Updated: 6/22/2021

Tumoral Calcinosis

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  • 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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(OBQ08.174) A 59-year-old dialysis-dependent diabetic caucasian female complains of palpable bump over her right hip. She denies pain in the hip and has not noticed any other bumps on any of her extremities. On physical exam, the bump is nontender and does not limit range of motion. A radiograph is provided in Figure A. What is the most likely diagnosis?

QID: 560
FIGURES:
1

Calcinosis of renal failure

74%

(1740/2353)

2

Synovial chondromatosis

7%

(153/2353)

3

Synovial sarcoma

1%

(26/2353)

4

Parosteal osteosarcoma

6%

(144/2353)

5

Myositis ossificans

12%

(279/2353)

L 2 D

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(OBQ07.29) A 30-year-old African-American female presents to your clinic with right hip swelling. These symptoms have been present for over 3 years, but this is the first time she is seeking treatment. Figures A-E show radiographs, an MRI, and histology. What is the most likely diagnosis?

QID: 690
FIGURES:
1

Osteochondroma

2%

(31/1456)

2

Chondrosarcoma

4%

(52/1456)

3

Dedifferentiated chondrosarcoma

6%

(83/1456)

4

Tumoral calcinosis

71%

(1037/1456)

5

Bizarre Parosteal Osteochondromatous Proliferation (Nora's disease)

17%

(249/1456)

L 3 D

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