Gene affected in Ewings sarcoma is ?
**Core Concept:** Ewing's sarcoma is a rare type of cancer that primarily affects bone tissue. It is characterized by the presence of a specific chromosomal translocation, t(11;22)(q24;q12), which leads to the fusion of the EWSR1 and FLI1 genes. This translocation results in the production of an oncoprotein, promoting uncontrolled cell growth and leading to the development of Ewing's sarcoma.
**Why the Correct Answer is Right:** The correct answer, option D (EWSR1), is the gene involved in the chromosomal translocation that leads to the development of Ewing's sarcoma. The fusion of EWSR1 and FLI1 genes results in the production of an oncoprotein, which drives cell growth and proliferation, causing the formation of Ewing's sarcoma.
**Why Each Wrong Option is Incorrect:**
A. FLI1 (Option A) is also involved in the fusion during Ewing's sarcoma formation but the correct answer, EWSR1, is also part of the fusion and should be considered together.
B. EWSR1 is the correct answer, not Option B (ETS family of transcription factors), which is not the primary driver in Ewing's sarcoma development.
C. Ewing's sarcoma is primarily caused by the fusion of EWSR1 and FLI1 genes, making Option C incorrect.
**Clinical Pearl:** Understanding the genetic basis of diseases like Ewing's sarcoma helps in understanding the pathogenesis and targeted therapy options for these malignancies. Knowledge of oncogenes and tumor suppressor genes is essential for medical students and practicing physicians alike, as it contributes to their understanding of cancer development and treatment strategies.