This site is targeted at medical and radiology professionals, contains user contributed content, and material that may be confusing to a lay audience. Use of this site implies acceptance of our Terms of Use.

Gorham disease

Gorham's disease or vanishing bone disease is a poorly understood rare skeletal condition which manifests with massive progressive osteolysis along with a proliferation of thin walled vascular channels. Other names for this condition include progressive massive osteolysis, Gorham-Stout disease and phantom bone disease10.

The disease starts in one bone but may spread to involve adjacent bony and soft tissue structures.

Epidemiology

Gorham's disease is thought to be non hereditary and there is no recognised gender predilection. It can potentially occur in any age group although most reported cases have been in young adults 2.  

Pathology

The osteolysis is thought to be due an increased number of stimulated osteoclasts 3, which is likely secondary to abundant non-neoplastic vascular and lymphatic proliferation in the affected region 9. The bone is subsequently replaced by variable amounts of fibrous connective tissue that is hypervascular10.

Location

Gorham disease can potentially involve any bone. Reported sites include

  • humerus (first reported case)
  • shoulder girdle
  • pelvis
  • skull 2
  • mandible

Splenic lesions and soft-tissue involvement represent extra-skeletal manifestations 6.

Radiographic features

Plain film and CT
  • intramedullary or subcortical lucent foci may be the earliest manifestation 1
  • this progresses to profound osteolysis with resorption of affected bone and lack of compensatory osteoblastic activity or periosteal reaction
Scintigraphy
  • Tc99m bone scan may initially be positive but later becomes negative with ongoing bone resorption

Etymology

It was first reported by Jackson in 1838, but later defined by Gorham and Stout in 1955.

Differential diagnosis

Imaging differential considerations include:


Related articles

Bone tumours

The differential diagnosis for bone tumours is dependant on the age of the patient, with a very different set of differentials for the paediatric patient.

Updating… Please wait.
Loadinganimation

Alert_accept

Error Unable to process the form. Check for errors and try again.

Alert_accept Thank you for updating your details.