Ew. Brien et al., BENIGN GIANT-CELL TUMOR OF BONE WITH OSTEOSARCOMATOUS TRANSFORMATION (DEDIFFERENTIATED PRIMARY MALIGNANT GCT) - REPORT OF 2 CASES, Skeletal radiology, 26(4), 1997, pp. 246-255
It is not uncommon for sarcomatous transformation of giant cell tumor
(GCT) of bone to occur af?er radiation, but rarely does malignant tran
sformation occur spontaneously, with less than 5 cases reported up to
1995. Only four of these cases have been documented in detail, We repo
rt two additional cases of GCT of bone spontaneously transforming or '
'dedifferentiating'' into osteosarcoma without radiation therapy. The
first case is absolutely unique and most interesting in that the dedif
ferentiation process occurred in one of multiple GCT lung metastases 6
years after successful eradication of a primary tibial tumor. The rig
ht lung was resected due to development: of a large tumor, and at path
ologic examination, demonstrated several small nodules of conventional
GCT and a much larger, 14-cm mass composed of a mixture of GCT and hi
gh-grade osteosarcoma. The second case involved a physician, who had a
large tumor in the sacrum with vague symptoms for 8 years. Open biops
y revealed conventional, benign GCT of bone with a secondary aneurysma
l bone cyst. Complete curettage 2 weeks later revealed, in addition to
areas of conventional, benign CCT a second component of very high gra
de osteosarcoma. Both patients died less than 1.5 years from diagnosis
. This report of osteosarcomatous transformation of a conventional GCT
of bone strengthens the theory that there is a mesenchymal cell line
in CCT that may spontaneously tansform to sarcoma.