Multiple sarcomas in Maffucci's syndrome

The association of dyschondroplasia with haemangiomata is known as Maffucci's syndrome (Maffucci, 1881). In spite of its rarity, it is a well-recognised clinical entity. Its predominance in males is striking. In a classical case, the patient is normal at birth and the bony lesions in the form of small nodules usually appear in the prepubertal years. These may be unilateral or markedly asymmetrical. They may interfere with the growth of long bones leading to low stature or unequality in the development of the two sides of the body. The vascular malformationc consists of multiple haemangiomata and phlebectasia in the subcutaneous tissues which appear as bluish soft compressible tumours. Both the bony and vascular abnormalities are undoubtedly congenital in origin but there is no evidence that they are inherited. The vascular lesions do not always follow the bone lesions despite their association. The chondromas may involve any bone but are more common in the hands, feet and long bones. The skull, the carpus and tarsus though not exempt are rarely involved (Carleton, Elkington, Greenfield and Robb-Smith, 1942). Strang and Rannie (1950) described a case in which a chondrosarcoma arising from the basisphenoid was found and referred to three cases in which the skull was involved.