iPath 2
"Haemangioendothelioma" of the Spleen
51 yrs old male underwent spleenectomy due to tumor.
CT: 6x4.5x5cm vascularized tumor with irregular contrast accumulation. Angiosarcoma? 0.9cm nidus in liver
S2 segment (most probably haemangioma).
MACRO (FIXED): 217g and 17x9x2,5 cm spleen. In the hillus- irregular 4,5x3 cm greyish- white tumor
(photo is appended).
HISTO: on the slides.
IH: CD31/CD34 (+++)100% (more sharp than in the spleen); vWF(++) 70%; Vimentin (+++) 100% (tumor
cells+ < spleen+); CD68(+) 30% (<< in sinusoids); CD21(-).
Ki67 (++) 5%; PanCK (+) 10%; CD8 (-)(littoral cells in the spleen +); Cam5.2(-).
PROPOSED DIAGNOSIS: Splenic angiosarcoma, low grade ("haemangioendothelioma" formerly)?
B08-33530_0005.jpg B08-33530_0006.jpg
TUMOR 1.jpg Fibrotic 1.jpg
"Haemangioendothelioma" of the Spleen 1
iPath 2
Sattellite 1.jpg Vein 1.jpg
Vein 2.jpg TUMOR 2.jpg
CD8 tumor 2.jpg CD8 tumor 1.jpg
"Haemangioendothelioma" of the Spleen 2
iPath 2
CD31.jpg CD31 tumor.jpg
CD31 border.jpg Vimentin.jpg
CD34.jpg Fibrotic 2.jpg
"Haemangioendothelioma" of the Spleen 3
iPath 2
TUMOR 3.jpg CD68 a.jpg
CD68 b.jpg CD68 border.jpg
CD68 SPLEEN.jpg CD68 TUMOR.jpg
"Haemangioendothelioma" of the Spleen 4
iPath 2
CD31 panorama.jpg CD34 panorama.jpg
vWF panorama.jpg
Annotations
bvrugt (2008-12-03 11:37):
Although I miss the small signet-cell-like vascular tumor cells, the morphology with epitheloid cells and mild
atypia in combination with the IHC is consistent with an epitheloid hemangioendothelioma / low-grade
angiosarcoma of the spleen.
kunze (2008-12-03 12:23):
I agree with you and Dr. Vrugt: the gross appearance, the different growth patterns and the
immunohistochemical results (CD31/CD34 + 10% PanCK) justify the diagnosis of an epithelioid splenic
angiosarcoma.
micug (2008-12-03 12:33):
Thank you. It's unusual to find out very low proliferative activity (Ki67 5%). After description in the literature
this kind of diagnosis is lethal (moths), if we have obviously malignant vascular tumor. The exception is
"haemangioenthotelioma"- more localalised- but does it exist at all?
bvrugt (2008-12-03 15:51):
There are several reports that suggest a better survival with hemangioendotheliomas than with the typical
high-grade angiosarcomas of the spleen: Am J Surg Pathol 1992;16:785, Arch Pathol Lab Med
1992;116:1079, and Arch Pathol Lab Med 1995;119:755.
micug (2008-12-03 18:06):
Annotations 5
iPath 2
Thank you once more. I'm worried a little about nidus in liver ("hemangioma").
drkaycee (2008-12-04 16:22):
The morphology and IP's point towards to Littoral cell haemangioendothelioma.
bvrugt (2008-12-04 17:42):
Although a littoral cell angioma has crossed my mind, in my view the absence of a papillary growth pattern
with sloughing of lesional cells argues against this possibility. However, littoral cell angioma are negativ for
CD8, but positiv for fVIII, CD31, CD68 and CD21. Especially CD21 is very useful in discriminating litteral
cell angioma from other vascular lesions. Would it be worthwile to perform CD21 just to make the diagnosis
complete or is everybody convinced of a hemagioendothelioma / low-grade angiosarcoma?
kunze (2008-12-04 22:38):
The staining pattern for CD34 seems nearly identic with CD31. That's not the case in littoral cell angiomas.
bvrugt (2008-12-05 09:27):
Prof. Kunze, what do you mean with different staining patterns between CD34 and CD31?
micug (2008-12-05 11:49):
Please find previous case with littoral cell haemangioma with CLL:
https://telemed.ipath.ch/lithuania/object/view/80807
kunze (2008-12-05 15:02):
The CD68-positive luminal cells of littoral cell angiomas are usually positive for CD31. Expression of CD34
is restricted to basal endothelial cells.
micug (2008-12-14 11:10):
CD21(-)(except FDC in follicles; not appended). Please find additional photos of CD34,CD31,vWF and
CD68: sinuses highly CD68+, tumor cells +/-. Multiple macropfages are present.
kunze (2008-12-14 12:56):
There is no difference between the immunostains for CD31 and CD34, i.e. the tumor cells are positive for
both endothelial markers. Together with the staining pattern for CD68 (scattered macrophages) these are
arguments against a littoral cell angioma.
micug (2008-12-14 13:08):
Thank you a lot.
Annotations 6