Markers and Immunoprofile of Vascular and Perivascular Tumors

, Hans Guski2 and Glen Kristiansen3



(1)
Carl-Thiem-Klinikum, Institut für Pathologie, Cottbus, Germany

(2)
Vivantes Klinikum Neukölln, Institut für Pathologie, Berlin, Germany

(3)
Universität Bonn, UKB, Institut für Pathologie, Bonn, Germany

 




Diagnostic Antibody Panel for Vascular Tumors

CD31, CD34, factor VIII, CD105, ERG, podoplanin, thrombomodulin (CD141), and Fli-1 [1].
























CD31 (PECAM-1)

Expression pattern: membranous/cytoplasmic

Main diagnostic use

Expression in other tumors

Expression in normal cells

Vascular tumors

Plasmacytoma, Langerhans cell histiocytosis and Langerhans sarcoma, granulocytic sarcoma, Ewing’s sarcoma, rare carcinoma types

Endothelial cells, megakaryocytes/platelets, macrophages/monocytes, Kupffer cells, osteoclasts, myoblasts, granulocytes, mantle zone B cells, T/NK cells and plasma cells

Positive control: appendix


Diagnostic Approach

CD31, also known as PECAM-1 (platelet endothelial cell adhesion molecule-1), is a transmembrane glycoprotein and member of the immunoglobulin family normally expressed on endothelial cell junctions and on the surface of platelets, monocytes, granulocytes, and B-lymphocytes. CD31 is a sensitive and specific marker for blood vessels and vascular tumors [1, 2].


Diagnostic Pitfalls

Low expression levels of CD31 are reported in rare nonvascular tumors such as chronic lymphocytic lymphoma, plasmacytoma, Langerhans cell neoplasia, leiomyosarcoma, mesothelioma, and glioma in addition to few carcinoma types such as carcinoma in situ and invasive breast carcinoma and papillary thyroid carcinoma.
























CD34

Expression pattern: membranous

Main diagnostic use

Expression in other tumors

Expression in normal cells

Vascular tumors, Kaposi’s sarcoma, GIST, dermatofibrosarcoma protuberans, solitary fibrous tumor, epithelioid sarcoma, AML (M0), granulocytic sarcoma, neurofibroma, liposarcoma

Pre-B-ALL, AML (M7), alveolar soft part sarcoma, congenital and infantile fibrosarcoma, inflammatory fibrous polyp of gastrointestinal tract, breast fibroadenoma, giant cell fibroblastoma, juxtaglomerular cell tumor, superficial acral fibromyxoma

Hematopoietic progenitor cells (myeloid, B- and T-lymphocyte precursors), endothelial cells, hepatic sinusoidal cells, interstitial cells of Cajal, endometrial stroma, fibroblasts

Positive control: appendix


Diagnostic Approach

CD34 is a cell surface adhesion glycoprotein expressed on the surface of precursor hematopoietic cells of myeloid and lymphoid lineage, a subset of mesenchymal stem cells and endothelial cells, and a large number of tumors originated from these cells. CD34 is a widely used marker to highlight blood vessels and vascular tumors, but it is less specific than CD31 (Fig. 24.1) [1, 2]. CD34 is also an important marker for other tumors such as dermatofibrosarcoma protuberans and GIST. Furthermore, CD34 is one of the essential markers for hematopoietic and mesenchymal stem cells that also label myeloid blast in AML.

A430796_1_En_24_Fig1_HTML.jpg


Fig. 24.1
CD34 labeling neoplastic endothelium in angiosarcoma


Diagnostic Pitfalls

Because of its broad expression spectrum, CD34 must be used as a screening marker supported by a panel of more specific antibodies [3].
























Factor VIII (von Willebrand factor)

Expression pattern: cytoplasmic

Main diagnostic use

Expression in other tumors

Expression in normal cells

Vascular tumors
 
Endothelial cells and endocardium, platelets and megakaryocytes, mast cells

Positive control: appendix


Diagnostic Approach

Factor VIII (von Willebrand factor) is a glycoprotein complex composed of three subunits with functional binding domains to platelet glycoproteins, collagen, and heparin. Factor VIII is synthesized by endothelial cells and megakaryocytes and stored in the Weibel-Palade bodies of endothelial cells. Factor VIII is a specific marker for blood vessels and vascular tumors. The intensity of factor VIII expression correlates with the differentiation grade of the vascular tumors and is very low in poorly differentiated vascular tumors such as angiosarcoma (Fig. 24.2).

A430796_1_En_24_Fig2_HTML.jpg


Fig. 24.2
Angiosarcoma with diffuse expression of factor VIII

















Podoplanin (D2-40)

Expression pattern: membranous/cytoplasmic

Main diagnostic use

Expression in other tumors

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Dec 25, 2017 | Posted by in ONCOLOGY | Comments Off on Markers and Immunoprofile of Vascular and Perivascular Tumors
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