Askin tumor

Askin Tumor is a rare type of cancer that primarily affects children and young adults. It is a part of the Ewing's sarcoma family of tumors (ESFT), which also includes Ewing's sarcoma of bone and peripheral primitive neuroectodermal tumor (PNET). Askin Tumor specifically involves the chest wall and is also known as a chest wall tumor of childhood.

History

The tumor was first described by Robert Askin, a pathologist, in 1979. He identified the tumor as a distinct entity within the Ewing's sarcoma family of tumors. Since then, the tumor has been named in his honor.

Characteristics

Askin Tumors are small, round, blue cell tumors. They are highly aggressive and have a high rate of metastasis, often spreading to the lungs, bones, and bone marrow. The tumors are typically located in the chest wall, including the ribs, soft tissues, and nerves.

Diagnosis

Diagnosis of Askin Tumor involves a combination of imaging studies, such as computed tomography (CT) scans and magnetic resonance imaging (MRI), and tissue biopsy. The tumor cells are identified by their characteristic appearance and by the presence of specific genetic markers, including the EWS-FLI1 fusion gene.

Treatment

Treatment for Askin Tumor typically involves a combination of surgery, chemotherapy, and radiation therapy. The goal of treatment is to remove or destroy the tumor and to prevent or manage metastasis. Despite aggressive treatment, the prognosis for Askin Tumor is often poor, with a five-year survival rate of less than 50%.

Research

Research into Askin Tumor is ongoing, with efforts focused on improving diagnosis, treatment, and understanding of the tumor's genetic basis. Clinical trials are also being conducted to test new treatments and treatment combinations.