Topic Overview > Sarcoma Awareness Month

Sarcoma Awareness Month


Sarcomas arise from cells of the mesenchymal supporting tissue and are therefore not limited to one organ or part of the body. The most common subtypes include liposarcomas and leiomyosarcomas. (www.krebsdaten.de). Several risk factors can contribute to the development of sarcomas, including genetic predisposition, radiation, certain chemicals and viral infections such as human herpesvirus 8 (HHV-8) and Epstein-Barr virus (EBV). It is important to have regular screenings and minimize risk factors to reduce the risk of sarcoma.

The differentiation of well-differentiated liposarcomas (WDLPS) and dedifferentiated liposarcomas (DDLPS) from benign adipose tumors or other poorly differentiated sarcomas is often morphologically difficult.

Binh et al. [1, 2] describe the immunohistochemical detection of CDK4 and MDM2 overexpression as very helpful and reproducible in the diagnosis of ALT/WDLPS and dedifferentiated liposarcomas. According to Weaver et al. [3], MDM2 overexpression is also a sensitive marker for differentiating liposarcomas from sclerosing mesenteritis and retroperitoneal fibrosis.

MDM2 amplification in different tissues

Tissue Frequency of MDM2 Amplification
Atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDLS) ~ 100 %
Dedifferentiated liposarcoma (DDLS) ~ 100 %
Pleomorphic liposarcoma ~ 40 %
Benign lipomatous lesions 0 %

Ki-67 clone K-2: marker for lipoblasts

Miller [4] describes the use of the Ki-67 antibody, clone K-2, as a marker for lipoblasts and liposarcomas. This antibody shows not only the usual Ki-67 reaction in the nuclei of proliferating cells but also a cytoplasmic reaction in lipoblasts. No reaction was observed in "pseudolipoblasts" in cases of inflammatory myxohyaline tumors and fat necrosis.

Zytomed Systems offers you this Ki-67 clone to complement your antibody panel for liposarcoma.

According to the Soft Tissue Sarcoma Guideline | Version 1.1 | June 2022, DKG, the following antibodies, among others, have proven their value for the initial diagnosis of soft tissue tumors:

Broad spectrum keratins Desmin MelanA ERG
EMA Caldesmon or calponin HMB45 CD117
Ki67 Myogenin ER/PR DOG1
S100 WT1 CD10 NSE
CD45/PanLeu Calretinin Smooth muscle actin CD56
CD21 BAP1 Synaptophysin SOX10
CD23 Podoplanin/D2-40 Chromogranin CD34
CD99

For extended immunohistochemical diagnostics, there are a number of relatively specific markers that can also be used partly for screening underlying genetic alterations:

beta-Catenin e.g. desmoid fibromatosis
MDM2/CDK4 highly differentiated/de-differentiated liposarcoma
BRAF V600E malignant melanoma
TLE1 Synovial sarcoma
STAT6 solitary fibrous tumor SFT
MUC4 low grade fibromyxoid sarcoma sclerosing epithelioid fibrosarcoma
TFE3 alveolar soft tissue sarcoma
SDHB SDH-mutated gastrointestinal stromal tumors GIST
HHV8 Kaposi's sarcoma
c-MYC Radiation-induced sarcomas, especially angiosarcomas
CAMTA1 epithelioid hemangioendotheliomas
FOSB pseudomyogenic hemangioendothelioma epithelioid hemangioma
INI-1 malignant rhabdoid tumor epithelioid sarcoma, epithelioid malignant nerve sheath tumor
H3K27me malignant peripheral nerve sheath tumor
Brachyury Chordome
ALK Chordome
NKX2.2 Ewing sarcoma
WT1/ETV4 CIC-rearranged small cell sarcomas
BCOR BCOR-rearranged small cell sarcomas

On our website you will find a large selection of CE/IVD compliant antibodies for your sarcoma diagnostics.

[1] Binh MB et al. Am J Surg Pathol, 29:1340-1347, 2005

[2] Binh MB et al. Am J Clin Pathol, 125:693-697, 2006

[3] Weaver J et al. Mod Pathol 22:66-70, 2009

[4] Miller RT. ProPath: The Focus Immunohisto-chemistry (Newsletter), February 2005