title: Congenital mesenchymal chondrosarcoma of the orbit: case report and review of the literature.
abstract: OBJECTIVE
  To report the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit.
  
  
  METHODS
  Interventional case report and review of the literature.
  
  
  METHODS
  Ophthalmologic examination and computed tomography scan of the orbit were performed. Histologic and histochemical examination and immunohistochemistry of the biopsy specimen were evaluated.
  
  
  METHODS
  Ocular and systemic disease control.
  
  
  RESULTS
  A 5-month-old girl was seen with a papillomatous lesion in the right lower tarsal conjunctiva present from birth. Histologic examination demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage, and immunohistochemistry studies revealing positivity for vimentin and S-100 were consistent with the diagnosis of mesenchymal chondrosarcoma. The patient was treated with combined chemotherapy (ifosfamide, epirubicin, and cisplatin), radiotherapy, and surgery (exenteration). She is alive with no evidence of disease after 4 years of follow-up. Facial asymmetry and dental malformations have developed as late effects. Further reconstructive surgery is planned for the malformations.
  
  
  CONCLUSIONS
  Extraskeletal mesenchymal chondrosarcoma in the orbit is extremely rare. This study reports the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit (also the youngest patient) both in skeletal and extraskeletal sites. Multimodality treatment (surgery, chemotherapy, and radiotherapy) may lead to long-term survival.
authors.affiliation: Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Samuray
authors.initials: S
authors.lastname: Tuncer
authors.forename: Rejin
authors.initials: R
authors.lastname: Kebudi
authors.forename: Gonul
authors.initials: G
authors.lastname: Peksayar
authors.forename: Misten
authors.initials: M
authors.lastname: Demiryont
authors.forename: Orhan
authors.initials: O
authors.lastname: Cizmeci
authors.forename: Inci
authors.initials: I
authors.lastname: Ayan
authors.forename: Omer
authors.initials: O
authors.lastname: Gorgun
authors.forename: Emin
authors.initials: E
authors.lastname: Darendeliler
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis; D018211:Chondrosarcoma, Mesenchymal / Q000737:chemistry / Q000151:congenital* / Q000000981:diagnostic imaging / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D009918:Orbital Neoplasms / Q000737:chemistry / Q000151:congenital* / Q000000981:diagnostic imaging / Q000628:therapy; D014057:Tomography, X-Ray Computed
chemical_list: D014408:Biomarkers, Tumor