A 12 years old Korean girl was seen on 10 Oct. 1969 with chief complaint of a large fleshy mass extruded out of the orbit associated with ocular pain of mild degree for several months.
Her parents were first noticed that the girl´s right eye had a peculiar reflex from the pupillary zone in 2 years of her age. She was advised by a local clinician to correct surgically someday later in her childhood. At the age of four, the pupillary margin of the affected eye had become distorted and they became aware of the eyeball was a little protruded and the grade of protrusion had slowly advanced as she had grown up. About a year ago the patient suffered from sudden attack of severe ocular pain associated with vomiting. Having experienced the recurrences of similar attack several times, she at last in . Sept. 1969 met a spontaneous outburst of the extruded mass from which the purulent and bloody contents. flowed out. It was to be regretted that the history could not reveal when and how the globe had been disorganized as completely as such that no trace of configuration of the anterior segment could be recognized
Familial and past history were not contributory except measly with good recovery at 2 years of age.
On examination, the left eye had good vision and no structural changes, whereas the right side. showed a marked extrusion of fleshy mass out of the lid aperture. The mass had a relatively smooth and congested coverage of the conjunctiva except it had the fistulae through which a small amount of the purulent and bloody discharge flowed out under the digital pressure. It was felt somewhat elastic. and movable with a broad base into the orbit On its surface no trace of the cornea or the other global structures could be found. Physical examinations of the whole body including regional lymphnodes, revealed normal.
X-ray films of chest, skull and optic foramina were normal.
On 14 Oct. 1969 under general anesthesia an orbital exentration was undertaken because of either probability of a malignant tumor or a long standing intraocular inflammation. In the way of procedure necrotic contents bursted out by mechanical pressure through the weak point, i.e. the fistulae, and correspondingly the tumorous mass became softened. Confirming no hard mass palpable into the orbit, the operating procedure shifted to do partial exentration. The extirpated mass was subjected to the histological study.
Grossly, the dissected area revealed that it contained a well preserved structure of sclera on the one side and completly necrotised material on the other, but it did not contain any remnants of the ordinary global structures such as cornea, iris, lens, vitreous and choroid. The hollowed space partially surrounded by scleral structure was filled with the dark and gelatinous material. The mass was as a whole rather soft.
Hitological examination showed numerous gland like proliferations composed of poorly differentiated and unpigment columner cells having relatively large nuclei, some of which showed a mitotic figure. These were conceivable to be of an embryonic retina.
The lumina of the gland like structures with the various sizes and shapes were filled with a pink staining coagulum and an extremly delicate and regularly arranged fibrillar structure. In some parts the tumor consisted of solid cellular conglomeration with rosett like arrangement and in the other necrotic masses containing tumor cells in all stages of degeneration. For the most part and the surrounding structure of the tumor cells, partly vessels being mostly of capillaries and recent hemorrhages, but the cartilage was not found.
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