Tumoral calcinosis

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Tumoral calcinosis
autosomal dominant and is not associated with dialysis
. Note the premature arterial calcification which is a clue that this is a renal patient. Vascular calcification contributes to an increase in morbidity.

Tumoral calcinosis is a rare condition in which there is

FGF-23,[2] Klotho (KL),[3] or GALNT3.[4] A zebrafish animal model with reduced GALNT3 expression also showed HFTC-like phenotype,[5]
indicating an evolutionary conserved mechanism that is involved in developing tumoral calcinosis.

Clinical features

The name indicates calcinosis (calcium deposition) which resembles tumor (like a new growth). They are not true neoplasms – they don't have dividing cells. They are just deposition of inorganic calcium with serum exudate. Children and adolescents (6 to 25 years) are the most commonly affected. The symptom that the accumulations cause is not pain but swelling around joints. They have propensity to enlarge progressively and ulcerate the overlying skin and extrude. They are most common around shoulders, hips and elbows. Laboratory evaluation reveal normal serum calcium levels and hyperphosphatemia. Rarely ALP (alkaline phosphatase – an enzyme active at sites of bone formation) may be elevated. Treatment is normalization of serum phosphate levels and resection of lesions. Surgical removal should be complete and if part of it is left, recurrence is likely to occur. Cutting through the excised calcium deposition reveals semifluid calcium suspension in albumin encapsulated by fibrous tissue.

Additional images

Oblique hand radiograph showing tumoral calcinosis
Oblique radiograph of the right hand demonstrating soft tissue calcification, characteristic of dialysis related metastatic calcification.
tumorous calcinosis over a malleolus in ultrasound

References

  1. ^ "Orthobullets". Retrieved 27 October 2014.
  2. PMID 16030159
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External links