Congenital mesenchymal chondrosarcoma of the orbit: case report and review of the literature.
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.
Tuncer
Samuray
S
Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Kebudi
Rejin
R
Peksayar
Gonul
G
Demiryont
Misten
M
Cizmeci
Orhan
O
Ayan
Inci
I
Gorgun
Omer
O
Darendeliler
Emin
E
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
D014408:Biomarkers, Tumor