Cases reported "Enchondromatosis"

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1/5. Malignant progression in multiple enchondromatosis (Ollier's disease): an autopsy-based molecular genetic study.

    Multiple enchondromatosis (Ollier's disease) is a nonhereditary disease characterized by multiple central (medullary) cartilaginous bone tumors of unknown pathogenesis. It usually involves the extremities with a unilateral predominance, and sarcomatous transformation may occur. We report an autopsy-based genetic study of a 34-year-old man presenting in early adolescence with multiple enchondromas of the extremities, predominantly left-sided, compatible with Ollier's disease. Twelve years after presentation, malignant transformation to a high grade chondrosarcoma occurred in a tibial enchondroma. The patient died after widespread metastatic disease. loss of heterozygosity (LOH), in the tibial chondrosarcoma and its metastases, was identified exclusively on chromosome bands 13q14 and 9p21, while being absent in the femoral enchondroma analyzed. Similarly, p53 overexpression was identified immunohistochemically in the tibial chondrosarcoma and its metastases, while being absent in the femoral enchondroma; LOH at 17p13 however, was not demonstrable. It is hypothesized that inactivation of putative tumor suppressor genes at 9p21 and 13q14, and overexpression of p53, identified in the chondrosarcoma and its metastases, but absent in enchondroma, may be related to sarcomatous transformation in Ollier's disease.
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keywords = tibia
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2/5. Bilateral multiple malignant transformation of Ollier's disease.

    We treated a 26-year-old man with a 19-year history of Ollier's disease. Secondary chondrosarcomas developed metachronously at four separate locations: both femora, left proximal tibia and fibular head. All four lesions were surgically excised, and each specimen was histologically identified as grade 1 or 2 chondrosarcoma. Clinical follow-up for 20 years beginning at the time of first tumor surgery has shown no evidence of local recurrence or metastasis. This is the first report of multiple bilateral metachronous malignant transformation of multiple chondromatoses in a patient with Ollier's disease.
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keywords = tibia
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3/5. The management of leg-length discrepancy in Ollier's disease with a fully implantable lengthening nail.

    Ollier's disease is characterised by severe deformity of the extremities and retarded growth because of multiple enchondromas. For correction of deformity, the Ilizarov method has been used although it has many complications. A 17-year-old boy with Ollier's disease had a limb-length discrepancy of 17.4 cm, with a valgus deformity of the right knee and recurvatum of the femur of 23 degrees . He had undergone three unsuccessful attempts to correct the deformities by using external fixators. We used a fully implantable, motorised, lengthening and correction nail (Fitbone) to achieve full correction of all the deformities without complications. We decided to carry out the procedure in three stages. First, we lengthened the femur by 3.6 cm and the tibia by 4 cm. We then exchanged the femoral nail for a longer implant and achieved a further 6 cm of length. This reduced the shortening to 3.8 cm. When the boy has finished secondary school we will adjust the remaining discrepancy.
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keywords = tibia
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4/5. Extensive limb lengthening in Ollier's disease: 25-year follow-up.

    A case of extensive lower limb lengthening (32 cm) in a 14-year-old male patient with Ollier's disease is reported. A varus deformity of the femur and a valgus deformity of the tibia were evident. The femur was successfully lengthened 22 cm by metaphyseal distraction, and the tibia was lengthened 10 cm by two-stage distraction-compression method with a cylindrical bone allograft. Ilizarov's distraction device was used. Radiologically, a good bone regenerate was formed. Host bone has incorporated (like sarcophagi) the allograft of tibia. No evidence of vascular or neural disturbances was found. The lengthening indices were counted for femur 22.5 days per centimeter and for tibia 21 days per centimeter, altogether 15.5 days per centimeter. bone lengthening was performed through the Ollier's disease foci. Fine needle biopsy investigation showed that most embryonic cartilage cells had been replaced with bone tissue. After five years and a 25-year follow-up the patient was satisfied with the result. The function of the knee joint was limited, but the limb was fully weight-bearing. Signs of knee osteoarthritis were found.
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keywords = tibia
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5/5. A 37-year follow-up evaluation of multiple-stage femur and tibia lengthening in dyschondroplasia (enchondromatosis) with a net gain of 23.3 centimeters.

    This case report presents the rare combination of circumstances of a child with (a) dyschondroplasia (enchondromatosis) limited to all the major epiphyseal plates of one lower extremity, (b) unusual capacity to cooperate, and (c) parents who refused permission to treat the limb by amputation but allowed the child to participate in a long-term clinical experiment. In dyschondroplasia (Ollier's disease), the long bones grow in diameter but not in length. In this condition, it was possible (by fasciotomy, osteotomy, and interposition of wedge blocks or hemicylindrical bone implants (stabilized by an intramedullary rod) to lengthen the leg 23.3 cm in ten stages, during the period between five and 14 years of age. With the additional procedure of epiphyseal arrest of the contralateral distal end of the femur at age 11, the eventual correction of discrepancy in leg length was 25.6 cm. Intraoperative and postoperative skin temperature recordings, toe plethysmography, and electromyography show that there were no deleterious changes in the circulatory or neurological functions of the extremity.
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ranking = 1.3333333333333
keywords = tibia
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