Table of Contents
What is Ollier disease?
Ollier disease is a rare skeletal disorder characterized by abnormal bone development (skeletal dysplasia). While this disorder may be present at birth (congenital), it may not become apparent until early childhood when symptoms such as deformities or improper limb growth are more obvious.
What is multiple Enchondromatosis?
Multiple enchondromatosis (Ollier disease) is a rare developmental abnormality characterized by the presence of enchondromas in the metaphyses and diaphyses of multiple bones. The disease appears in early childhood and is neither hereditary nor familial and is considered to be a dysplasia.
What is Ollier’s and Maffucci syndrome?
Main. Ollier disease (enchondromatosis) and Maffucci syndrome are rare, classically nonfamilial conditions that are characterized by multiple intramedullary (central) cartilaginous tumors. In Maffucci syndrome, tumors also occur as soft tissue hemangiomas, which are absent in Ollier disease.
What is the meaning of Enchondromatosis?
Listen to pronunciation. (en-kon-DROH-muh-TOH-sis) A rare disorder that causes benign (not cancer) growths of cartilage in the bones. These growths usually occur in the bones of the hands and feet, but they may also occur in the skull, ribs, and spine.
How is Maffucci syndrome diagnosed?
The diagnosis of Maffucci syndrome is made by a detailed history, thorough physical examination and radiologic assessment. Surgical removal and microscopic study of the skeletal lesions confirm the presence of enchondroma and distinguish the tumor from chondrosarcoma.
How many people have Maffucci syndrome?
Maffucci syndrome is very rare. Since it was first described in 1881, fewer than 200 cases have been reported worldwide.
Can chondroblastoma become malignant?
Aggressiveness in chondroblastoma can be subdivided into three types; malignant chondroblastoma, benign chondroblastoma with lung metastasis and benign chondroblastoma with subsequent development of high-grade malignancy. Subsequent development of malignancy is common in post-irradiation cases.
What is Ollier’s disease?
Background: Ollier’s disease is a non-hereditary, benign bone tumor which is usually characterized by presence of multiple radiolucent lesions (enchondromas) in the metaphysis of long bones with unilateral predominance. The disease is a rare clinical entity with 1/100000 occurrence in early childhood.
What is the difference between Ollier disease and enchondromatosis?
Some authors make a distinction between Ollier disease and enchondromatosis on the basis of distribution. In the original description of Ollier disease, the enchondromas were predominantly confined to one side and limited to the limbs.
What is Ollier disease (Spranger type 1)?
Ollier Disease is also known as Spranger type I, based on the classification of multiple enchondromatosis (Kumar, 2015). Enchondromas are benign, slow-growing hyaline cartilage tumors that arise from the medullary canal (Lubahn, 2016). Ollier Disease is found in approximately 1 in 100,000 people.
What is the difference between Ollier disease and multiple hereditary exostosis?
The key distinguishing factor between Ollier disease and multiple hereditary exostosis (MHE) is the location of the lesions. In Ollier disease the enchondromas typically originate from the intramedullary canal but in MHE, osteochondromas are found at the cortices (Kumar, 2015).