J Korean Ophthalmol Soc.  1967 Jun;8(1):69-72.

A Case of Orbital Rhabdomyosarcoma

Affiliations
  • 1Department of Ophthalmology, College of Medicine Pusan National University, Pusan, Korea.

Abstract

A 54-year-old farmer, Korean, was first seen in March of 1965, with complaints of frontal headache and ocular pain, proptosis, and blindness OD of 5 months duration. The onset was insidious. Past history and family history were not contributory. On examination, left eye was within normal limit. Right eye was displaced down- and in ward and there was moderate proptosis (about 6mm. of exophthalmos). The upper lid was partially ptotic and immobile. The ocular motility was markedly limited to those directions of lateral, upper lateral and lower lateral and moderately to the directions of the rest. Pupil was mydriatic. No mass could be felt in the orbital region. Funduscopic examination revealed pallor optic disc. There were neither papilledema nor retinal fold. X-ray films of chest, skull and optic foramen were negative except inactive signs of old tuberculosis of chest. In March of 1965, attempting to obtain a biopsy, the author adopted the Kronlein's approach by which a white-gray firm mass located in the retrobulbar space, enclosing the optic nerve firmly was taken out. For the mass was diagnosed as sclerosing angioendothelioma, irradiations of X-ray was recommended. About 7 months later, in October of 1965, the patient readmitted and then exenteration of the orbital contents was carried out. The tumor mass was attached firmly to the orbital wall at the apex and the posterior part with a considerable bony destruction. The mass taken out was a rather soft mass measuring 4 by 4 by 4 cm, and its cut surface was homogenously gray with mottled surface. of necrotic and hemorrhagic areas. Histologicatly, the mass was composed of pleomorphic bizzarre cells with hyperchromatic, abnormally mitotic nuclei, which are freely floating in alveolar lumina surrounded by well vacularized scanty fibrous septa. Cross striations could be rarely be found in high power field. Pathological diagnosis confirmed as alveolar rhabdomyosarcoma. It is suggestive that early loss of vision is caused by an optic atrophy due to direct pressure by the tumor.


MeSH Terms

Biopsy
Blindness
Diagnosis
Exophthalmos
Headache
Humans
Middle Aged
Optic Atrophy
Optic Nerve
Orbit*
Pallor
Papilledema
Pupil
Retinaldehyde
Rhabdomyosarcoma*
Rhabdomyosarcoma, Alveolar
Skull
Thorax
Tuberculosis
X-Ray Film
Retinaldehyde
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