title: Orbital rhabdomyosarcoma.
abstract: BACKGROUND
  Although rhabdomyosarcoma (RMS) is a rare tumor among the entire group of mesenchymal malignancies, it is a relatively common lesion and significant challenge for the ocular oncologist in terms of its diagnosis and management.
  
  
  METHODS
  A comprehensive literature search of articles published over the past 30 years in PubMed was conducted.
  
  
  RESULTS
  Orbital RMS usually presents as a space-occupying lesion in the orbit during the first decade and may mimic other neoplastic or inflammatory masses. The tumor has predilection for the superior nasal quadrant of the orbit. The clinical manifestations depend on the location of the tumor within the orbit and its rate of growth. The common histopathologic types are embryonal and alveolar varieties. CT and MR imaging are important in the evaluation of this tumor. Particular attention should be placed on the bone invasion and extension of the tumor into the intracranial cavity and paranasal sinuses. Treatment usually consists of a combination of chemotherapy and radiation therapy following excisional biopsy.
  
  
  CONCLUSIONS
  Survival of orbital RMS has improved due to advances in chemotherapy and radiotherapy. Posttreatment complications, including side effects of radiotherapy and secondary orbital malignancies, as well as visual dysfunction, occur more often and present new challenges due to improved long-term survival.
authors.affiliation: Department of Ophthalmology and Cancer Center, Tulane University Health Sciences Center, New Orleans, LA 70112, USA. zkarcio@tulane.edu
authors.forename: Zeynel A
authors.initials: ZA
authors.lastname: Karcioglu
authors.forename: Doris
authors.initials: D
authors.lastname: Hadjistilianou
authors.forename: Marta
authors.initials: M
authors.lastname: Rozans
authors.forename: Sonia
authors.initials: S
authors.lastname: DeFrancesco
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005134:Eye Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D013508:Ophthalmologic Surgical Procedures / Q000379:methods; D011379:Prognosis; D011878:Radiotherapy / Q000379:methods; D012208:Rhabdomyosarcoma / Q000175:diagnosis* / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents