Giant Cell Tumor
Giant cell tumor of bone is defined as an intramedullary bone tumor, with a specific predilection for age and location, composed of mononucleated cells and osteoclast-like multi-nucleated giant cells, and having a variable and unpredictable potential for
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Definition Giant cell tumor of bone is defined as an intramedullary bone tumor, with a specific predilection for age and location, composed of mononucleated cells and osteoclast-like multinucleated giant cells, and having a variable and unpredictable potential for growth 1• The giant cell tumor usually behaves as a benign tumor, but has a significant tendency to recur locally and, rarely, may produce pulmonary metastases. These have the same histological features as the primary tumor and are frequently curable. Rarely, a giant cell tumor may transform into a high-grade sarcoma.
Epidemiology The frequency is approximately 1/million/year, about half that of osteosarcoma. Sarcomatous change occurs in less than 5% of all giant cell tumors. Giant cell tumor has a slight preference for females (in contrast to most tumors of bone). It usually occurs in young adults, 15-20 to 40-50 years old. It is rare in the skeletally immature subjects (about 2% of the cases), and also rare after the age of 50 years.
Localization Giant cell tumors have a remarkable preference for the end of the long bones. The distal femur and the proximal tibia, in that order, are the preferred sites, and over 50% of the cases are located around the knee. Less frequent locations are, in decreasing order, the distal radius, proximal femur, proximal humerus, distal tibia, proximal fibula. Amongst the flat and short bones, the giant cell tumor infrequently occurs in the spine, particularly lumbar, and in the sacrum, followed by the pelvis and tarsal bones. It is rare in the tubular bones of the hand and foot, and truly exceptional in the scapula, ribs, sternum, clavicle and skull (particularly sphenoid). In long bones the tumor usually involves both the metaphysis and epiphysis. There are occasional cases in the adult where it is centered in the metaphysis. The rare tumors occurring
1 The giant cell tumor of bone was first defined in 1940 by Jaffe, Lichtensein and Portis, who differentiated it from other bone lesions containing osteoclast-like giant cells, such as histiocytic fibroma, chondroblastoma, and aneurysmal bone cyst.
M. Campanacci, Bone and Soft Tissue Tumors © Springer-Verlag Wien 1999
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