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A case of congenital orbital malignant rhabdoid tumor: systemic metastasis following exenteration.

Authors
Kook, KH; Park, MS; Yim, H; Lee, SY; Jang, JW; Grossniklaus, HE
Citation
Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift fur Augenheilkunde, 223(4):274-278, 2009
Journal Title
Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift fur Augenheilkunde
ISSN
0030-37551423-0267
Abstract
A newborn girl presented with massive proptosis of the right eye. Physical and radiologic examination disclosed that the primary orbital mass was confined to the site. A diagnosis of malignant rhabdoid tumor was made by histopathologic examination of an incisional biopsy specimen. Exenteration was performed, and the resection margins were free from tumor cells. However, distant metastasis developed in the liver 1 month after surgery. Despite chemotherapy, the patient died 2 months later due to tumor invasion into the central nervous system, which was confirmed by autopsy. To the best of our knowledge, this is the first case of congenital orbital malignant rhabdoid tumor showing systemic metastasis after exenteration, which suggests the need for aggressive systemic treatment rather than exenteration, even in a case of locally confined tumor.
MeSH terms
BiopsyDiagnosis, DifferentialFatal OutcomeFemaleHumansInfant, NewbornLiver Neoplasms/secondary*Magnetic Resonance ImagingNeoplasm MetastasisOrbit Evisceration/methods*Orbital Neoplasms/congenitalOrbital Neoplasms/pathology*Orbital Neoplasms/surgeryRhabdoid Tumor/congenitalRhabdoid Tumor/secondary*Rhabdoid Tumor/surgery
DOI
10.1159/000213643
PMID
19390226
Appears in Collections:
Journal Papers > School of Medicine / Graduate School of Medicine > Ophthalmology
Journal Papers > School of Medicine / Graduate School of Medicine > Pediatrics & Adolescent Medicine
Journal Papers > School of Medicine / Graduate School of Medicine > Pathology
AJOU Authors
국, 경훈박, 문성임, 현이
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