A Case of Alveolar Soft Part Sarcoma in the Orbit

ABSTRACT

Alveolar soft part asrcoma is a primary malignant neoplasm that very rarely occurs in the orbit and it's histogenesis is uncertain. Authors reported a case of alveolar soft part sarcoma occurred primarily in the orbit. The patient, 58 years old Korean male, was admitted to Busan National University Hospital on June 9, 1972 with the chief complaints of the protrusion of the orbital content and pain in the left eye which were gradually developed after receiving enucleation for treatment of the orbital tumor, lasted for 3 months, on Feb. 21, 1972 at local clinic. The patient family history was not contributory. His medical history revealed prior episodes of typhoid fever, liver disease, and a chronic ethanol intake. Physical examination revealed chronic ill appearence in general condition, absent breathing sound at the right lower quadrant of the chest and 3 finger palpable liver. On eye examination, the right eye had good vision and no structual abnormalities, whereas the left side showed anophthalmos, protrusion of the intraorbital content on which surface was rather soft and smooth. We found tortous and dilated superficial skin vessels on the zygomaticotemporal area at which a soft mass was palpated in small fist size. On X-ray studies, there were bony destructive lesion on superior and lateral orbital margin in the skull, atlectatic pattern on the right lower lobe and notching pattern on the right diaphragm in the chest, and other long bone series showed no specific abnormal findings. On liver scanning, mottled areas on the right lower lobe were noted. There were no abnormal findings in C.B.C., urinalysis, C.S.F., stool and blood chemistry. The further studies for the abnormal findings containing liver lesion and atlectatic lung pattern could not perform because of patient's incooperation. We tried the exenteration of left orbit under the general anesthesia on July 8, 1972 but biopsy only performed because the tumor seemed to be extended to pterygoid fossa through the destructed zygomatic bone and to adjacent orbital bone. Histopathological findings showed striking features of alveolar soft part sarcoma; The tumor cells were arrlinged in alveolated or organoid patterns with delicate fibrous band or endothelium lined spaces. The cells were characterized by large, round, oval, and polyhedral cells with rather abundant cytoplasm, which were acidophilic and occasionally contained fine granules and vacuoles in H-E stain. The nuclei were almost located in eccentric, and binucleated, hyperchromatic, anaplastic and occasional mitotic figures were evident.