Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Tumoral calcinosis associated with systemic sclerosis
- Radiologic Findings
- Lung window setting of chest CT scan shows honeycombing change and pulmonary fibrosis in the both lungs.
Mediastinal window setting of the chest CT scan shows multilobulated soft tissue mass with calcification in the left scapulothoracic area and esophageal stenosis.
- Brief Review
- Classic tumoral calcification is lobular, densely calcified masses consisting of pleomorphic calcium phosphate (hydroxyapatite) crystals and confined to the soft tissue, generally at the extensor surface of the joint in the anatomical distribution of a bursa.
Common location is hip, elbow, shoulder, foot and wrist. Smack et al classified tumoral calcinosis into 3 categories: a) Primary normophosphatemic tumoral calcinosis, b) Primary hyperphosphatemic tumoral calcinosis, c) Secondary tumoral calcinosis
Secondary tumoral calcinosis is associated with renal failure, hemodialysis, collagen vascular disease (particularly scleroderma or dermatomyositis), sarcoidosis, pseudoxanthoma elasticum, massive osteolysis and other conditions. Pathogenesis is thought to chronic soft-tissue inflammation and dystrophic calcification, but not clearly known.
Tumoral calcinosis has a typical appearance on radiographs: Usually involving subcutaneous tissues and paraarticular distribution, macroscopic masses of amorphous calcified material and multilobulated mass of several centimeters with amorphous calcific deposit. A cystic component may be present as fluid-sedimentation level or calcium layering known as "sedimetation" sign.
- References
- 1. Kathryn M. et al.,Tumoral Calcinosis: Pearls, Polemics, and Alternative Possibilities, Radiographics 2006; 26: 871-885
2. Smack et al., Proposal for a pathogenesis-based classification of tumoral calcinosis, Int J Dermatol 1996;174:215-222
3. Elias P. et al., Extensive Tumoral Calcinosis in a Patient With Systemic Sclerosis, Am J Orthop. 2010;39(10):E108-110
- Keywords
- Lung, Metabolic and storage lung disesae, Interstitial lung disease,