Chondrosarcoma of the Scapula

Clinical Cases 20.11.2003
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 19 years, male
Authors: Cebe L, Ly JQ, LaGatta LM
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Details
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AI Report

Clinical History

The patient presented with aching pain in the left shoulder. Shoulder radiography was obtained, which revealed an abnormality prompting further evaluation with skeletal scintigraphy and MR imaging.

Imaging Findings

The patient presented with aching pain in the left shoulder. Shoulder radiography was obtained, which revealed an abnormality prompting further evaluation with skeletal scintigraphy and MR imaging.
Radiography (Fig. 1A) revealed an osteolytic lesion with popcorn calcifications and a sclerotic margin, measuring 3.5 x 4.1 cm, and involving the mid to inferior scapular body. Skeletal scintigraphy (Fig. 1B) showed focal increased uptake at the medial scapular border. At MR imaging, the lesion demonstrated ring and arc contrast enhancement, cortical destruction, and demonstrated signal characteristics consistent with chondroid matrix (Fig. 1C).
The patient underwent partial scapulectomy for tumor removal. Grossly, the mass involved cortical bone and appeared to extend into the adjacent soft tissues. Near the center of the mass there was a dense, irregular calcification. Histopathologic examination (Fig. 1D) showed a cartilaginous neoplasm with lobular architecture, well-preserved chondroid matrix, and variable cellularity. Most of the cells appeared uniform with hyperchromatic nuclei and absence of significant nuclear pleomorphism or mitotic figures. A permeative growth pattern with focal cortical bone penetration was also noted.

Discussion

Chondrosarcomas are malignant neoplasms of cartilage-forming cells and are also the third most common primary malignant tumor of bone. They are the most common malignant neoplasm of the scapula and may arise as a primary bone tumor or as a secondary lesion from a pre-existing enchondroma or exostosis. Most patients with scapular chondrosarcoma present with shoulder pain. Chondrosarcomas can also arise secondarily from other preexisting lesions to include enchondromas, osteochondromas, Paget’s disease, and fibrous dysplasia. The different types of primary chondrosarcoma include conventional intramedullary, clear cell, juxtacortical, myxoid, mesenchymal, extraskeletal, and dedifferentiated. The conventional type is the most common, involving the diaphysis or pelvis in the majority of cases (1).
Radiography usually demonstrates a lytic and sometimes expansile process, and can resemble an aneurysmal bone cyst or giant cell tumor (true aneurysmal bone cysts typically occur in a younger age group and do not contain popcorn calcifications). Periosteal reaction is often seen with chondrosarcomas and is an indication of a more aggressive process. Chondrosarcomas typically will contain ring and arc or popcorn-like calcifications, which can be seen radiographically and at cross-sectional imaging. In general, it is the high grade lesions that result in cortical bone destruction, deep endosteal scalloping, and soft tissue extension. Skeletal scintigraphy usually will show increased uptake. On MR imaging, calcifications appear hypointense on all pulse sequences, while cartilaginous matrix manifests as high T2 and low T1 signal areas, representing the high water content of the hyaline cartilage (2). High grade chondrosarcomas tend to show more variable MR appearance. The typical lobular appearance of chondroid matrix is not seen with the dedifferentiate type. Low-grade chondrosarcomas have been notoriously difficult to differentiate from enchondromas. However, a recent study by Geirnaerdt et al concluded that the combination of early and exponential enhancement correlated significantly with a malignant cartilage-forming tumor (3).
Histologically, chondrosarcoma cells are binucleated with myxoid areas. Permeative borders and invasion of soft tissue are characteristic features and can help to distinguish chondrosarcomas from enchondromas (4).
Local staging is needed to display the extent of the tumor in relation to anatomical landmarks for surgical planning. The standard treatment is surgical excision with the goal of leaving a wide margin; limb sparing resection is associated with a good prognosis for grade I chondrosarcomas. When a grade I chondrosarcoma recurs, it is usually considered then to be a high-grade lesion (5).

Differential Diagnosis List

Low-grade chondrosarcoma of the scapula

Final Diagnosis

Low-grade chondrosarcoma of the scapula

Liscense

Figures

Conventional Radiography of the Scapula

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Conventional Radiography of the Scapula

Skeletal Scintigraphy

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Skeletal Scintigraphy

MRI Scapula

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MRI Scapula

Histology

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Histology