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. 2013 Jul;27(3):167-75.
doi: 10.1016/j.sjopt.2013.06.004.

Orbital rhabdomyosarcomas: A review

Lama Jurdy  1 , Johanus H M Merks, Bradly R Pieters, Maarten P Mourits, Roel J H M Kloos, Simone D Strackee, Peerooz Saeed
Affiliations

Orbital rhabdomyosarcomas: A review

Lama Jurdy et al. Saudi J Ophthalmol. 2013 Jul.

Abstract

Rhabdomyosarcoma (RMS) is a highly malignant tumor and is one of the few life-threatening diseases that present first to the ophthalmologist. It is the most common soft-tissue sarcoma of the head and neck in childhood with 10% of all cases occurring in the orbit. RMS has been reported from birth to the seventh decade, with the majority of cases presenting in early childhood. Survival has changed drastically over the years, from 30% in the 1960's to 90% presently, with the advent of new diagnostic and therapeutic modalities. The purpose of this review is to provide a general overview of primary orbital RMS derived from a literature search of material published over the last 10 years, as well as to present two representative cases of patients that have been managed at our institute.

Keywords: AMORE; Brachytherapy; Eye; Genetics; Orbit; Pediatrics; Rhabdomyosarcoma; Tumor.

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Figures

Figure 1
Figure 1
Embryonal orbital RMS. Elongated to round spindle cells with features of skeletal muscle in different stages of embryogenesis with a highly eosiniphilic cytoplasm and hyperchromatic nuclei.
Figure 2
Figure 2
Alveolar orbital RMS. The cells are large, with abundant eosinophilic cytoplasm and round to polygonal in shape with vesicular nuclei which are a diagnostic feature of alveolar type as opposed to embryonal.
Figure 3
Figure 3
MR orbits and brain. Large intraorbital mass with local invasion of the lamina papyracea and no intracranial extension.
Figure 4
Figure 4
A silicon mold with three parallel plastic tubes positioned in the medial part of the orbit.
Figure 5
Figure 5
Galea flap attached to a calvarian bone.
Figure 6
Figure 6
Galea flap attached to a calvarian bone, brought together to the orbit to reconstruct the medial wall and orbital floor.
None
See this image and copyright information in PMC

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