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Pathophysiology WPS Office

Ewing's sarcoma is a bone cancer primarily affecting children and teenagers, originating from primitive neuroectodermal cells. The disease is characterized by a gene fusion involving the EWSR1 gene, which promotes uncontrolled cell growth and survival, leading to aggressive tumor formation. Understanding its pathophysiology is crucial for effective diagnosis and treatment.

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Pathophysiology WPS Office

Ewing's sarcoma is a bone cancer primarily affecting children and teenagers, originating from primitive neuroectodermal cells. The disease is characterized by a gene fusion involving the EWSR1 gene, which promotes uncontrolled cell growth and survival, leading to aggressive tumor formation. Understanding its pathophysiology is crucial for effective diagnosis and treatment.

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Ritchell Cena
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© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Pathophysiology

Ewing's sarcoma originates from uncontrolled proliferation of primitive neuroectodermal cells. The
hallmark of Ewing's sarcoma is the presence of the EWSR1 gene fusion, which typically involves
chromosomes 22 and 11, resulting in the fusion of the EWSR1 gene with an ETS transcription factor gene
(such as FLI1). This genetic alteration leads to tumorigenesis by promoting cell growth and survival and
interfering with normal cell cycle regulation.

Ewing's sarcoma is a type of bone cancer that primarily affects children and teenagers. Understanding its
pathophysiology involves looking at how it develops in the body.

### Key Points:

1. **Origin of the Tumor**:

- Ewing's sarcoma begins in cells called "primitive neuroectodermal cells," which are early cells that
can develop into different types of tissues, including nerve tissue.

2. **Genetic Changes**:

- A significant feature of Ewing's sarcoma is a genetic alteration called a "gene fusion." This usually
involves a fusion of the **EWSR1** gene with other genes, such as **FLI1**. This fusion leads to the
creation of an abnormal protein that promotes cell growth and survival, ignoring the normal signals that
would tell a cell to stop growing.

3. **Tumor Growth**:

- Because of the genetic changes, the cells can multiply uncontrollably, forming a tumor. This tumor
often grows in the bones but can also develop in soft tissues around the bones.

4. **Aggressive Nature**:

- Ewing's sarcoma tends to be aggressive. It can invade nearby tissues and can spread (metastasize) to
other parts of the body, such as the lungs or bone marrow.
5. **Inflammatory Response**:

- The presence of the tumor can lead to an inflammatory response in the body, which may contribute
to symptoms like pain, swelling, and fever.

### Summary:

In simple terms, Ewing's sarcoma starts from special cells that change due to genetic mutations. These
changes cause the cells to grow out of control, forming tumors that can be aggressive, leading to
significant health challenges for affected children. Understanding this process helps in diagnosing and
treating the condition.

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