title: Rhabdomyosarcoma of the head and neck in children: review and update.
abstract: OBJECTIVE
  To review the clinical presentation, histology, staging, treatment modalities, and survival for pediatric head and neck rhabdomyosarcoma (non-orbital).
  
  
  METHODS
  Retrospective chart review at a tertiary pediatric hospital of children treated over 18 years (1996-2014) for primary head and neck non-orbital rhabdomyosarcoma.
  
  
  METHODS
  Medical charts were examined for clinical presentation, staging, histology, genetic abnormalities, treatment modalities, recurrence and complications from treatment.
  
  
  RESULTS
  Our cohort was 17 children (7 male, 10 female) with rhabdomyosarcoma with a median age of 6.3 years (range <1-19). The majority of tumors were of parameningeal location (13/17) with embryonal histology (11/17). Twenty-nine percent (5/17) demonstrated advanced metastatic disease at initial referral. Fifty-three percent (9/17) had skull base erosion and/or cranial nerve deficits. PET CT scan was performed in 4 patients. The overall survival was 75% for the duration of the study. Primary surgical excision was performed in all 4 patients with nonparameningeal tumors as compared to only 1 patient with a parameningeal tumor. All received chemotherapy and radiotherapy, as none had completely resectable disease.
  
  
  CONCLUSIONS
  Pediatric non-orbital primary rhabdomyosarcoma of the head and neck usually has a rapid onset and presents with advanced disease. Our analysis found that the majority of patients in our series had a cranial neuropathy at presentation, which highlights how advanced the disease is in these patients at presentation. The first mode of surgical intervention should be directed toward biopsy in junction with a metastatic work-up. Primary excision with negative microscopic margins for nonparameningeal rhabdomyosarcoma is ideal to spare radiotherapy but was not achievable in our cohort. The benefits of second-look biopsy after chemotherapy and radiation are still unproven; however, we believe that it was beneficial in two patients in our review for further resection thus decreasing subsequent radiation. Fluorodeoxy-d-glucose positron emission tomography (PET) to evaluate disease post treatment may further define the role for second look surgery.
authors.affiliation: Children's National Medical Center, Division of Otolaryngology, George Washington University Medical Center, 111 Michigan Avenue, NW Washington, DC 20010, United States. Electronic address: Breilly@childrensnational.org.
authors.forename: Brian K
authors.initials: BK
authors.lastname: Reilly
authors.affiliation: Center for Cancer and Blood Disorder Children's National Medical Center, 111 Michigan Ave, NW Washington, DC 20010, United States. Electronic address: AeKim@childrensnational.org.
authors.forename: AeRang
authors.initials: A
authors.lastname: Kim
authors.affiliation: Children's National Medical Center, Sheikh Zayed Campus for Advanced Children's Medicine, 111 Michigan Avenue, NW Washington, DC 20010, United States. Electronic address: Mpena@childrensnational.org.
authors.forename: Maria T
authors.initials: MT
authors.lastname: Peña
authors.affiliation: George Washington University Medical Center, 2300 Eye St, NW Washington, DC 20037, United States. Electronic address: Tdong@gwmail.gwu.edu.
authors.forename: Tiffany A
authors.initials: TA
authors.lastname: Dong
authors.affiliation: Department of Pathology Children's National Medical Center, 111 Michigan Avenue, NW Washington, DC 20010, United States. Electronic address: Ctrossi@childrensnational.org.
authors.forename: Christopher
authors.initials: C
authors.lastname: Rossi
authors.affiliation: Children's National Medical Center, Department of Diagnostic Imaging and Radiology, NW Washington, DC, United States. Electronic address: Jmurnick@childrensnational.org.
authors.forename: Jonathan G
authors.initials: JG
authors.lastname: Murnick
authors.affiliation: Pediatric Otolaryngology, Children's Hospital of Pittsburgh, Pittsburgh, PA, United States; Otolaryngology, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States. Electronic address: Sukgi.choi@chp.edu.
authors.forename: Sukgi S
authors.initials: SS
authors.lastname: Choi
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D059186:Chemoradiotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003389:Cranial Nerve Diseases / Q000209:etiology; D005260:Female; D006258:Head and Neck Neoplasms / Q000150:complications / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000628:therapy; D049268:Positron-Emission Tomography; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000150:complications / Q000556:secondary / Q000628:therapy; D015996:Survival Rate; D014057:Tomography, X-Ray Computed; D055815:Young Adult
keywords: Head and neck cancer; Non-parameningeal; Parameningeal; Pediatric neck mass; Rhabdomyosarcoma; Second-look