title: Outcomes after interdisciplinary management of 7 patients with Askin tumor.
abstract: OBJECTIVE
  Askin tumors are rare but highly malignant chest wall tumors, which require multimodal therapy including often extensive resection of the thoracic wall. This study evaluated the outcome of Askin tumor in seven patients with an interdisciplinary approach.
  
  
  METHODS
  Patients' records, treated between 1994 and 2011, were reviewed retrospectively. Seven patients (three male, four female; mean age 12.3 years; range 2-21 years) were included. All patients received neoadjuvant chemotherapy. After reduction of initial tumor volume, radical tumor resection and thoracic wall reconstruction were performed. All survivors were evaluated in 2011 by clinical examination and lung function test.
  
  
  RESULTS
  Five-year survival rate in our group of patients is 86 % and overall survival is 71 %. There were two mortalities. One patient passed away 7.5 years after the primary management, mainly attributed to tumor progression, which demanded aggressive surgical procedures and irradiation. Another patient died 18 months after the first diagnosis after several surgical interventions for recurrent multiple pulmonary metastases. Three years after the first diagnosis, one patient suffered from clear cell sarcoma of the contralateral kidney and developed a local recurrence of Askin 1 year later. The large chest wall defects arising after surgery have been successfully reconstructed using combination of latissimus dorsi muscle flaps and biomaterials.
  
  
  CONCLUSIONS
  Data of pediatric patients with Askin tumor is scarce. Analysis of our seven patient series indicates that improved outcomes (71 % over all survival rate and 86 % 5-year survival rate) can be achieved by aggressive interdisciplinary management including radical surgery and chemotherapy. Chest wall stability can be achieved by utilization of local muscle flaps and biomaterials to cover surgical chest wall defects.
authors: Department of Pediatric and Adolescent Surgery, Medical University of Graz, Auenbruggerplatz 34, 8010 Graz, Austria. alireza.basharkhah@pro.medunigraz.at
authors: Alireza
authors: A
authors: Basharkhah
authors: Jasmin
authors: J
authors: Pansy
authors: Christian
authors: C
authors: Urban
authors: Michael E
authors: ME
authors: Höllwarth
mesh_terms: D000293:Adolescent; D001859:Bone Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D020360:Neoadjuvant Therapy; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D019637:Orthopedic Procedures / Q000379:methods; D019651:Plastic Surgery Procedures; D012272:Ribs / Q000601:surgery; D012512:Sarcoma, Ewing / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D035441:Thoracic Wall / Q000601:surgery; D016896:Treatment Outcome; D055815:Young Adult
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