Primary embryonal carcinoma of the orbit in a 10-month-old female: a seven-year follow-up.
OBJECTIVE
Extra-gonadal germ cell tumors (GCTs) are rare and can be highly aggressive. If correctly identified and treated with multimodality chemotherapy, their prognosis can be significantly improved. We examined a 10 month-old female with primary embryonal carcinoma of the orbit.


METHODS
Case report and literature review.


METHODS
Case study with 7-year follow-up and literature review of intracranial and intraorbital GCT cases.


RESULTS
The patient presented with progressive proptosis and ophthalmoplegia. CT scan revealed an orbital apex mass and biopsy demonstrated a nongerminomatous GCT--an embryonal carcinoma. The patient is tumor-free 7 years after multimodality chemotherapy. She has mild amblyopia and a right micro esotropia.


CONCLUSIONS
Nongonadal GCTs of the orbit can occur and should be considered in the differential diagnosis of a young child with proptosis and ophthalmoplegia. Five-year survival rates improve significantly with accurate identification and treatment.
Rodriguez
Sylvia L
SL
Queen's University, Kingston, Ontario, Canada.
Kostick
David A
DA
Hered
Robert W
RW
Joyce
Michael J
MJ
D014408:Biomarkers, Tumor / Q000032:analysis; D018236:Carcinoma, Embryonal / Q000737:chemistry / Q000000981:diagnostic imaging / Q000473:pathology*; D005094:Exophthalmos / Q000175:diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D009886:Ophthalmoplegia / Q000175:diagnosis; D009918:Orbital Neoplasms / Q000737:chemistry / Q000000981:diagnostic imaging / Q000473:pathology*; D014057:Tomography, X-Ray Computed
D014408:Biomarkers, Tumor