ENCHONDROMATOSIS MULTIPLE PDF

Enchondromatosis is a rare heterogeneous skeletal disease characterized by multiple enchondromas, and it includes several different subtypes of which Ollier . Enchondromas are common benign cartilage tumors of bone. They can occur as solitary lesions or as multiple lesions in enchondromatosis. Enchondromatosis, also known as Ollier disease (see Terminology section), is a non-hereditary, sporadic, skeletal disorder characterised by multiple.

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Enchondromatosis is a rare heterogeneous skeletal disease characterized by multiple enchondromas, and it includes several different subtypes of which Ollier disease and Maffucci syndrome are most common.

Enchondromas are benign hyaline cartilage forming tumors in the medulla of metaphyseal bone. Ollier disease also known as dyschondroplasia, multiple cartilaginous enchondromatosis, en- chondromatosis Spranger type I is characterized by multiple enchondromatosis with an asymmetric distribution.

Lesions are usually distributed in the appendicular skeleton the skull enchomdromatosis vertebral bodies are very rarely involved. In particular, enchondromas are most commonly seen in phalanges and metacarpals and rarely in the carpal bones. However, there is a wide variety of presentations in terms of size, number, location, evolution of enchondromas, age of onset and of diagnosis, and requirement for surgery.

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Clinical problems caused by enchondromas include skeletal deformities, limb-length discrepancy, and the potential risk for malignant change to chondrosarcoma. Radiologically, Ollier disease presents with asymmetrical osteolytic lesions with well-defined, sclerotic margins.

The condition in which multiple enchondromatosis is associated with soft tissue hemangiomas is known as Maffucci syndrome. The pathogenesis of enchondromatosis is not clearly understood. The inheritance pattern of Ollier disease is unknown but is thought to not be simply a Mendelian pattern.

Multiple Enchondromatosis: Olliers Disease- A Case Report

The diagnosis is based on clinical and conventional radiological evaluations. Histological analysis has a limited role and is mainly used if malignancy is suspected.

Ollier disease must be differentiated from multiple hereditary exostosis, and radiologically, Ollier disease may mimic osteitis enchondromatoeis cystica. There is no medical treatment for enchondromatosis, but treatment of Ollier disease is usually conservative. Surgery is performed in cases of complications and malignant transformation. Soft tissue hemangiomas Maffucci syndromemultiple enchondromas with skeletal deformities and potential risk for malignant change to chondrosarcoma.

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OMIM Entry – % – ENCHONDROMATOSIS, MULTIPLE, OLLIER TYPE

Multiple enchondromas, manifesting as central end eccentric osteolytic lesions and deformities in the metacarpals and phalanges of the fourth and fifth ray of the right hand.

The enchondromas have increased in size and some are more evidently visible compared to the previous study. This has resulted in deformity of the fourth finger. Int J Clin Exp Pathol ;3: Gene PTHR1 gene, 3p Phenotype Soft tissue hemangiomas Maffucci syndromemultiple enchondromas with miltiple deformities and potential risk for malignant change to chondrosarcoma. Main biochemical alterations Slightly high PTH.

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