Secondary Chondrosarcoma of the Proximal Part of the Humerus Arising in a Four-Year-Old Boy with Ollier Disease: A Case Report.

Abstract

Chondrosarcomas are the second most common primary malignant bone tumors; they usually affect older individuals, with a peak incidence in the fourth to sixth decades of life1. They can be classified as primary or secondary, according to origin. Primary chondrosarcoma arises de novo in normal bones, whereas secondary chondrosarcoma arises from preexisting benign lesions such as osteochondroma, or enchondroma in Ollier disease and Maffucci syndrome. Secondary chondrosarcomas are rarely encountered, accounting for approximately 8% to 20% of all chondrosarcomas2-4. Although secondary chondrosarcoma affects younger individuals more frequently than primary chondrosarcoma, to our knowledge, its occurrence in children younger than ten years of age has not been documented previously. We report the case of secondary chondrosarcoma in a young boy with Ollier disease. The patient and his family were informed that data concerning the case would be submitted for publication, and they provided consent. A four-year-old boy was brought to a local hospital with a one-week history of right shoulder pain. He had no contributory medical or family history. Radiographs revealed a geographic, mildly expansile, lytic lesion with mild cortical thinning and slight intratumoral calcification in the proximal part of the right humerus (Figs. 1-A and 1-B). Computed tomography (CT) demonstrated multiple additional bone lesions in locations other than the humerus, including the right scapula and radius, with a unilateral distribution suggestive of enchondromatosis. A sample obtained from the humeral lesion by needle biopsy via a transdeltoid approach was diagnosed as enchondroma at another hospital, suggesting a diagnosis of Ollier disease with multiple lesions in the right upper extremity. Periodic medical examinations were performed; six months after the biopsy, the tumor started to show a rapid increase in size. The patient was referred to our hospital for additional evaluation and management. Fig. 1 Anteroposterior ( Fig. 1-A ) and …