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CASE REPORTS
Large Chondrosarcomas of The Shoulder Girdle - A Report of Three Cases
A Duggal, S Gupta, A Puri, MG Agarwal
Chondrosarcoma is a primary malignant tumour of the bone characterized by differentiation of cartilage cells and associated matrix. Conventional chondrosarcoma is the most common malignant neoplasm in the chondrogenic group. It is subdivided in a variety of ways but the single most important prognostic sub-classification is the histological grade.1,2 Chondrosarcomas are known to grow to large sizes in the pelvis.3 Chondrosarcomas of the shoulder girdle, because of their superficial location are generally detected early. We present three cases of unusually large chondrosarcomas around the shoulder girdle.
CASE 1
A 55 year old male was referred to us with the history of a gradually increasing painless swelling of the back for the last fifteen years (Fig. 1).
On examination a 40 cm x 20 cm x 17 cm hard, non-tender swelling was seen over the back on the right side. The overlying skin showed three areas of ulceration extending into the sub-cutaneous tissue. Movements of the right shoulder joint were restricted. The axillary artery pulsations were felt over the anterior chest wall due to displacement by the large radiolucent mass with speckled calcifications.
Plain and post-contrast helical CT-scans for the thorax and right shoulder with 8-mm collimations revealed destruction of almost the entire right scapula (except for the acromian process) by a large soft tissue mass which showed extensive coarse calcifications within it. There was no extension into the thoracic cavity.
There was no clinical or radiological evidence of metastases. Biopsy from the lesion showed a differentiated chondroid lesion in favour of a chondrosarcoma.
At surgery a large well-circumscribed mass was seen extending laterally into the axilla and superiorly in the supraclavicular fossa. Wide excision of the tumour along with right scapulectomy was performed and a primary closure achieved. The specimen weighed 15 kg and measured 44 cm x 30 cm x 17 cm. The histopathological examination of the excised tumour revealed a conventional grade I chondrosarcoma.
The patient had an event-free postoperative course and is doing well with no evidence of local recurrence of metastases at one-year follow-up.
CASE 2
A 40 year old male presented to us with history of swelling over the left side of the back for the last 14 years. It was excised ten years back but the swelling recurred after three years and had been growing since then (Fig. 2).
On examination a 30 x 30 cms bosselated mass with 5 x 5 cms ulceration was present over the left scapula. The mass was non-tender except in the area of ulceration. There was neither restriction of movement at the left shoulder joint nor any distal neuro-vascular deficit. Roentgenograms revealed a mixed lytic-sclerotic lesion arising from the left scapula with a wide one of transition.
There was an associated large soft tissue mass with areas of punctate calcification. CT-scans of the scapula and thorax revealed a sclerotic lesion with irregular margins involving the infero-lateral margin of the left scapula with a large associated soft tissue mass showing coarse calcification. Core biopsy from the lesion showed a well-differentiated chondrosarcoma.
There was no clinical or radiological evidence of metastases.
The patient underwent wide excision of the lesion and a partial scapulectomy. The mass weighed six kgs and measured 30 x 21 x 17 cms. (Fig. 3)
The final histo-pahological report was well-differentiated chondrosarcoma of the scapula involving the periscapular soft tissues and overlying skin.
CASE 3
A 38 year old male was referred to us with a large swelling in the left axillary area. On examination the patient had three swellings in the left axilla and proximal arm. The largest swelling measured 20 cms x 15 cms. The other two were 10 x 10 cms and 10 x 5 cms respectively. The left shoulder was kept in an attitude of abduction due to the axillary swellings. There was no distal neuro-vascular deficit.
Roentgenograms revealed a large soft tissue mass in the left axillary area with punctate calcifications. There was no bony involvement however.
MRI revealed a soft tissue mass involving the left axilla and extending into the arm. The mass infiltrated the flexor group of muscles and the major neuro-vascular bundle was encased by the mass. The humerus was uninvolved.
Tru-cut biopsy from the lesion revealed a well-differentiated chondrosarcoma.
Limb salvage was considered unlikely due to the neurovascular encasement. The decision of amputation was deferred till imaging findings were confirmed intraoperatively. The patient was taken up for surgery with a prior consent for shoulder disarticulation if considered unsalvageable. During surgery the tumour was found to be widely infiltrating the median and ulnar nerves and the brachial artery. Limb salvage was not considered possible and shoulder disarticulation was done. The final histopathological report revealed an extra-skeletal chondrosarcoma.
DISCUSSION
Chondrosarcoma is the second most common primary malignant spindle cell tumour of bone.1 Chondrosarcomas form a heterogeneous group of tumours whose basic neoplastic tissue is cartilaginous without any evidence of direct osteoid formation. The five types of chondrosarcomas are central, peripheral, mesenchymal, dedifferentiated and clear cell.4
Central chondrosarcomas arise from within the medullary canal whereas the peripheral ones originate from the surface of the bone. Chondrosarcomas are also classified as primary or secondary. Primary chondrosarcomas arise de novo and are not associated with any pre-existing lesion. Secondary chondrosarcomas arise in benign chondroid tumours like solitary and multiple osteochondromas and multiple enchondromas.5
About one half of chondrosarcomas occur above the age of forty years. The most common sites are the pelvis (31%), femur (21%) and the shoulder girdle (13%).6 The clinical presentation varies. The peripheral chondrosarcomas may become large without causing pain and local symptoms develop only due to mechanical irritation. In India, due to lack of awareness and proper medical facilities many patients even with tumours in superficial areas, present quite late, as is seen in the cases presented.
A primary central chondrosarcoma has a typical radiographic appearance. It is usually a metaphyseal lesion showing bone destruction with intralesional calcification and periosteal reaction. Due to the complex cortical shape and narrow medullary diameter of the major flat bones, chondrosarcomas of the pelvis and the scapula may be difficult to discern in plain films, especially if calcific mottling is absent.
Histologically the chondrosarcomas as graded as grade I, II and III lesions is 47%, 38% and 15% respectively.7 However, Marcove et al8 in their study found no significant difference in survival in grade I or II chondrosarcomas but mortality rate of grade III was significantly higher.
Lee et al11 pointed out that the rate of local recurrence was not found related to the operative margins in the patients who has a low-grade tumour; however, such a relationship was detected in the patients who had a high-grade tumour.
The fact that, patients in our setting present with large tumours should not, per se, make them candidates for mutilating ablative procedures. As illustrated by the cases presented, limb salvage was possible in two of the three very large chondrosarcomas, as they were of low grade and oncologic principles were not violated during surgery.
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