Benign giant cell tumor of bone with osteosarcomatous transformation (”dedifferentiated” primary malignant GCT): report of two cases |
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Authors: | E W Brien Joseph M Mirra Steven Kessler M Suen J K S Ho W T Yang |
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Institution: | (1) Musculoskeletal Tumor Service, Orthopaedic Hospital, 2400 Flower St. no. 523, Los Angeles, CA 90007, USA, US;(2) Division of Surgical Pathology, Oregon Health Sciences University, Portland, Oregon, USA, US;(3) Department of Pathology, Chinese University of Hong Kong, Hong Kong, HK;(4) Department of Surgery, Chinese University of Hong Kong, Hong Kong, HK;(5) Department of Radiology, Chinese University of Hong Kong, Hong Kong, HK |
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Abstract: | It is not uncommon for sarcomatous transformation of giant cell tumor (GCT) of bone to occur after radiation, but rarely
does malignant transformation occur spontaneously, with less than 15 cases reported up to 1995. Only four of these cases have
been documented in detail. We report 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 dedifferentiation
process occurred in one of multiple GCT lung metastases 6 years after successful eradication of a primary tibial tumor. The
right lung was resected due to development of a large tumor, and at pathologic examination, demonstrated several small nodules
of conventional GCT and a much larger, 14-cm mass composed of a mixture of GCT and high-grade osteosarcoma. The second case
involved a physician, who had a large tumor in the sacrum with vague symptoms for 8 years. Open biopsy revealed conventional,
benign GCT of bone with a secondary aneurysmal bone cyst. Complete curettage 2 weeks later revealed, in addition to areas
of conventional, benign GCT a second component of very high grade 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 GCT that may spontaneously tansform to sarcoma. |
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Keywords: | Dedifferentiated Primary giant cell tumor of bone Osteosarcomatous transformation of giant cell tumor |
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