Dr Abdelaziz Elamin, MD, PhD, FRCPCH College of Medicine Sultan Qaboos University TUMORAL CALCINOSIS...
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Transcript of Dr Abdelaziz Elamin, MD, PhD, FRCPCH College of Medicine Sultan Qaboos University TUMORAL CALCINOSIS...
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Dr Abdelaziz Elamin, MD, PhD, Dr Abdelaziz Elamin, MD, PhD, FRCPCHFRCPCH
College of MedicineCollege of MedicineSultan Qaboos UniversitySultan Qaboos University
TUMORAL CALCINOSISTUMORAL CALCINOSIS
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BACKGROUND•Virchow in 1855 used the term Calcinosis Cutis to describe deposition of calcium in the skin & S/C tissues.
•It is classified into 4 major types according to etiology:
Dystrophic
Metastatic
Iatrogenic
Idiopathic
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DYATROPHIC CALCINOSIS
Occurs in the setting of normal calcium & phosphate levels
The primary abnormality is damaged, inflamed, neoplastic or necrotic tissue
Tissue damage may follow mechanical, chemical, or infectious factors
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Causes of Dystrophic Calcinosis
•Localized:•Trauma•Infections•Varicose veins•Pancreatic calcification
•Generalized:•Connective tissue diseases•Inherited disorders•Tumors•S/C fat necrosis of the newborn
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METASTATIC CALCINOSIS
Occurs in the setting of abnormal calcium & phosphate metabolism
Has identifiable underlying cause
Hypercalcemia and or hyperphosphatemia are usually present
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Causes of Metastatic Calcinosis
•Primary or Secondary Hyperparathyroidism
•Paraneoplastic Hypercalcemia
•Destructive Bone Disease
•Milk-Alkali Syndrome
•Hypervitaminosis D
•Sarcoidosis
•Chronic Renal Failure
•Calciphylaxis
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Causes of Iatrogenic Calcinosis
•Parenteral calcium
•Parenteral inorganic phosphate
•Tumor lysis syndrome
•Repeated heel pricks in the newborn
•Prolonged use of calcium- containing electrode paste (EEG, EMG, BAEP)
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IDIOPATHIC CALCINOSIS
No causative factor is identifiable
It occurs in the absence of known tissue injury or systemic metabolic defect
Plasma calcium, phosphate, PTH & alkaline phosphatase levels are usually normal
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Recognized types of idiopathic Calcinosis
Tumoral calcinosis
Subepidermal calcified nodules
Milialike idiopathic calcinosis
Calcinosis of scrotum/ penis/ vulva
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TUMORAL CALCINOSIS
Unknown etiology but an error in renal phosphate regulation is likely
Increased intestinal absorption of phosphate has also been described
More common in Africans & in Arabs
Familial type is recognized with autosomal recessive pattern of inheritance
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TUMORAL CALCINOSIS /2
Early presentation may mimic osteomyelitis
Calcium pyrophosphate crystals has been detected in the medullary canal of long bones
Association with mild iron deficiency anemia has been described
Affects males >females, commonest in the second decade of life, but can affect any age
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TUMORAL CALCINOSIS /3
•The calcified nodules have the following characteristics:
Localized in S/C tissues & muscles
Progressive enlargement
Juxta-articular location
Tendency to recur after surgical removal
Ability to encase adjacent normal structure
Provocation of local inflammatory response
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CLINICAL PRESENTATIONS
•Large painless masses around big joints
•Subcutaneous deposits with ulceration
•Compression of neural structuresCompression of neural structures
•Sinus tract and infectionSinus tract and infection
•Low grade fever & painLow grade fever & pain
• Regional LymphadenopathyRegional Lymphadenopathy
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DIFFERENTIAL DIAGNOSIS
Early presentation constitutes a diagnostic dilemma with several differential diagnoses
When calcified lesions are present, all possible causes of dystrophic & metastatic calcifications should be ruled out
CT & radio-isotope scans are more useful than plain x-ray films in early diagnosis
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INVESTIGATIONSPlasma levels of albumin, ca, ph, ALP & PTH
Urea & electrolytes and full blood count
CT & bone scans, US & plain x-ray films
Level o vitamin D metabolites
24 hour urinary excretion of ca & ph
Arterial blood gases, CK & serum amylase
Biopsy & histopathologic examination is diagnostic
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TREATMENT
MEDICAL
SURGICAL
DIETARY
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MEDICAL CARE
Medical therapy is of limited value & several drugs have been tried with variable benefits.
Aluminum or Magnesium antiacids
Analgesics & NSIAD
Intra-lesional corticosteroids
Warfarin has shown benefit in some patients
Probenecid & Colchicine
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NEW DRUG TRAILS
Calcitonin
Calcium-channel blockers
Sodium diphosphonates
Acetazolamide
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SURGICAL CARE
Indication for surgical removal include: Pain Recurrent infection Ulceration Functional impairment
Surgical trauma may stimulate calcification
Recurrence is common
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DIETARY INTERVENTION
If hyperphosphatemia is present restrict dietary phosphate
Calcium restriction is not generally recommended
A ketogenic diet may be helpful but difficult to maintain
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CONCLUSION
Tumoral calcinosis is a rare disorder of uncertain etiology
Early diagnosis is difficult
Surgical excision is the best available Rx
Medical treatment is of limited value