Classic osteosarcoma

Clinical Cases 16.11.2002
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 15 years, male
Authors: R. Perriss, M. Court-Payen, H.S. Thomsen
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AI Report

Clinical History

Swelling of the lower thigh.

Imaging Findings

The patient presented to the emergency department with a one-week history of swelling of the lower right thigh. There had been no direct trauma, but the patient had attended a football training weekend one week previously. Examination revealed a swelling medial on the right thigh, just above the knee. There was no erythema and knee movements were normal. The patient underwent ultrasound scanning initially to exclude a haematoma. This revealed a well-vascularised mass arising from the femur (Fig. 1), raising the strong possibility of a malignant bone tumour.

A plain film revealed all the classic signs of an osteosarcoma: bone destruction, new bone formation with a "sun ray" pattern, Codman's triangle (periosteal elevation at the edge of the tumour) and a soft tissue mass (Fig. 2). Subsequent MRI scanning revealed an intramedullary mass in the metaphysis of the femur with a breach of the cortex and a large extramudullary component (Fig. 3).

There were no signs of skip lesions in the rest of the femur or upper tibia. A plain chest film was normal. A biopsy confirmed the diagnosis of high-grade conventional osteosarcoma. Chemotherapy was initiated.

Discussion

Though not a common tumour overall, representing just 0.2% of all malignant tumours, osteosarcoma is the second most frequent primary malignant neoplasm of bone after myeloma. The peak incidence of primary osteosarcoma is between 10 and 25 years of age and it is more common in males. Osteosarcomas can also arise secondarily in long standing Paget's disease and after radiation exposure. The tumour tends to metastasise early to the lungs and, less commonly, spreads to regional lymph nodes; there is also a 25% incidence of "skip" metastases, which are simultaneous foci of osteosarcoma either within the same bone or across an adjacent joint. Around 75% of osteosarcomas are of the classic or conventional type and they arise most commonly in the metaphysis of a long bone with 50–75% occurring around the knee.

Plain radiography is still the examination of choice in the initial assessment of a bone tumour. Classic osteosarcoma is typically seen as an ill-defined intramedullary lesion in the metaphysis, with extension through the cortex and formation of a soft tissue mass. Most commonly both bone destruction and formation are present, though purely osteolytic or osteosclerotic lesions can occur. Rapid formation of immature bone leads to the "sun ray" appearance. Periosteal reaction in the form of Codman's triangle is also common.

Scintigraphy has been shown to commonly overestimate the extent of malignant bone tumours and this role has been taken over by CT and MRI. CT is also the best modality for detecting lung metatases.

Today MRI has a central role in the assessment of malignant bone tumours. It is the best modality for local staging as it shows well both the intra- and extraosseous extent of the tumour, as well as neurovascular, epipyseal and joint involvement. Scanning of the whole bone is used to exclude skip lesions. MRI also plays a key role in assessment of response to chemotherapy and follow-up after surgery. Gadolinium enhancement is primarily used to monitor tumour response. Treatment of osteosarcoma consists of a combination of neoadjuvant chemotherapy and surgery, which in 90% of cases now is a limb sparing procedure. Advances in imaging, chemotherapy and surgery have led to a dramatic improvement in the prognosis of osteosarcoma over the last two decades, with a rise from 5% 5-year survival to around 50-75% today.

Differential Diagnosis List

Classic osteosarcoma

Final Diagnosis

Classic osteosarcoma

Liscense

Figures

Ultrasonography

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Ultrasonography
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Ultrasonography
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Ultrasonography
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Ultrasonography

Plain radiography

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Plain radiography
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Plain radiography

MRI

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MRI
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MRI
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MRI
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MRI