Tumoral calcinosis

Last revised by Sonam Vadera on 14 Apr 2022

Tumoral calcinosis, also known as Teutschlaender disease, is a rare familial condition characterized 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.

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

Most patients present with lumps adjacent to joints. They are usually painless, but some patients describe pain and tenderness. Symptoms may arise secondary to mass effect on adjacent structures. On occasion, the overlying skin may ulcerate, leading to exudation of a chalky fluid. Involvement of large joints is typical, although the knee is rarely involved. The underlying bone is normal.

One-third of cases are familial (autosomal dominant) with abnormal FGF23 protein 7, and serum calcium is normal (some sources state that hyperphosphatemia is common).

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

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

  • hip 
  • elbow
  • shoulder
  • foot
  • wrist

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

  • 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
  • it may show cystic appearance with multiple fluid-calcium levels caused by calcium layering (sedimentation sign)

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.

Tumoral calcinosis was first described by Giard in 1898, followed by a report by Duret in 1899 2. From 1935 onwards, Otto Richard Teutschlaender​ (1874-1950), a German pathologist, studied this condition and described it in several publications 8,9; hence its eponym which is found widely in the European - but unknown in the American - literature. In 1943, the Cuban pathologist Alberto Inclan and colleagues, unaware of the European discoveries, described three cases and introduced the term tumoral calcinosis for the entity 2,10.

General imaging differential considerations include:

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