Tumoral calcinosis
Updates to Article Attributes
Tumoral calcinosis is a rare familial condition characterized by painless, peri-articular 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 predominately affects young black patients with an equal male to female ratio. A third of cases are familial (autosomal dominant) with abnormal FGF 237, 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).
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.
Radiographic features
Conventional radiography
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 adjacent soft-tissue masses which is another distinguishing finding of tumoral calcinosis from other pathologies.
MRI
MR imaging with T2-weighted sequences generally shows inhomogeneous high-signal intensity even though there is a large amount of calcification. T1-weighted sequences usually show inhomogeneous lesions with low signal intensity.
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 tendonitis
- synovial osteochondromatosis
- synovial sarcoma
- osteosarcoma
- calcific myonecrosis
- tophaceous gout
-<p><strong>Tumoral calcinosis</strong> is a rare familial condition characterized by painless, <a href="/articles/peri-articular-masses">peri-articular masses</a>. 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 <a href="/articles/soft-tissue-calcification-1">soft-tissue calcification</a> in general.</p><p>The condition predominately affects young black patients with an equal male to female ratio. A third of cases are familial (autosomal dominant), and serum calcium is normal (some sources state that <a href="/articles/hyperphosphataemia">hyperphosphataemia</a> 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).</p><h4>Clinical presentation</h4><p>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.</p><h4>Radiographic features</h4><h5>Conventional radiography</h5><p>Tumoral calcinosis has a typical appearance on plain radiographs with amorphous and multilobulated ("cloud-like") calcification located in a periarticular distribution.</p><h5>CT</h5><p>CT better delineates the calcific mass. There is no erosion or osseous destruction by adjacent soft-tissue masses which is another distinguishing finding of tumoral calcinosis from other pathologies.</p><h5>MRI</h5><p>MR imaging with T2-weighted sequences generally shows inhomogeneous high-signal intensity even though there is a large amount of calcification. T1-weighted sequences usually show inhomogeneous lesions with low signal intensity.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include</p><ul>- +<p><strong>Tumoral calcinosis</strong> is a rare familial condition characterized by painless, <a href="/articles/peri-articular-masses">peri-articular masses</a>. 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 <a href="/articles/soft-tissue-calcification-1">soft-tissue calcification</a> in general.</p><p>The condition predominately affects young black patients with an equal male to female ratio. A third of cases are familial (autosomal dominant) with abnormal FGF 23<sup>7</sup>, and serum calcium is normal (some sources state that <a href="/articles/hyperphosphataemia">hyperphosphataemia</a> 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).</p><h4>Clinical presentation</h4><p>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.</p><h4>Radiographic features</h4><h5>Conventional radiography</h5><p>Tumoral calcinosis has a typical appearance on plain radiographs with amorphous and multilobulated ("cloud-like") calcification located in a periarticular distribution.</p><h5>CT</h5><p>CT better delineates the calcific mass. There is no erosion or osseous destruction by adjacent soft-tissue masses which is another distinguishing finding of tumoral calcinosis from other pathologies.</p><h5>MRI</h5><p>MR imaging with T2-weighted sequences generally shows inhomogeneous high-signal intensity even though there is a large amount of calcification. T1-weighted sequences usually show inhomogeneous lesions with low signal intensity.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include</p><ul>
References changed:
- 7. Larsson T, Davis SI, Garringer HJ et-al. Fibroblast growth factor-23 mutants causing familial tumoral calcinosis are differentially processed. Endocrinology. 2005;146 (9): 3883-91. <a href="http://dx.doi.org/10.1210/en.2005-0431">doi:10.1210/en.2005-0431</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/15961556">Pubmed citation</a><span class="auto"></span>
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