Enchondromatosis (Subscribe)  

Enchondroma and Enchondromatosis Last Updated: June 10, 2005 Author: Felix S Chew, MD, EdM, MBA, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington Coauthor(s): Catherine Maldjian, MD, Associate Professor, Director, Radiology Research Center, Department of Radiology, Westchester Medical Center Background: Enchondromas are...

"Benign growths of cartilage in the metaphyses of several bones." Enchondromatosis aka/or Maffucci syndrome aka/or Multiple enchondrosis aka/or Ollier's disease may cause or feature - Miscellaneous syndromes Bone lysis Symptoms and Signs Disproportionate short stature Exostoses Hemangioma Subcutaneous nodules X-ray abnormalities Exostoses may be a risk factor for Chondrosarcoma may be associated with Spindle cell hemangioendothelioma

There is expansion and replacement of the bone of the middle metaphysis of the right hand. A smaller lytic lesion is seen in the right 5th metacarpal with some minimal expansion. The cortex of metacarpals is intact. There are defects in the phalanges, particularly right middle and left ring fingers...

Enchondroma with multiple angiomas (Maffucci syndrome) was first reported by Maffucci in 1881 after a 40-year-old woman died from complications following amputation of an arm. The patient had frequent and severe hemorrhage from a vascular tumor for which she was admitted to the hospital. In view of the profuse bleeding,...

Maffucci's syndrome is defined as multiple enchondromatosis with soft tissue and visceral hemangiomas. Patients with Maffucci's syndrome are believed to be at far greater risk for malignant transformation than those with Ollier's disease. Diagnostic Radiology/Musculoskeletal Imaging/Tumors Basic/Maffucci syndrome From Wikibooks, the open-content textbooks collection

* rare
* multiple enchondromata
o 40 - 45% malignant transformation --> chondrosarcoma
* multiple cavernous hemangiomata
o may degenerate --> angiosarcoma

Radiology teaching, the hand in Mafucci syndrome, a single radiology case example.
There is expansion and replacement of the bone of the middle metaphysis of the right hand. A smaller lytic lesion is seen in the right 5th metacarpal with some minimal expansion. The cortex of metacarpals is intact. There...

Illustrated cases of Ollier's Disease (Multiple enchondromatosis)

ENCHONDROMATOSIS, MULTIPLE Alternative titles; symbols OSTEOCHONDROMATOSIS DYSCHONDROPLASIA MAFFUCCI SYNDROME, INCLUDED OLLIER DISEASE, INCLUDED enchondromatosis was found by Hopyan et al. (2002) to be caused by mutation in the PTH/PTHRP type I receptor (PTHR1; 168468.0007). However, Rozeman et al. (2004), in an extensive study of enchondromas and chondrosarcomas from 31 enchondromatosis patients from 3 different...

Multiple Enchodromatosis (Ollier's Disease) Discussion: - originally was described by Ollier in late 1800's; - characterized by multiple enchondroma lesions primarily located w/ in the metaphyseal regions of tubular bones; - usually diagnosed in childhood; - pts w/ Ollier's...

* multiple enchondromata
* hamartomas of cartilage within bone
* may be limited to one extremity or to extremities of one side
* femur + tibia most often involved
* may degenerate --> chondrosarcoma

Objectives
1. Describe the radiographic features of solitary enchondroma
2. Describe the clinical and radiographic features of Ollier's disease
3. Describe orthopaedic problems secondary to Ollier's disease
4. Discuss risk of chondrosarcoma in relation to Ollier's disease

Ollier's Disease is a rare condition in which benign tumors form in developing bones and prevent proper bone growth. It is also known as Multiple Enchondromatosis. The condition was first described by French surgeon Louis Ollier (1830-1899). In individuals with Ollier's disease, benign tumors called enchondromas form in the cartilage cells...