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Updated: Jun 22 2021

Tumoral Calcinosis


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Images calcinosis lateral_moved.jpg
  • summary
    • Tumoral Calcinosis is a rare, hereditary metabolic dysfunction of phosphate regulation associated with development of massive periarticular calcinosis in the extra-capsular soft tissues. Patients most commonly present with palpable lesions around the hip and shoulder.
    • Diagnosis is made radiographically with amorphous, cystic, circular, well-demarcated calcification in a periarticular location.
    • Treatment is observation for asymptomatic lesions. Surgical excision is indicated for symptomatic lesions associated with decreased function. 
  • Epidemiology
    • Demographics
      • more common in females and African-Americans
      • onset in childhood or early adolescence
    • Anatomic location
      • around joints, primarily the hip (trochanteric bursa is most common location) > shoulder
  • Etiology
    • Pathophysiology
      • unknown
        • inborn abnormality of phosphorus metabolism
          • FGF-23 defect leading to familial hyperphosphatemic tumoral calcinosis
  • Presentation
    • Symptoms
      • mass or swelling typically around joints
      • pain secondary to compression of normal surrounding structures
    • Physical exam
      • inspection
        • palpable mass around joint
  • Imaging
    • Radiographs
      • amorphous, cystic, lobular (circular or oval) well-demarcated calcification in periarticular location
      • direct involvement of the bones or joints is rare
    • CT
      • findings
        • may demonstrate fluid-fluid levels within some of these masses
          • calcium layering ("sedimentation sign")
        • or may have homogenous appearance
          • reduced metabolic activity, lower likelihood of growth
    • MRI
      • findings
        • diffuse, low signal intensity
        • bright, nodular pattern with alternating areas of high signal intensity and signal void
        • may demonstrate fluid-fluid levels (as with CT)
    • Bone scan
      • radionuclide bone scan may reveal intense uptake in the calcific masses
  • Studies
    • Labs
      • normal or slightly elevated renal and parathyroid function
      • normal or slightly elevated serum calcium, phosphorus, uric acid, and alkaline phosphatase
    • Histology
      • lobulated soft tissue masses with well-defined capsules and thick septae
      • masses are filled with calcareous material (calcium phosphate, calcium carbonate and calcium hydroxyapatite) and fluid
  • Differentials
    • Calcinosis of renal failure (secondary tumoral calcinosis)
      • approximately 1% prevalence of periarticular masses
      • may occur independent of concomitant hyperparathyroidism
      • no radiologic/histologic differences with primary tumoral calcinosis
        • diagnosis is based on vitamin D levels, GFR, hyperphosphatemia, and history of chronic renal failure/long term dialysis
    • Calcific tendinitis
      • shoulder > hip > elbow > wrist > knee
      • occurs within tendon
      • no sedimentation of calcium (makes it different from tumoral calcinosis)
    • Synovial osteochondromatosis
      • occurs within joint/tendon sheath
      • "rings and arcs" and intra-articular location (makes it different from tumoral calcinosis)
    • Myositis ossificans and heterotopic ossification
      • not lobular
      • evolves rapidly from faint calcification to organized cartilage and bone in 6 weeks
      • late lesions of HO have well developed cortex and medullary cavity
    • Gout
      • may have hyperuricemia
      • focal erosions of underlying bone
  • Treatment
    • Nonoperative
      • observation
        • indications
          • treatment of choice for non-symptomatic lesions
    • Operative
      • complete surgical excision
        • indications
          • treatment of choice for symptomatic lesions
        • technique
          • complete surgical excision is required to decrease the rate of local recurrence
        • results
          • risk of recurrence
            • if lesion is poorly circumscribed
            • if excision is performed while lesion is actively growing
  • Complications
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