric endoscopy: normal. Sonoscopy: cystic changes in ovaries and "carcinomatosis" in omentum.  
SURGERY PROTOCOL: Tumor masses in both ovaries with cystic changes and destruction. Nodular deposits in parietal peritoneum (0.5cm), omentum, liver (2cm nodule), paraaortic lymphadenopathy. Multiple jejunal adhesions.  
BLOOD: WBC 17.6; PLT 651; RBC 3.61.  
HISTO: Cervix normal. Myomata. Single mural lymphoma infiltrate. Endometrium resting. TUMOR: difusse infiltrate of small- medium calibered, darka and light "blastoid" cells in endometrium, myometrium. The tumor cells with ovoid, irregular, angulated nuclei, multiple mitoses, minimal cytoplasm. Both adnexa and omentum: Identical infiltrates in tubal mucosa, muscular layer, subserosa. Focal "starry sky" areas and "clearer" nodules/zones. Intravascular spread and prominent angiothropism.  
IH: CD20 (+/++) 95%, CD3/CD5(-), CD10(++/+++) 95%, BCL6(+) 30%, MUM1(+) 5%, Ki67(+++)95%, CD23/CD30(-), EBV LMP1(-), TdT/CD34(-), Bcl2(+++) 100%. CD23+ FDC network isa absent. Slight nodularity in central zones of the infiltrate may be caused in part by artefacts, in part infiltrated structures (vascular spaces, follicles, etc.).  
FOLLOW UP: Soon after surgery and preliminary pathology report (with URGENT SITUATION mentioned) issuing the patient moves to Russia/Moscow for further treatment due to economical reasons. Some days before she falls into the coma (CNS involvement???) and after words of the relatives "leukaemia" was additionally detected and "supportive chemotherapy" was administered. The material will be sent to the Russia after request. Direct contact with hematologists here is absent.  
VIRTUAL SLIDES ARE APPENDED.  
 
PROPOSAL: Diffuse aggressive B lymphoma with "blastoid" (TdT negative) and Burkitt's lymphoma features (retaining FC/FL immunophenotype CD10+; Bcl6+/-; Bcl2+; Mum1-): diffuse large B cell lymphoma with Burkitt like features, most probably double/triple hit?  
 
PROPOSED LITERATURE: 1. Diffuse blastoid B-cell lymphoma: a histologically aggressive variant of t(14;18)-negative follicular lymphoma. Chiu A, Frizzera G, Mathew S, Hyjek EM, Chadburn A, Tam W, Knowles DM, Orazi A. Mod Pathol. 2009 Jul 24. [Epub ahead of print].PMID: 19633642  
2. Blastic/blastoid transformation of follicular lymphoma: immunohistologic and molecular analyses of five cases. Natkunam Y, Warnke RA, Zehnder JL, Jones CD, Milatovich-Cherry A, Cornbleet PJ. Am J Surg Pathol. 2000 Apr;24(4):525-34. PMID: 10757399 [PubMed - indexed for MEDLINE]  
 
Thank you for being 2gether.

diane.c.farhi 2009-08-17 21:00	I agree with your description and evaluation. However, I think I would rather use a diagnosis like 'high-grade B cell lymphoma with features of Burkitt lymphoma' rather than a transformation of follicular lymphoma, since we don't have good evidence of prior or concomitant follicular lymphoma. Clearly this patient is doing poorly and treatment as Burkitt lymphoma would probably give her the best chance of survival.
hurwitz 2009-08-18 14:19	This is a highly aggressive B-cell lymphoma with features of BL. Morphology and immunnophenotype (in particular the almost 100% Ki67 rate) are highly suggestive for the, in the recent WHO classification introduced provisory entity DLBCL intermediate to BL.
ugnius 2009-08-18 15:49	Thanx. Unfortunatelly I cannot check the molecs right now there... DLBCL with Burkitt features was formulated. Hopefully I will get a feedback abot consequences, provide my mobile to hematologists in Russia, but they just requested blocks/slides only at the moment...
SergeyN 2009-08-20 09:38	It seems CD44 could be used for discriminating MYC status (see http://www.infectagentscancer.com/content/4/S2/P6).   In children it seems to work well.     I'm a bit afraid to ask about HIV possibility, surely, there could be no surprises from this side?
tzankov 2009-08-21 06:21	In my daily routine I would probably call this case "aggressive B-cell lymphoma with features intermediate between Burkitt and diffuse large B-cell lymphoma" and perform a MYC FISH. Of course an FL can transform in a Burkitt-like manner, but there should be evidence or a clinical history of FL.
ugnius 2009-08-21 10:29	Thanx. The short description of surgery findings (above) shows widelly distributed and "irresectable" visceral tumor. Any responses and feedback from Russia up to date (this link was provided to hematologists here).
torlakovic 2009-08-30 05:57	I personally do not like this morphology for BL. Also, a strong expression of Bcl-2 is conceptually wrong for BL. This lesion may have double hit (IHG;BCL2 and any of BL-type translocations involving c-myc), but some of such cases may show c-myc amplification, or no involvement of c-myc at all. If this lesion looked more uniform like BL, I would favor "aggressive B-cell lymphoma with features intermediate between Burkitt and diffuse large B-cell lymphoma" , but some of the cells look very large and very irregular so I favor DLBCL with high proliferation rate. It may be that it is fixation or how morphology/cytology shows as presented on images here. I am not sure that you can really fully characterize this lesion without complete molecular work up.
hurwitz 2009-09-09 22:22	The diagnosis of aggressive B-cell lymphoma with features intermediate between DLBCL and Burkitt's lymphoma has been favored in most comments. Tumors belonging to this category can express bcl2 in contrast to Burkitt lymphoma which are negative for bcl2. The large seize and irregularity of tumor cells does not exclude this diagnosis. In a recent diagnostic review of lymphomas in Africa, we have seen such cases with considerable variations in cell seize containing rather large cells.
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