Giant Chondrosarcoma in Ollier’s Disease

Case Report

Austin Biomark Diagn. 2015; 2(2): 1022.

Giant Chondrosarcoma in Ollier’s Disease

Zvonimir Zore* and Mladen Stanec

Department of Surgical Oncology, University Hospital Center Sestre milosrdnice, Zagreb, Croatia

*Corresponding author: Zvonimir Zore, Department of Surgical Oncology, University Hospital Center Sestre milosrdnice, Zagreb, Croatia

Received: July 08, 2015; Accepted: November 24, 2015; Published: November 26, 2015


Ollier’s disease; Enchondromatosis; Chondrosarcoma


A 30-year-old male presented with increasing tumors size, limited motion and pain in the left upper arm of approximately 5 years duration, surgically treated 15 years ago. Final histopathological exam was confirmed clinically diagnosis, that it was well-differentiated giant chondrosarcoma. The patient is alive and well 15 years after amputation upper limb.


Enchondromas (Multiple Enchondromatosis, Dyschondroplasia, and Enchondromatosis) are common intraosseous benign cartilaginous tumors. When multiple enchondromas are present, the condition is called enchondromatosis, also known as an Ollier’s disease by the terminology of the World Health Organization [1]. Ollier’s disease is very rare. The estimated prevalence of Ollier’s disease is 1/100,000 [2]. Boys are affected twice as often as girls. The disease tends to be bilateral but predominate on one side and primarily affects the long bones and cartilage of the joints of the arms and legs. It may occur at anytime. Symptoms may also arise secondary to mass effect resulting in pain and loss of motion, compression or stretching peripheral nerves and pathological fractures. The diagnosis is based on clinical and conventional radiological evaluations. Surgery is indicated in case of complications: pathological fractures, growth defect and malignant transformation. The reported incidence of malignant trasformation (e.g., chondrosarcomas) is variable and estimated to occur in 5-50% [3].

Chondrosarcoma-Case Report

In 1998, a 30 year old male patient was presented with a dominant tumorous mass of the proximal left humerus (Figure 1). He had been operated few ways, last operation was performed for 10 years previously because he had multiple exostosis since childhood. The patient has had not completed documentation for previously operations, only for last operation. That operation has been done on both forearms because there was exostosis. After that patient has had fractures of both forearms. Physical exam showed no abnormalities except a tumorous mass of proximal left humerus sized approximately 30 cm, reduced length of both forearms and exostosis on both proximal lower legs.Patient has had two times a transient peripheral paralysis of nerves facials. Intraoral findings are massing on the hard palate look like torus palatinus (exostosis on the hard palate). In patients family nobody has the same mass. Plain radiograph shows a large ill-defined, destructive, humerus metaphysis intramedullary lesion with bone destruction and periosteal reaction (Figure 2). The lesion is associated with a soft tissue mass. Furthermore, plain radiographs showed hiperostotic change of the lateral part of right clavicle, metaphysis of neck and trochanter of both femurs, bones of head and intraoral findings. Scintigraphy showed enhanced accumulation radionuclide on proximal right humerus, right shoulder and right trochanter of femur. CT demonstrated chondrosis of metaphysis of right humerus and cranial part of the shoulder blade. Preoperative biopsies showed chondrosarcoma.

Citation: Zore Z and Stanec M. Giant Chondrosarcoma in Ollier’s Disease. Austin Biomark Diagn. 2015; 2(2): 1022. ISSN:2378-9867