Enchondromas: Common benign metaphyseal cartilage tumors
M/C tumour of hands & feet
Classification
Ollier disease (M/C subtype)
Also known as dyschondroplasia, multiple cartilaginous enchondromatosis, enchondromatosis Spranger type I is defined by the presence of multiple enchondromas with asymmetric distribution
Maffucci syndrome:
Also known as dyschondro-dysplasia with haemangiomas, enchondromatosis with multiple cavernous haemangiomas, Kast syndrome, haemangiomatosis chondrodystrophica, enchondromatosis Spranger type II is a non-hereditary enchondromatosis and characterized by the presence of multiple enchondromas combined with multiple haemangiomas of soft tissue or, less commonly, lymphangiomas
Other subtypes (rare):
Metachondromatosis
Genochondromatosis
Spondyloenchondrodysplasia
Dysspondyloenchondromatosis
Cheirospondyloenchondromatosis
Clinical features
Ollier disease:
Clinical manifestations in Ollier disease often appear in the first decade of life and usually start with the appearance of palpable bony masses on a finger or a toe, an asymetric shortening of an extremity with limping, osseous deformities associated or not with pathologic fractures.
Short tubular bone of hand (M/C site)
Others: Femur, tibia, fibula, humerus, radius, and ulna
Skeletal abnormalities:
Generally first observed with angular deformity or growth discrepancy during childhood which peak before 10 years of age.
Bending, shortening, pathological fracture, and asymmetric deformity
Complications during adulthood:
Arthritis
Deformity
Malignant transformation: Chondrosarcoma
Diagnosis
Diagnosis depends on clinical, radiographic and pathologic findings
Radiogrpahy:
Multiple, radiolucent, homogenous lesions with an oval or elongated shape and well defined slightly thickened bony margin. The lesions and long bone axis run parallel
Frequently assembled as clusters, thus resulting in metaphyseal widening
Notch-like image: Enchondromas localized at the bone border
Magnetic resonance (MR) studies:
Lobulated lesions with intermediate signal intensity on T1-weighted images
Predominantly high signal intensity on T2-weighted sequences
Bone biopsy:
Low cellularity with an abundant hyaline cartilage matrix sometimes with extensive calcifications
Management
There is no definitive medical treatment.
Surgical management:
Indicated in case of complications (pathological fractures, growth defect, malignant transformation).