Tumoral calcinosis

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.

Epidemiology

The condition predominantly affects young black patients with an equal male to female ratio.

Clinical presentation

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.

Pathology

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 calcium / hydroxyapatite crystals in suspension).

Location

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

• hip 

• elbow

• shoulder

• 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

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

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.

History and etymology

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.

Differential diagnosis

General imaging differential considerations include:

• hyperparathyroidism: most frequently in chronic renal failure

• calcium pyrophosphate deposition disease (CPPD)

• myositis ossificans

• calcinosis circumscripta

• calcinosis universalis

• milk-alkali syndrome

• hypervitaminosis D

• calcinosis of chronic renal failure

• calcific tendinitis

• synovial osteochondromatosis

• synovial sarcoma

• osteosarcoma

• calcific myonecrosis

• tophaceous gout