Osteochondromyxoma of bone - A congenital tumor associated with lentiginesand other unusual disorders

This article describes the clinical and pathologic features of four unusual bone tumors. Three were congenital or most likely so: the fourth, detected at age 1 year, was probably of considerable duration. The patients. three boys and one girl, each presented with a painless mass. Two had the Carney complex, a familial lentiginous and multiorgan tumorous syndrome: another p robably had this disorder: the fourth did not show it. but his mother did. The tumors occurred in the nasal region (n = 2) and the diaphysis of the ti bia and radius (n = 1 each). Roentgenographically. three had benign charact eristics; the fourth, malignant features. Grossly, the tumors were gelatino us, cartilaginous, and bony. Microscopically, they featured benign-appearin g polymorphic cells with few division figures arranged in sheets and lobule s set in a myxomatous. cartilaginous, osseous, and hyaline fibrous matrix. Cellularity was low to moderate. The tumors eroded bone, one infiltrated be tween bony trabeculae. and three had soft tissue extension. Complete resect ion of one tumor was curative: incomplete excision of two tumors resulted i n local recurrence (intracranial and fatal) in one and persistence in the o ther: the fourth tumor remains under observation after biopsy. No tumor met astasized.