Enchondroma

Case contributed by Dr Hani Makky Al Salam

Presentation

Withheld.

Patient Data

Age: Child
x_ray

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 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.

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