BACKGROUND: @entity4236 ( @entity4236 ) is a rare @entity5 in @entity1 . Radical surgery followed by postoperative radiation is considered the standard of care in adults. A similar approach in @entity1 can lead to significant long-term morbidity. PROCEDURE: A retrospective multi-institutional review of @entity1 less than 21 years of age diagnosed with @entity4236 between 1990 and 2014 was performed. Clinical features, treatment, and outcome were obtained from the medical records. RESULTS: Twenty-four @entity1 were identified with a median age of 14 years (range 0.6-20 years) at diagnosis. The majority (75%) were females. @entity10 was the most common presenting symptom, followed by nasal obstruction and @entity1415 . Eight @entity1 had Kadish stage B @entity5 and 16 had Kadish stage C @entity5 . Nine @entity1 had @entity3 . Gross total resection was achieved at diagnosis in eight @entity1 and after neoadjuvant chemotherapy in four @entity1 . Twenty-one @entity1 received radiation therapy (45-68.4 Gy). Thirteen @entity1 received neoadjuvant chemotherapy with 84% objective response rate. Seven @entity1 experienced disease progression or relapse-five in central nervous system, one local, and one in cervical lymph node. Fifteen @entity1 were alive at the last follow-up. The 5-year disease-free survival and overall survival were 74% and 73%, respectively. Late effects were observed in 78% of long-term survivors. Four @entity1 developed subsequent @entity5 . CONCLUSIONS: Pediatric @entity4236 is a chemosensitive disease. Preoperative chemotherapy-based multimodal approach should be used in @entity1 with advanced stage disease. Radiation therapy is effective for local control, but lower doses should be considered in @entity1 .

Multimodality Treatment of XXXX .

@entity1 :: ('9606', 'Species') :: ['children', 'patients'] @entity5 :: ('MESH:D009369', 'Disease') :: ['malignant neoplasms', 'tumors', 'cancer of the nasal cavity'] @entity1415 :: ('MESH:D004844', 'Disease') :: ['epistaxis'] @entity4236 :: ('MESH:D018304', 'Disease') :: ['ENB', 'Esthesioneuroblastoma'] @entity3 :: ('MESH:D009362', 'Disease') :: ['metastatic disease'] @entity10 :: ('MESH:D006261', 'Disease') :: ['Headache']
@entity4236 :: (MESH:D018304,Disease) :: ['Pediatric Esthesioneuroblastoma']
