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Eur J Cardiothorac Surg 2002;22:147
© 2002 Elsevier Science NL

Images in cardio-thoracic surgery

Giant chondrosarcoma and multiple hereditary exostoses: a very rare case report

Unal Sakinci, Erkan Yildirim*, Koray Dural, Serdar Han

Department of Chest Surgery, Ankara Numune Education and Research Hospital, Ankara, Turkey

Received 23 November 2001; received in revised form 20 February 2002; accepted 25 March 2002.

* Corresponding author. Tel.: +90-312-482-7279; fax: +90-312-310-3460
e-mail: erseyda{at}yahoo.com

A giant chondrosarcoma of the anterior chest wall together with multiple hereditary exostoses of the lower extremities is presented. The 20-year-old male patient had been living with the lesion for 14 years during which time it had grown to giant dimensions (Fig. 1) . The tumour was excised en bloc and the defect was repaired using just skin flaps. The pedigree tree is shown in Fig. 2 .



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  		Fig. 1. The giant mass lesion (chondrosarcoma) of 20x18x20 cm dimensions originating from the right arcus costarium together with bilateral multiple exostoses at the lower extremities are seen.

 


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  		Fig. 2. Pedigree tree. Individual IV 6, who transmitted the gene to his children, died of leukemia. Proband is the only one who had chondrosarcoma. The patient's chromosome analysis revealed normal karyotype with polymorphism of 46, XY, Inv 9 (p1.1, q1.3). These could have been an example of EXT 1 mutation and LOH on this locus.

 




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