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

Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift für Augenheilkunde
Koung Hoon KookHans E Grossniklaus

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.

References

Feb 1, 1992·Histopathology·N WalfordP A Voute
Apr 1, 1992·Ophthalmology·J H NiffeneggerD M Albert
Oct 26, 1999·Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus·D B StidhamD A Plager
Jan 29, 2005·Ophthalmic Plastic and Reconstructive Surgery·Chloe GottliebJohn T Harvey

Citations

Jun 16, 2014·Survey of Ophthalmology·Benjamin P Erickson, David T Tse
Mar 13, 2010·Journal of Pediatric Surgery·Hart Isaacs
May 3, 2014·Orbit·Kaustubh Mulay, Santosh G Honavar
Jan 18, 2015·Survey of Ophthalmology·F A JakobiecSuzanne K Freitag
Feb 27, 2017·Canadian Journal of Ophthalmology. Journal Canadien D'ophtalmologie·Maya HadaSeema Kashyap
Jul 15, 2015·Clinical Pediatrics·Michael StoltenRaj Warrier
Apr 24, 2018·Journal of Pediatric Ophthalmology and Strabismus·Eva Ayala BarrosoRamón Medel Jiménez
Mar 12, 2019·Ophthalmic Plastic and Reconstructive Surgery·Archana SrinivasanJurij R Bilyk
Dec 1, 2020·Pediatric Radiology·Tamara Feygin, Larissa T Bilaniuk
Jan 5, 2019·Journal of Pediatric Hematology/oncology·Ahitagni BiswasMehar C Sharma

Related Concepts

Biopsy
Differential Diagnosis
Malignant Neoplasm of Liver
Magnetization Transfer Contrast Imaging
Neoplasm Metastasis
Orbital Neoplasms
Orbit Evisceration
Fatal Outcome
Rhabdoid Tumor
Autopsy

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