Presentation
Withheld.
Patient Data
A well-defined lytic lesion in the mid to distal aspect of the proximal phalanx of the index finger. No cortical breach and no periosteal reaction. No fracture can be seen. No other lesions. No soft tissue calcification.
Case Discussion
Enchondroma is a benign cartilaginous neoplasm in the medullary cavity. Most enchondromas are
asymptomatic but may present as pathological fractures. Peak age is 10-30 years.
Location:
tubular bones (hand, foot): 50%
femur, tibia, humerus
Radiographic features:
lytic lesion in the bones of the hand or foot
chondroid calcifications: rings and arcs pattern ("O" and "C")
scalloped endosteum
expansion of cortex but no cortical breakthrough unless fractured
no periosteal reaction or soft tissue mass
Pearls:
in the absence of a fracture, a painful enchondroma is considered malignant until proven otherwise
malignant transformation occurs but is rare
Enchondromatosis (Ollier disease)
Ollier disease is a nonhereditary abnormality in which multiple enchondromas are present. Many lesions become stable at puberty. Risk of malignant transformation to chondrosarcoma is 25%.
Radiographic features:
multiple radiolucent expansile masses in hand and feet
hand and foot deformity
tendency for unilaterality
Maffucci syndrome
Maffucci syndrome comprises enchondromatosis and multiple soft tissue hemangiomas. Unilateral involvement of hands and feet. Malignant transformation is much more common than in Ollier's disease.