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[The initial clinical manifestations of rhabdomyosarcoma]
H D Schworm1, K P Boergen, F H Stefani
1Augenklinik, Universität München.
Abstract:
Unilateral proptosis, dislocation of the globe and impairment of motility in childhood are known to be typical presenting signs of rhabdomyosarcoma of the orbit. In a retrospective analysis of 17 patients with rhabdomyosarcoma (1974-1993) only 8 patients presented with typical signs. 9 patients did never show the typical signs which initially lead to a wrong diagnosis in many cases. A 5-year-old boy complained of a conjunctival cyst in the lower fornix of the left eye. Two weeks later the cyst was replaced by a solid tumor. The tumor was not completely resectable, histology revealed an embryonal rhabdomyosarcoma. Presenting signs and symptoms of orbital rhabdomyosarcoma show a sometimes misleading variety including conjunctival cyst formation. In any recent onset of unilateral rapidly progressing alterations of the lid, the conjunctiva or the caruncle in childhood the physician should consider a rhabsomyosarcoma of the orbit.