Enchondromatosis, Multiple, Ollier Type

Description

Enchondromas are common benign cartilage tumors of bone. They can occur as solitary lesions or as multiple lesions in enchondromatosis. When hemangiomata are associated, the condition is known as Maffucci syndrome (OMIM ). Clinical problems caused by enchondromas include skeletal deformity and the potential for malignant change to osteosarcoma (Schwartz et al., 1987). Classification of the EnchondromatosesIn their classification of the enchondromatoses, Spranger et al. (1978) called Ollier disease and Maffucci syndrome types I and II enchondromatosis, respectively; metachondromatosis (OMIM ), type III; spondyloenchondrodysplasia (OMIM ), type IV; enchondromatosis with irregular vertebral lesions, type V; and generalized enchondromatosis, type VI. Halal and Azouz (1991) added 3 tentative categories to the 6 in the classification of Spranger et al. (1978).Pansuriya et al. (2010) suggested a new classification of enchondromatosis (multiple enchondromas).

Clinical Features

Phenotypes and symptoms related to Enchondromatosis, Multiple, Ollier Type

  • Seizures
  • Neoplasm
  • Platyspondyly
  • Hemangioma
  • Hemiplegia
  • Osteosarcoma
  • Enlarged joints
  • Chondrosarcoma
  • Multiple enchondromatosis
  • Abnormality of long bone morphology

Incidence and onset information

— Currently we don't have prevalence information about this disease (Not enough data available about incidence and published cases.)
No data available about the known clinical features onset.

Alternative names

Enchondromatosis, Multiple, Ollier Type Is also known as dyschondroplasia, ollier disease, osteochondromatosis.

Researches and researchers

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Sources and references

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