PURPOSE: Multiple hereditary exostoses of the forearm typically form in the distal ulna, causing disturbances in the growth of the ulna and functional disability. Multiple hereditary exostoses inhibit the growth of the ulna, leading to an acquisition of a varus deformity in the radius, which sometimes leads to dislocation of the radial head, the development of limitations in the pronation-supination of the forearm, and cosmetic problems. MATERIALS AND METHODS: We retrospectively reviewed the cases of four patients who had deformities of the forearm with radial head dislocation associated with multiple hereditary exostoses, and evaluated the radiologic and clinical results of excision of the osteochondromas from the distal ulna and gradual ulnar lengthening with an Ilizarov external fixator. RESULTS: Good clinical and radiological results were obtained after a mean follow-up of 25 months. At the most recent follow-up, radial bowing, ulnar shortening, carpal slip, and the pronation/supination arch of the forearm had improved. There was little change in terms of preoperative radial articular angle and the flexion/extension arch of the elbow by the most recent follow-up. CONCLUSION: Treatment of four forearms from four patients by excision of osteochondromas and gradual lengthening of the ulna with an Ilizarov external fixator spontaneously reduced dislocations of the radial heads without the need for any additional operative intervention. All patients were satisfied with the final results.
Adolescent ; Child ; Child, Preschool ; Exostoses, Multiple Hereditary/*radiography/surgery ; Female ; Humans ; Male ; Radius/radiography ; Retrospective Studies ; Ulna/radiography ; Young Adult

OBJECTIVETo explore the clinicopathologic features of Ollier's disease, its chondrosarcomatous transformation and related differential diagnoses.

METHODSA total of 19 cases of Ollier's disease and 8 control cases of pure multiple enchondroma were investigated by imaging studies including X-ray, CT or MRI, and hematoxylin and eosin stain.

RESULTSAmong 19 cases of Ollier's disease, 12 were men and 7 were women with a mean age of 20 years (range, 5-66 years). Ollier's disease involving short tubular bones of extremity were lytic defects with bony expansion, thinning or disappearance of the overlying cortex surrounded by periosteal fibrous tissues. When occurring in the long bones, the disease showed radiolucent columns of dysplastic cartilage that extended from the metaphysis to diaphyseal and created bowing deformation and limb asymmetry. Microscopically, the cartilage present in the small bones of the hands and feet tended to be more hypercellular with more abundant enlarged or binucleated nuclei. The lesion in long bones appeared multicentric, surrounding with a thin rim of bone and calcification. Six cases of Ollier's disease had developed secondary low-grade chondrosarcoma.

CONCLUSIONSLow-grade chondrosarcomatous transformation can occur in dysplastic cartilage of Ollier's disease. The diagnosis of such sarcomatous transformation should be determined by invasion, but not atypicality of the cartilagenous cells. There are certain differences between the secondary chondrosarcoma of Ollier's disease and conventional chondrosarcoma on clinical, radiographical and pathological grounds.

Adolescent ; Adult ; Aged ; Bone Neoplasms ; diagnostic imaging ; etiology ; pathology ; surgery ; Cell Transformation, Neoplastic ; Child ; Chondrosarcoma ; diagnostic imaging ; etiology ; pathology ; surgery ; Diagnosis, Differential ; Enchondromatosis ; complications ; diagnostic imaging ; pathology ; surgery ; Exostoses, Multiple Hereditary ; pathology ; Female ; Humans ; Male ; Middle Aged ; Radiography ; Young Adult

OBJECTIVETo investigate the clinicopathologic, radiological and immunohistochemical characteristics of osteochondroma with malignant transformation.

METHODSThe clinical data, radiological imagings and hematoxylineosin stained histologic sections were reviewed in 463 cases of osteochondroma diagnosed in Shanghai 6th Hospital from 1991 to 2008, including 11 cases with malignant transformation. Immunohistochemical two-step staining was used to detect CK, vimentin, S-100 protein, p53 and c-myc expression in seven cases of osteochondroma with malignant transformation and 10 cases without malignant transformation. The relevant literature was reviewed.

RESULTSAmong the 11 cases with malignant transformation, five were single osteochondroma (5/408, 1.2%), and six were multiple osteochondromas (6/55, 10.9%). The male to female ratio was 10:1. These 11 cases were derived from femur (4 cases), tibia (3 cases), ilium (3 cases), shoulder bone (1 case) and pubis (1 case). There was one case that showed malignant transformation in both the femur and ilium. The mean ages for the malignant and non-malignant cases were 39.8 years and 20.4 years respectively. All the malignant cases showed large sized lesions with prominent calcification in the thick cartilage caps. The malignant component was low grade, peripheral chondrosarcoma (grade I-II). In some areas the tumor cells infiltrated the peripheral soft tissue and bone marrow. Of the seven cases with malignant transformation that had immunohistochemical staining, all were positive for vimentin and S-100 protein; p53 protein was positive in 2 of 7 cases.

CONCLUSIONSMalignant transformation of osteochondroma was usually encountered in multiple lesions. Most patients were more than 30 years old with a long clinical history and with a male predominance. These tumors showed thick cartilage caps with prominent calcification. The lobulated nature of the tumors was evident and they infiltrated the surrounding soft tissue. The sarcomatoid component was peripheral type, well differentiated chondrosarcoma. p53 mutation may explain part of the molecular mechanism in the malignant transformation.

Adult ; Aged ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Cell Transformation, Neoplastic ; pathology ; Chondrosarcoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Exostoses, Multiple Hereditary ; diagnostic imaging ; metabolism ; pathology ; surgery ; Female ; Humans ; Male ; Middle Aged ; Osteochondroma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Radiography ; S100 Proteins ; metabolism ; Tumor Suppressor Protein p53 ; metabolism ; Vimentin ; metabolism ; Young Adult