Clear-cell chondrosarcoma of the femoral head

Clinical Cases 02.07.2003
Scan Image
Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 57 years, female
Authors: Papanagiotou P, Karantanas AH, Zibis AH, Hantes M , Malizos KN
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AI Report

Clinical History

Three-month history of pain in the right hip.

Imaging Findings

The patient presented with a three-month history of pain in the right hip. X-ray images of the pelvis showed a well-defined lytic lesion in the right femoral head, with central matrix calcification (Fig. 1). A Computed Tomography scan demonstrated the lesion with sclerotic rim and central calcification (Fig. 2). Magnetic resonance (MR) imaging revealed a low signal intensity lesion on T1-weighted images (Fig. 3a), while on T2-weighted images the non-mineralised matrix had high signal intensity. There was no oedema surrounding the lesion (Fig. 3b). After intravenous gadolinium administration the lesion was enhanced in all but the areas of calcification (Fig. 3c).

With these findings, the differential diagnosis included chondroblastoma and clear-cell chondrosarcoma (CCCS). Because of the absence of oedema and inflammatory tissue, the size of the lesion, its extension to the metaphysis, and the age of the patient, the most probable diagnosis was CCCS. The histopathological examination confirmed this diagnosis.

The treatment included surgical resection with total hip arthroplasty (Fig. 4).

Discussion

Clear-cell chondrosarcoma (CCCS) is a rare cartilaginous tumour of low-grade malignancy, representing about 2% of all chondrosarcomas. Its clinical, roentgenographic, and pathological characteristics separate it from conventional chondrosarcoma. The most common location of CCCS is the epiphysis of long bones, especially of the proximal femur and humerus. It affects adults in the third to fifth decades of life and males more frequently than females (1, 2).

The histological hallmarks of CCCS are the large tumour cells with distinct boundaries and clear cytoplasm. Areas of conventional chondrosarcoma may occur within the tumour (2).

Radiologically the tumour appears as a well-demarcated, often calcified lytic lesion at the epiphysis of a long bone. The lesion is usually slightly expansile with a sharp margin between the tumour and the adjacent normal bone and extends often to the metaphysis. The size of CCCS is usually 4-8cm in diameter (3).

MR imaging is useful for the assessment of the extent of the tumour. On T1-weighted images the lesion is most often demonstrated with low signal intensity. The absence of oedema around the lesion is characteristic of CCCS. After intravenous gadolinium administration the tumour is usually enhanced except for areas of calcification (4).

CCCS is often radiologically indistinguishable from chondroblastoma and therefore frequently presents diagnostic difficulties. Chondroblastoma occurs in younger patients, is smaller and more confined to the epiphysis. It also presents with bone marrow oedema and periosteal reaction, which are uncommon in CCCS. Histological examination is necessary for the final diagnosis (4).

The treatment of CCCS is "en bloc" recection. The prognosis is good with a 5-year survival rate of more than 80% (1, 5).

Differential Diagnosis List

Clear-cell chondrosarcoma of the femoral head

Final Diagnosis

Clear-cell chondrosarcoma of the femoral head

Liscense

Figures

Lytic lesion of the right femoral head

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Lytic lesion of the right femoral head

CT

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CT

MR imaging

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MR imaging
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MR imaging
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MR imaging

Gross specimen

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Gross specimen