Tumoral calcinosis

Tumoral calcinosis is a rare familial condition characterised by painless, periarticular masses. The term should be strictly used to refer to a disease caused by a hereditary metabolic dysfunction of phosphate regulation associated with massive periarticular calcinosis and should not be used to refer to soft-tissue calcification in general.

Epidemiology

The condition predominantly affects young black patients with an equal M:F.

Clinical presentation

Most patients present with lumps adjacent to joints. They are usually painless, but some patients describe pain and tenderness. Involvement of large joints is typical, although the knee is rarely involved. The underlying bone is normal.

Pathology

One-third of cases are familial (autosomal dominant) with abnormal FGF 23 7, and serum calcium is normal (some sources state that hyperphosphataemia is common).

It is characterised by large amorphous calcific densities that surround joints. These are separated into lobules by fibrous septa and may demonstrate fluid/calcium levels (milk of calcium / hydroxyapatite crystals in suspension).

Location

The most common locations are (in descending order of frequency) 2:

  • hip 
  • shoulder
  • elbow
  • foot
  • wrist

Radiographic features

Plain radiograph

Tumoral calcinosis has a typical appearance on plain radiographs with amorphous and multilobulated ("cloud-like") calcification located in a periarticular distribution.

CT

CT better delineates the calcific mass. There is no erosion or osseous destruction by the adjacent soft-tissue masses which is another distinguishing finding of tumoral calcinosis from other pathologies.

MRI

MR imaging with T2-weighted sequences shows inhomogeneous high-signal intensity, even though there is a significant amount of calcification. T1-weighted sequences usually show inhomogeneous lesions with low signal intensity.

Differential diagnosis

General imaging differential considerations include:

Edit Article Share
URL of Article

Article Information

rID: 2226
System: Musculoskeletal
Section: Pathology
Synonyms or Alternate Spellings:
  • Idiopathic tumoural calcinosis
  • Tumoural calcinosis
  • Primary tumoral calcinosis
  • Familial tumoral calcinosis
  • Drag
    Case 1
    Drag here to reorder.
  • Drag
    Case 1
    Drag here to reorder.
  • Drag
    Case 2
    Drag here to reorder.
  • Drag
     Case 3
    Drag here to reorder.
  • 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.