title: Multimodality Treatment of Pediatric Esthesioneuroblastoma.
abstract: BACKGROUND
  Esthesioneuroblastoma (ENB) is a rare cancer of the nasal cavity in children. Radical surgery followed by postoperative radiation is considered the standard of care in adults. A similar approach in children can lead to significant long-term morbidity.
  
  
  METHODS
  A retrospective multi-institutional review of patients less than 21 years of age diagnosed with ENB between 1990 and 2014 was performed. Clinical features, treatment, and outcome were obtained from the medical records.
  
  
  RESULTS
  Twenty-four patients were identified with a median age of 14 years (range 0.6-20 years) at diagnosis. The majority (75%) were females. Headache was the most common presenting symptom, followed by nasal obstruction and epistaxis. Eight patients had Kadish stage B tumors and 16 had Kadish stage C tumors. Nine patients had metastatic disease. Gross total resection was achieved at diagnosis in eight patients and after neoadjuvant chemotherapy in four patients. Twenty-one patients received radiation therapy (45-68.4 Gy). Thirteen patients received neoadjuvant chemotherapy with 84% objective response rate. Seven patients experienced disease progression or relapse-five in central nervous system, one local, and one in cervical lymph node. Fifteen patients 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 patients developed subsequent malignant neoplasms.
  
  
  CONCLUSIONS
  Pediatric ENB is a chemosensitive disease. Preoperative chemotherapy-based multimodal approach should be used in patients with advanced stage disease. Radiation therapy is effective for local control, but lower doses should be considered in children.
authors.affiliation: Baylor College of Medicine, Texas Children's Cancer Center, Texas Children's Hospital, Houston, Texas.
authors.forename: Rajkumar
authors.initials: R
authors.lastname: Venkatramani
authors.affiliation: Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Hubert
authors.initials: H
authors.lastname: Pan
authors.affiliation: Saint Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Wayne L
authors.initials: WL
authors.lastname: Furman
authors.affiliation: Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts.
authors.forename: Jonathan M
authors.initials: JM
authors.lastname: Marron
authors.affiliation: Children's Hospital Los Angeles, Los Angeles, California.
authors.forename: Josephine
authors.initials: J
authors.lastname: Haduong
authors.affiliation: Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts.
authors.forename: Paola
authors.initials: P
authors.lastname: Friedrich-Medina
authors.affiliation: Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Anita
authors.initials: A
authors.lastname: Mahajan
authors.affiliation: Baylor College of Medicine, Texas Children's Cancer Center, Texas Children's Hospital, Houston, Texas.
authors.forename: Abhishek
authors.initials: A
authors.lastname: Bavle
authors.affiliation: Department of Pathology, Baylor College of Medicine, Houston, Texas.
authors.forename: Hao
authors.initials: H
authors.lastname: Wu
authors.affiliation: Baylor College of Medicine, Texas Children's Cancer Center, Texas Children's Hospital, Houston, Texas.
authors.forename: Murali
authors.initials: M
authors.lastname: Chintagumpala
mesh_terms: D000293:Adolescent; D059248:Chemoradiotherapy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018304:Esthesioneuroblastoma, Olfactory / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009296:Nasal Cavity; D009669:Nose Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: chemotherapy; childhood; esthesioneuroblastoma; radiation; surgery