What is the treatment for Askin tumor?

Treatment of Askin Tumor

The optimal treatment for Askin tumor requires an aggressive interdisciplinary approach combining neoadjuvant chemotherapy, radical surgical resection, and radiotherapy to achieve the best survival outcomes. 1

Overview of Askin Tumor

• Askin tumor is a rare but highly malignant peripheral primitive neuroectodermal tumor (PNET) that occurs in the thoracopulmonary region, primarily affecting children and young adults 2
• Common presenting symptoms include chest wall mass, pain, bony involvement, fever, dyspnea, and occasionally weight loss, cough, or hemoptysis 3
• The tumor is often misdiagnosed as it can be mistaken for other small round-cell tumors 2

Treatment Algorithm

First-Line Treatment Approach

• Neoadjuvant (pre-surgical) multiagent chemotherapy to reduce tumor volume 4
• Followed by radical surgical resection of the tumor with chest wall reconstruction when necessary 4, 1
• Post-operative radiotherapy, particularly for cases with incomplete resection or high-risk features 1

Surgical Management

• Complete surgical excision should be performed after tumor volume reduction with neoadjuvant chemotherapy 3
• Large chest wall defects after surgery can be successfully reconstructed using latissimus dorsi muscle flaps and biomaterials 4
• Surgery alone is insufficient and must be combined with other treatment modalities 1

Chemotherapy Protocols

• Aggressive multiagent chemotherapy regimens show better outcomes than less intensive approaches 3
• Studies indicate that more aggressive chemotherapy protocols achieve higher complete remission rates compared to simpler regimens (e.g., vincristine and cyclophosphamide alone) 3

Radiotherapy

• Local radiotherapy is an essential component of the treatment plan, especially for cases with residual disease after surgery 3, 1
• Optimal radiotherapy is an important prognostic factor influencing outcome 1

Prognostic Factors

• Age ≥18 years is associated with inferior survival outcomes 1
• Poor response to induction chemotherapy indicates worse prognosis 1
• Presence of pleural effusion is a negative prognostic indicator 1
• Primary tumor size affects overall outcome 1
• Metastatic disease at presentation carries a very poor prognosis, with 56% of patients dying within 1 month of diagnosis 1

Treatment Outcomes

• Combined therapy (surgery + chemotherapy + radiotherapy) yields superior results compared to mono-therapy approaches 2
• Studies report 5-year survival rates of up to 86% and overall survival rates of 71% with aggressive interdisciplinary management 4
• Median survival time for patients receiving combined therapy is significantly longer than for those receiving mono-therapy (15 months vs. 7 months in one study) 2
• Median time to relapse is approximately 25 months 1

Follow-up and Monitoring

• Close follow-up is essential due to the aggressive nature of the disease 5
• Regular imaging studies should be performed to detect local recurrence or metastatic disease 2
• Recurrence most commonly occurs at the local site 3

Important Considerations and Pitfalls

• Delayed diagnosis is common due to the rarity of the condition 5
• Accurate preoperative diagnosis can be challenging and requires microscopy and immunohistochemical staining 5
• Treatment should ideally be performed in specialized oncological centers as part of clinical studies 5
• Patients with metastatic disease have extremely poor outcomes and require particularly aggressive management 1