• The Journal of the Korean bone and joint tumor society
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• v.11 no.2
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• pp.208-212
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• 2005

Maffucci syndrome was first reported by Maffucci, an Italian, in 1881. This rare, nonhereditary syndrome is characterized by multiple enchondromas combined with hemangiomas and less commonly lymphangiomas. Maffucci syndrome is sometimes confused with Ollier disease, which was described in 1899 and consists of multiple enchondromas without hemangiomas. Although the prevalence of malignant change in skeletal lesions in Maffucci syndrome and Ollier disease is quite similar, it is important to differentiate between these two conditions because of the greater risk of developing nonmusculoskeletal malignancies in the former. We experienced a case of this rare syndrome and because of it's rarity, we report with review of literature.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup1
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• pp.133-136
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• 2001

Maffucci's syndrome is generally characterized by chondromatosis with vascular lesions of mesodermal origin, however, intracranial chondroma is rare. We present a case of Maffucci syndrome with paracellar chondroma and the review of literatures.

• The Journal of the Korean bone and joint tumor society
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• v.12 no.1
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• pp.71-77
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• 2006

Chondrosarcoma is one of the most common types of primary bone sarcoma. With the exception of the mesenchymal subtype, chondrosarcomas are usually low-grade lesions and rarely show multicentricity or distant metastasis. Only rare cases of multicentric chondrosarcomas have been reported in association with Ollier's disease and Maffucci's syndrome. To our knowledge, no report has been issued of a synchronous multicentric chondrosarcoma occurrence across a joint. We experienced a 30-year-old man with a synchronous monomelic juxta-articular multicentric chondrosarcoma across a shoulder joint in the absence of pulmonary and visceral metastases. He was treated by curettage and cement filling with allograft in the acromion and wedge resection with cement filling in the proximal humerus. At the 18-month follow-up, there was no evidence of recurrence, and the patient had full range of motion without pain.

• The Journal of the Korean bone and joint tumor society
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• v.3 no.2
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• pp.112-118
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• 1997

Multicentric chondrosarcoma other than the mesenchymal subtype is rare separate entity. We experienced a case with nonmonomelic synchronous multicentric chondrosarcoma without any preexisting lesions of Oilier's disease or Maffucci's syndrome. To our knowledge, there was no report of synchronous nonmonomelic multicentric chondrosarcoma. A thirty-three year old man had right distal thigh pain of one and half year. Bone scan showed hot lesions on medial condyle of right femur and shaft of left femur. Plain X-ray showed osteolytic lesion on right femur and slight cortical thickening and calcific lesion was observed on left femoral shaft. Curettage and bone cement filling was done on both lesions. The pathology reports were grade I chondrosarcoma on both side of femur. At one month from operation, pathologic fracture of left femur occurred on bone cement-host bone junction. Conservative treatment and radiotherapy of 60Gy was done. At 8 months from operation, nonunion was evident. Segmental resection of left femur with contralateral fibula graft and second look operation on right condyle lesion were done. At 6 months from revision, fracture occurred at host-graft bone junction. We removed previous hardware and applied long DCP and massive autogenous bone graft. Afterwards, the patient looks good and union was progressing. But at 4 years from last operation, hypertrophic nonunion occurred. Another revision was done with condylar plate and bone graft and now he is well without any sign of local recurrence or metastasis.