NOONAN-SYNDROME ASSOCIATED WITH CENTRAL GIANT-CELL GRANULOMA

We report a case of Noonan syndrome associated with central giant cell granuloma. The patient was a 10 1/2-year-old boy with the chief compl aint of proptosis of the right eye. He also had various malformations such as short stature. webbed neck, pectus excavatum, cubitus valgus, pulmonary valve stenosis and patent foramen ovale, a characteristic fa ce appearance and cryptorchidism and so on. Chromosome analysis showed a 46,XY karyotype. A computed tomographic scan and magnetic resonance imaging showed a mass originated from the lateral wall of the right m axillary sinus. The patient underwent Caldwell-Luc operation. Histolog ical examination of the mass showed the characteristics of central gia nt cell granuloma. This case report describes a patient with the featu res of the recently described Noonan-like/multiple giant cell lesion s yndrome.