Tumoral Calcinosis

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Topic updated on 03/10/13 8:09am
Introduction
  • Rare and poorly understood entity characterized by calcium salt deposits in the extra-capsular soft tissues
  • Epidemiology
    • demographics
      • more common in females and African-Americans
      • onset usually in childhood or early adolescence
    • location
      • typically occurs around joints, primarily the hip and shoulder 
  • Pathophysiology
    • unknown
      • there is evidence suggesting an inborn abnormality of phosphorus metabolism
  • Associated conditions
    • calcinosis of renal failure  
      • radiographically similar appearance to tumoral calcinosis, but not as strongly associated with hip and shoulder distribution
      • renal etiology differentiates it from tumoral calcinosis
      • associated with hyperphosphatemia, chronic renal failure, and long term dialysis
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
    • circular or oval, well-demarcated masses of calcium around joints 
    • direct involvement of the bones or joints is rare
  • CT
    • may demonstrate fluid-fluid levels within some of these masses
  • MRI
    • may demonstrate fluid-fluid levels within some of these masses
  • 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
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 excisionis  required to decrease the rate of local recurrence
IBank
 
Location
Xray
Xray
CT
B.Scan
MRI
MRI
Histo(1)
Case A femur              
Case D femur              
(1) - histology does not always correspond to clinical case 


 

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Qbank (2 Questions)

TAG
(OBQ08.174) A 59-year-old dialysis-dependent diabetic 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? Topic Review Topic
FIGURES: A          

1. Calcinosis of renal failure
2. Synovial chondromatosis
3. Synovial sarcoma
4. Parosteal osteosarcoma
5. Myositis ossificans

PREFERRED RESPONSE ▶
TAG
(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? Topic Review Topic
FIGURES: A   B   C   D   E  

1. Osteochondroma
2. Chondrosarcoma
3. Dedifferentiated chondrosarcoma
4. Tumoral calcinosis
5. Bizarre Parosteal Osteochondromatous Proliferation (Nora's disease)

PREFERRED RESPONSE ▶




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