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

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

Maffucci's syndrome is a congenital, non-hereditary mesodermal d splasia manifested by multiple enchondromas and hemangiomas. Several case reports and reviews indicate that there is a tendency toward malignant transformation of the enchondroma. The incidence of malignancy may be as high as 30%. We report a case of 10-year-old female with multiple hemangiomas on the face, palms, and soles and skeletal chondromatoses of the multiple bones.
Child ; Chondroma ; Chondromatosis ; Enchondromatosis ; Female ; Hemangioma ; Humans ; Incidence ; Mesoderm

A case of Maffucci's syndrome is reported in a 6-year-old girl. She had multiple enchondromas of the long bones and the phalangeal bones with hemangiomatous skin lesions. The hemangiomas were the capillary type and were not anatomically related to the bone lesions.
Capillaries ; Child ; Chondroma ; Enchondromatosis ; Female ; Hemangioma ; Hemangioma, Capillary ; Humans ; Skin

Maffucci's syndrome is a very rare, congenital and non-hereditary mesodermal dysplasia manifested by multiple enchondromas and soft tissue hemangiomas. Since Maffucci had reported this sysdrome in 1881, there have been more than 100 cases reported, and also there has been reported that Maffuddi's syndrome has various interstitial tumor. However there hasn't been any report about Maffucci's syndrome with testicular teratoma. Here we report in this paper that the patient was diagnosed as Maffucci's syndrome of enchondroma and liver hamangioma and also had testicular teratoma. He was admitted for the evaluation of gynecomatia and diagnosed as enchondroma by bone biopsy of the right rib and tibia. Liver hemangioma was also found through abdominal ultrasonogram, CT scan and liver biopsy. And the testicular teratoma was confirmed through testicular biopsy. He is finally diagnosed as Maffucci's syndrome with testicular teratoma and literatures were reviewed.
Biopsy ; Chondroma ; Enchondromatosis ; Hemangioma ; Humans ; Liver ; Mesoderm ; Ribs ; Teratoma* ; Tibia ; Tomography, X-Ray Computed ; Ultrasonography

Maffuccis syndrome was first reported by Maffucci, in 1881. This syndrome is manifested by multiple enchondromas and hemangiomas, characterized by non-hereditary mesenchymal neoplasia that has a propensity for malignant transformation. Involvement of long bones, particularly the bones of the hands and feet, is most common. The most important complication of this syndrome is the high frequency of chondr osarcomatous metaplasia. We report a case of Maffuccis syndrome in a 56-year-old woman with multiple hemangioma on both the hands and forearms, left foot and enchondromas of multiple bones. The disease started at the age of 8 and was associated with deformity, but malignant transformation was not found.
Chondroma ; Congenital Abnormalities ; Enchondromatosis ; Female ; Foot ; Forearm ; Hand ; Hemangioma ; Humans ; Metaplasia ; Middle Aged

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

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

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