Sarcomatous Degeneration Bone Exostosis

Clinical Cases 12.02.2002
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 20 years, female
Authors: A. Keller, V. Prues-Latour, A.M. Kurt
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AI Report

Clinical History

Clinical and imaging findings, and the age of the patient were suggestive of a sarcomatous transformation of a unique bone exostosis.

Imaging Findings

The patient with no medical history other than the appearance of a swelling over the right proximal tibia one year previously. This lesion was initially painless, but more recently direct contact began to trigger pain.
Radiography of the right knee A-P view shows sessile exostosis arising from the anterior surface of the right tibia surrounded by a soft tissue mass without calcification.
Bone scintigraphy - Tc99m MDP (not shown) demonstrates intense focal hyperactivity of a solitary tibial lesion.
MR of the knee includes an axial GRE T2-weighted image which shows an exostosis covered with a cartilaginous cap which demonstrates a higher signal than all other tissues except fat. On Gd-enhanced sagittal SET1-weighted image there is rim of intense Gd-enhancement of the lesions surface and a focal thickening of the cortical bone underlying the exostosis. The signal of the marrow is normal.
Pathology of the resected specimen reveals on the microscopic view (EH* 100) replacement of marrow fat by cartilaginous tissue which is trapped by host lamellar bone. On microscopic view (EH* 400), compared with normal cartilage the cellularity is increased, and the chondrocyte nuclei show an irregular size and sometimes binucleation.
Clinical and imaging findings, and the age of the patient were suggestive of a sarcomatous transformation of a unique bone exostosis. Pathological examination confirms the diagnosis of grade I chondrosarcomatous degeneration. The continuity of the cortical and trabecular bone with the exostosis excludes a juxta- of paraosteal sarcoma.

Discussion

Osteochondromas or exostoses are benign, pediculated or sessile outgrowths arising from the surface of a bone near the metaphysis. They haves a cortex and a medullary cavity which are contiguous with the host bone and are covered by a hyaline cartilage cap. Their size varies from 1 cm to more than 40 cm. They are localized on any bone which develops by enchondral ossification, especially the femur, humerus, and tibia. Osteochondromas may be solitary or multiple (autosomal dominant) and occur spontaneously or following injury or irradiation. 70-80% of solitary osteochondromas occur in patients younger than 20 years of age. Potential complications include fracture, vascular injury with pseudoaneurysm, neurologic compromise, bursa formation and malignant transformation. The risk of degeneration varies from 1 to 27% depending on whether the patient has a solitary or multiple osteochondroma(s). The resulting tumor most commonly is a chondrosarcoma. The signs of malignant transformation are enlargment of the cap, irregular cartilaginous calcifications and poorly defined margins.
On imaging, the major difficulty in differential diagnosis arises in distinguishing between a benign osteochondroma and a peripheral chondrosarcoma. Radiographic criteria for chondrosarcoma are a large size, lysis with ill-defined densities and margins, bone expansion, focal thickening versus thinning of cortices or a broad cortical scalloping. In clinical practice, this differential diagnosis is resolved by the anatomopathologic examination. Increased cellularity associated with enlarged chondrocyte nuclei, binucleation and occasional mitotic figures represent cytologic evidence of malignancy. Histologic criteria for malignancy are replacement of marrow fat by islands of cartilage which are trapped into the host lamellar bone, conversion of marrow fat into bands of fibrous tissue and invasion of haversian systems.

Differential Diagnosis List

Sarcomatous transformation of a unique bone exostosis

Final Diagnosis

Sarcomatous transformation of a unique bone exostosis

Liscense

Figures

Radiography of the right knee

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Radiography of the right knee

MR of the knee

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MR of the knee
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MR of the knee

Microscopic view

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Microscopic view
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Microscopic view