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Uncertain disseminated process (angiosarcoma?) in the BM (755450)
Uncertain disseminated process (angiosarcoma?) in the BMnew
Subtitle: B16-20276
Type:
HEMATO
Sender:
ugnius
2016-06-22 15:00
INCTR - EBMWG Hematopathology Online
CASE IS READY. I'M SORRY FOR ABSENSE OF "CONFOCALLITY" (FRAGMENTATION OF THE SECTIONS):  
 
61 yrs female (MD) with aggressive clinics with suspition of the disseminated neoplastic process (vertebral fractures, radiologic multiple focci in the liver, adrenal, spleen, supraclavicular nodes. NO BIOPSIES before Exitus, except isolated THREPINE (below).  
CLINICS: weakness, bed regimen only, icterus, hepatic and pulmonary insuficiency, ECOG 3, cutaneous haematomas, edema of the legs, hydrothorax, DIK sindrome, etc... Shortly after threpine biopsy- Exitus letalis in the outer clinics (further data unknown).  
BLOOD: WBC 6,46; Anemia (E 1,96; Hb 64), throbocytopenia (T 27), Kreatinine 30, Bilirubin gen. 350,5; LDH high: 1506. Blasts in the aspirate not found (diluted specimen).  
In 2015 herpetic eruption in the chest and legs (before main disease course).  
TREATMENT: pallation, blood transfusions.  
CLINICIAN OPINION: Nothing definite.  
 
HISTO: a) Panhyperplasia of BM, massive erythroid hyperplasia/dyserythropoiesis;  
b) Reticulin and collagen fibrosis MF2 up to 70% space with "blastoid" cells clusters up to 25% of population and "retiform" or spindled fibroblastic proliferation, focal neoosteogenesis;  
 
IH: a) "blastoid" cells in the fibroblastic areas: CD34+; Bcl2+; CD10+ (?); MPO/CD68/CD123/CD117/HLA DR/TdT(-); Pax5/CD3(-);  
b) Spindle cells in myxoid background ERG+, CD34/CD31+ focally.  
NO EPITHELIAL tumor cells detected.  
 
 
PROPOSAL: Acute panmyelosis/myelofibrosis (AML subvariant)???  
Extramyeloid ANY kind of tumor/proliferation (myueloid sarcoma? other tumor/mts?) cannot be excluded.  
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tzankov
2016-06-22 17:49
I vote against APMF... the clinics and the morphology do not fit. Can you stain for mastcelltryptase and plasmacell-markers and ERG? can you please provide a high-resolution of CD34?
ugnius
2016-06-24 17:42
CD117+ mastocytes only scattered. ERG totally positive in the most cells (in heametopoeitic too): angio proliferation was disucussed, but not definite... I will proceed with 40x, CD117 and ERG (restricted in our centre still...). Thanx.
ugnius
2016-06-24 17:47
CD138+ conventional clusters NOS. I eill append too. Really I have no other ideas (sad story).
tzankov
2016-06-24 20:48
Plesse attach some illustrative Material on erg, tryptase and CD 138
ugnius
2016-06-28 07:36
Please find some adds: CD138+ usual pattern: perivascular/interstitial and sporadic in spindle areas; CD117+ scattered cells (the same with mast cell tryptase). ERG diffuselly positive, except erythroids.  
The spindle population (in part CD34/CD31+) HHV8(-).
tzankov
2016-06-28 10:07
I end with an angiosarcoma. The clinics fits perfectly, the morphology fits (more or less) and the phenotype fits...
ugnius
2016-06-28 10:19
Tak. It not seems to be very "high grade". Probably splenic? I will append some enlargements from spindle cell zone with CD34... Thank you a lot for idea.
ugnius
2016-06-28 10:41
Please find "blastic" and "spindle" zones with CD34+. Yes, if we regard "blastoid" cells as angio, it fits to high grade angiosa- all discohesive around vessels and weaker CD34+...
tzankov
2016-06-28 17:29
thank you.  
the additional images strengthen my assumption of angiosarcoma
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Last modified: 2016-06-28 10:28:12