OSTEOCHONDROMA

 

OSTEOCHONDROMA

 

DEFINITION:

A benign chondrogenic tumor of bone characterized by a mass and pain.

EPIDEMIOLOGY:

  • incidence: most common benign bone tumor in children
  • age of onset:
    • peak between 10-20 years of age in 80% of cases
  • risk factors:
    • familial - usually sporadic
      • autosomal dominant (in Multiple Exostoses)
      • chrom.#: ?8q23-q24.1
      • gene: ?
    • M = F

PATHOGENESIS:

1. Tumor Origin

1. Major

  • distal femur, proximal tibia, proximal humerus
  • in all 3, the tumor arises from the ends of the long bones at the site of tendon insertion
  • there appears to be a misdirected growth of the physis resulting in the lateral protrusion of portions of the growth plate causing the development of eccentric, cartilage-capped bony prominences
  • the lesion is usually located in the metaphyseal area but with growth, moves away from the physis

2. Minor

  • distal radius, distal tibia and proximal and distal fibula
  • carpal and tarsal bones
  • flat bones (scapula, ilium, rib, skull, sternum)

2. Histologic Type

1. Chondrogenic Cells

  • usually benign and do not metastasize
  • bone arising from cartilage
  • in 0.25%, chondrosarcomatous (malignant) changes can occur in either solitary or multiple osteochrondromas

CLINICAL FEATURES:

1. Primary Tumor

1. Pain

  • tumor is usually not tender but may become so if:
    • following direct trauma to the tumor resulting in an injury to the tumor or a fracture through the stalk of the tumor (hip pain and/or knee pain)
    • the tumor impinges on a nerve
    • the tumor is associated with a bursal sac (exostosis bursata) which is irritated by the movement of adjacent muscles and tendons
    • associated with a malignant transformation

INVESTIGATIONS:

1. Imaging Studies

1. Skeletal X-Ray/CT/MRI

  • 1. Bony Protuberance (Exostosis)
    • juxtaphyseal in location
    • cortex and spongiosa are continuous with that of the affected bone
    • pedunculated: sessile (broad-based or cauliflower forms) or circumferential
    • cartilaginous cap usually not visible unless calcified
    • distinct and well demarcated external surface of the tumor
  • 2. Bone Scan

    • increased uptake of technetium
    • cannot differentiate between a benign or malignant exostosis

    MANAGEMENT:

    1. Surgery

    • excision (recurrences rare)

     

     

    Pediatric Database - OSTEOCHONDROMA

    Pediatric Organization - Pedbase [at] Gmail.com