OIiier disease is a rare nonhereditary disorder characterized by multiple enchondromas in which malignant changes may occur. We report the case of a 15-year-old male with Ollier disease who presented with a large mass in the left upper arm for several months. Radiography revealed hugelytic mass in the proximal half to two-thrids of the left humerus. Bone scan showed irregularly increased tracer uptake in the head to mid shaft of the left humerus that is suggestive of malignant disease. Pathology analysis demonstrated proximal humeral chondrosarcoma, grade 1. In Ollier disease, bone scan may be used for monitoring the lessions with suspicion for malignant transpormation.
Chondrosarcoma ; Enchondromatosis

Adult ; Enchondromatosis ; diagnosis ; diagnostic imaging ; Femur ; diagnostic imaging ; Humans ; Male ; Radiography

The triad of rash, arthritis, and uveitis seems to be characteristic for early-onset childhood sarcoidosis. We describe an interesting case of early-onset childhood sarcoidosis coexisting enchondromatosis, which clinically masquerade as Langerhans cell histiocytosis. A 33 months old girl presented with skin rash, subcutaneous nodules with polyarthritis, and revealed the involvement of lymph nodes as well as spleen during work-up. She also presented with multiple osteolytic lesions which pathologically proven enchondromatosis. Oral prednisone was prescribed at 2 mg/kg/day for 2 months until when subcutaneous nodules and joint swellings almost disappeared, and then slowly tapered over a period of 5 months. We report an unusual case of early-onset childhood sarcoidosis presented with osteolytic bone lesions which were irrelevant to sarcoidosis.
Administration, Oral ; Anti-Inflammatory Agents/therapeutic use ; Arthritis/complications ; Child, Preschool ; Diagnosis, Differential ; Enchondromatosis/*complications/*diagnosis/drug therapy/radiography ; Exanthema/etiology ; Female ; Humans ; Positron-Emission Tomography and Computed Tomography ; Prednisone/therapeutic use ; Sarcoidosis/*complications/*diagnosis/drug therapy/radiography ; Whole Body Imaging

Ameloblastoma ; pathology ; Bone Neoplasms ; classification ; genetics ; pathology ; Chondromatosis ; pathology ; Chondrosarcoma ; classification ; pathology ; Enchondromatosis ; pathology ; Humans ; Osteosarcoma ; classification ; pathology ; Sarcoma, Ewing ; pathology ; Societies, Medical ; United States ; World Health Organization

PURPOSE: We analyzed the oncological outcome and prognostic factor of the chondrosarcoma arising from benign bone tumor due to malignant transformation. MATERIALS AND METHODS: From April 1986 to April 2009, 18 cases were considered eligible. We analyzed retrospectively the patient's characteristics and prognostic factors that affect to the local recurrence and distant metastasis. RESULTS: As classified by primary benign bone tumor, 4 cases were solitary osteochondroma, 11 cases were multiple osteochondromatosis and 3 cases were multiple enchondromatosis. The mean follow-up period was 85 months. The 5-year disease free survival rate of 18 patients was 85.9%. Their overall MSTS score was 25.2 (84%). There were local recurrence in 3 cases and no distant metastasis. We found that tumor location and surgical margin affected to the prognosis significantly. CONCLUSION: In secondary chondrosarcoma patients, the prognosis was good relatively and tumor location and surgical margin are important prognosis factor.
Chondrosarcoma ; Disease-Free Survival ; Enchondromatosis ; Follow-Up Studies ; Humans ; Neoplasm Metastasis ; Osteochondroma ; Osteochondromatosis ; Prognosis ; Recurrence ; Retrospective Studies

PURPOSE: To analyze clinical, radiological and pathological features as well as clinical outcome after surgical treatment of patients with secondary chondrosarcoma arising from osteochondroma(tosis). MATERIALS AND METHODS: We retrospectively reviewed clinical records, radiographs, pathologic slides of 14 patients. Nine patients were male and five were female. The mean age was 34 years. The mean follow-up period was 54 months. RESULTS: All patients had a history of previous mass since childhood or puberty. Preexisted osteochondroma was single in 3 patients and multiple in 10. Remaining 1 patient had multiple osteochondromatosis with enchondromatosis. MRI clearly provided thickness of cartilage cap, which was over 2 cm except in 2 cases. Chondrosarcoma was grade 1 in all except 1 case, which was grade 2. Wide excision was performed in 10 patients, marginal excision in 3 and amputation in 1. Twelve patients were doing very well without evidence of disease. Among 3 patients with marginal excision, 1 patient had local recurrence and 1 patient died of disease. CONCLUSION: Comprehensive understanding of clinical, radiological and pathological features of secondary chondrosarcoma is warranted for accurate diagnosis. The best result can be expected with early recognition of malignant change of osteohcondroma(tosis) and wide excision.
Amputation ; Cartilage ; Chondrosarcoma ; Enchondromatosis ; Female ; Follow-Up Studies ; Humans ; Male ; Osteochondroma ; Osteochondromatosis ; Puberty ; Recurrence ; Retrospective Studies

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

Adult ; Enchondromatosis ; pathology ; Female ; Granulosa Cell Tumor ; pathology ; Humans ; Ovarian Neoplasms ; pathology

PURPOSE: Retrospectively, to evaluate the result of treatment by curettage only, enchondroma of the digits. MATERIALS AND METHODS: Fifty-nine cases in 30 patients (male 11, female 19), from July 1997 to Febuary 2002, were reviewed with a minimum follow up of 1 year. Thirty patients were treated for enchondroma of the digits (including Ollier's disease 9, and Maffucci syndrome 2) by curettage only, and the degree of healing was evaluated radiologically. The average duration of follow up was 28.6 months (12-65 months). RESULTS: The radiologic results were evaluated according to the degree of new bone formation by the criteria suggested by Tordai. Group I achieved 79.7% overall and 89.5% for solitary enchondroma, meaning complete healing with normal cortical thickness and a bone defect of less than 3 mm. The average duration of consolidation was 12.4 months, and 8.1months for solitary enchondorma. No recurrence or other complication occured. CONCLUSIONS: Curettage alone is a good treatment option for enchondroma of the digits as comparing with autogenous bone graft, which has the disadvantages of pain, infection, and immobilization.
Chondroma* ; Curettage* ; Enchondromatosis ; Female ; Follow-Up Studies ; Humans ; Immobilization ; Osteogenesis ; Recurrence ; Retrospective Studies ; Transplants

Maffucci syndrome is a rare congenital non-inherited condition characterized by multiple enchondromas and cutaneous hemangiomas. It is associated with increased risk of malignancy, including chondrosarcomas, and because of generalized mesodermal dysplasia, aneurysms can develop. We present a case of Maffucci syndrome associated with intracranial chondrosarcoma and aneurysm.
Aneurysm* ; Chondroma ; Chondrosarcoma* ; Enchondromatosis ; Hemangioma ; Intracranial Aneurysm ; Mesoderm