What are the other Names for this Condition? (Also known as/Synonyms)

• Ewing Sarcoma Family of Tumors, Askin’s Tumor Subtype
• Ewing's Tumor of Chest Wall
• Primitive Neuroectodermal Tumor of Thoracopulmonary Region

What is Askin’s Tumor? (Definition/Background Information)

Ewing’s Family of Tumors includes the following tumors:

• Ewing’s Sarcoma of Bone
• Extraosseous Ewing’s Tumor
• Primitive Neuroectodermal Tumor (PNET), including Medulloblastoma - Adult type and Medulloblastoma - Childhood type
• Askin’s Tumor: This tumor is also sometimes called, Primitive Neuroectodermal Tumor of Chest Wall, and Ewing’s Sarcoma of Chest Wall
• Primary Cutaneous Ewing’s Sarcoma (PCES)

All the above-mentioned tumors arise from a primitive stem cell.

• Askin's Tumor is PNET tumor that develops from soft tissues of the chest wall. It is a rare, life-threatening and malignant type of tumor that affects young children. Askin’s Tumor is very uncommon in adults
• It is believed that the tumor is caused by genetic defects on chromosome 11, chromosome 22, chromosome 8, and chromosome 12
• However, with a global incidence rate of less than 1 in 500,000, Ewing’s Family of Tumors are rare types of tumors
• The treatment modalities used to treat this tumor are surgery, chemotherapy, and radiation therapy. The prognosis is generally poor, but depends on the stage of the tumor. Metastatic tumors have a poor prognosis

Who gets Askin’s Tumor? (Age and Sex Distribution)

• Children are primarily affected by Askin's Tumor
• Adults over the age of 30 years, are highly unlikely to get this condition
• Boys are affected more commonly than girls
• Caucasians are most commonly affected, compared to any other racial group

What are the Risk Factors for Askin’s Tumor? (Predisposing Factors)

• Genetic mutations could be a possible causal factor for Askin's Tumor
• Prior radiation therapy for a completely different tumor may cause the development of Askin's Tumor (at the site of radiation therapy)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Askin’s Tumor? (Etiology)

• Askin's Tumor display recurrent chromosomal translocation (between chromosomes 11 and 22) with minor chromosomal anomalies (on chromosomes 8 and 12)
• Specific translocations between chromosomes 11 and 22 is present, in a majority of the patients
• Such genetic mutations are known to have potentially cancerous traits. However, a specific reasons for these mutations to occur, is not established yet

What are the Signs and Symptoms of Askin’s Tumor?

Signs and symptoms of Askin's Tumor may include:

• The tumor usually develops in the paravertebral region in the soft tissues of the chest wall
• Breathlessness
• Weight loss
• Persistent cough, with or without blood
• Painful chest wall mass
• Enlarged regional lymph nodes
• Horner’s syndrome
• Fever, which may be observed frequently, may indicate an infection
• Anemia (low red cell count in blood); leukocytosis (above normal levels of white blood cells)

How is Askin’s Tumor Diagnosed?

Diagnostic tools for Askin's Tumor include:

• Physical exam with complete medical history evaluation
• X-ray of the chest may show soft tissue mass that involves nearby ribs, causing their destruction. There may be pleural effusion (fluid in the lungs) on the chest x-ray
• MRI scan of lung, CT scan of chest, to determine the extent of the tumor
• Biopsy of tumor - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis
• Genetic tests and analysis, to test for specific mutations associated with Askin's Tumor

Differential diagnosis, to eliminate the following tumor types may be considered, before arriving at a definitive diagnosis:

• Dedifferentiated synovial sarcoma
• Desmoplastic small round cell tumors
• Lymphoma
• Medulloblastoma
• Mesenchymal chondrosarcoma
• Rhabdomyosarcoma
• Small cell osteosarcoma

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Askin’s Tumor?

Complications due to Askin's Tumor are:

• Metastasis of the tumor to other vital body organs, such as the lungs and bone marrow
• Damage of the bones, vital nerves, and blood vessels, during surgery
• Side effects from chemotherapy (such as toxicity), radiation therapy
• Recurrence of the tumor after treatment

How is Askin’s Tumor Treated?

Treatment measures for Askin’s Tumor include the following:

• The treatment is usually performed by a multidisciplinary team. Moreover, because the tumor is rare, well-defined treatment protocols are yet to be established
• The treatment is based on a variety of factors, such as:
  • The tumor stage
  • Location of the tumor
  • Tumor size
  • And whether the tumor has metastasized
• Any combination of chemotherapy, radiation therapy and invasive procedures are used to treat this tumor. Generally, the treatment is administered at a medical center that has prior experience in treating such tumors

How can Askin’s Tumor be Prevented?

• Current medical research have not established a way of preventing Askin's Tumor
• Genetic counseling and genetic testing could help those individuals having a family history of the condition, planning for a child
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured Askin's Tumor
• Besides, due to its high metastasizing potential, often, several years of active vigilance is necessary

What is the Prognosis of Askin’s Tumor? (Outcomes/Resolutions)

• The prognosis is better, if Askin's Tumor is:
  • Diagnosed at an early stage
  • Features a small-sized tumor that has not spread to other areas
  • The individuals’ response to the treatment procedure is good
• Over 65% of children, who have localized Askin's Tumor (low-stage tumors) survive for a longer period
• If the tumor has spread to other parts of the body, then less than one-third of the individuals, survive the condition
• Improved outcomes have been obtained with radiotherapy and chemotherapy and the survival rate is above 40%

Additional and Relevant Useful Information for Askin’s Tumor:

• Askin’s Tumor and its management can cause physical and emotional distress. Often, supportive care and encouragement help positively in bringing about a measure of relief, to the patients.
• Both, Ewing’s Sarcoma and Primitive Neuroectodermal Tumor, are round cell sarcomas (types of cancer), with ES lacking neuroectodermal differentiation, while PNET presents such features
• This tumor was described in 1979 by Askin; hence, the name Askin’s Tumor
• It is estimated that 200 new cases of Ewing’s Family of Tumors are diagnosed newly in the US per year