Fact-sheet: Tumoral calcinosis
Updated on 09/30/2019 at 9:53 AM
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Definition
Tumoral calcinosis or pseudotumoral calcinosis:
Pathophysiology: deposits of calcium phosphate crystals (apatite) in the periarticular soft tissues, forming large calcified masses.
Contributing role of articular microtrauma and hyperphosphatemia
Etiologies:
- Chronic dialysis patients (0.5 to 7%) with a highly variable delay
- Vitamin D intoxication
- Scleroderma
- Dermatopolymyositis
- Sarcoidosis
- Idiopathic (Teutschländer's tumoral calcinosis, an autosomal dominant disease)
Location on the extensor/friction surface of large joints:
- Primarily the shoulder
- Hip/elbow/knee/hand
- Potentially any peripheral joint
- Some atypical locations (scapula, cervical spine)
Clinical features
Periarticular swelling of gradual onset
Limitation of joint mobility
Adjacent nerve or vascular compression
Local inflammation if dissolution of the calcium deposits occurs, potentially mimicking septic arthritis
Possible cutaneous fistulization
Laboratory findings
Unremarkable, possible inflammatory syndrome in case of dissolution of the calcium deposits
Radiography
Multilocular para-articular lesion with juxtaposition of well-circumscribed "clusters" of dense, amorphous calcifications
Ultrasound
Variable sonographic appearance depending on the calcium density: attenuating hyperechoic mass or presence of cavities with fluid-fluid levels
CT
As on radiographs, a multilocular para-articular lesion with juxtaposition of well-circumscribed "clusters" of dense, amorphous calcifications
Loculated areas with sedimentation (fluid-fluid level) may be seen, reflecting sedimentation of calcium crystals in a serous supernatant
Possible erosion of the adjacent bone cortex due to granulomatous reaction.
Defines the local extent and relationship with adjacent structures
MRI
The calcified masses are well-defined, with multiple loculations, hypointense on T1, and variable signal on T2 depending on the amount of calcium and serous supernatant (fluid-fluid level)
Nuclear medicine
Early detection of periarticular calcifications due to bisphosphonate uptake
Hypermetabolism if local inflammation from resorption on 18FDG PET
Management
Several options depending on symptoms:
- Surgical resection, particularly in case of compression
- Medical treatment (phosphate binders)
- Treatment of hyperparathyroidism or renal failure
Differential diagnosis
Osteoblastic metastases to bone
Primary malignant bone or soft-tissue tumor