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
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
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
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
METASTATIC CALCINOSIS
Occurs in the setting of abnormal calcium & phosphate metabolism
Has identifiable underlying cause
Hypercalcemia and or hyperphosphatemia are usually present
Causes of Metastatic Calcinosis
•Primary or Secondary Hyperparathyroidism
•Paraneoplastic Hypercalcemia
•Destructive Bone Disease
•Milk-Alkali Syndrome
•Hypervitaminosis D
•Sarcoidosis
•Chronic Renal Failure
•Calciphylaxis
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)
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
Recognized types of idiopathic Calcinosis
Tumoral calcinosis
Subepidermal calcified nodules
Milialike idiopathic calcinosis
Calcinosis of scrotum/ penis/ vulva
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
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
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
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
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
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
TREATMENT
MEDICAL
SURGICAL
DIETARY
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
NEW DRUG TRAILS
Calcitonin
Calcium-channel blockers
Sodium diphosphonates
Acetazolamide
SURGICAL CARE
Indication for surgical removal include: Pain Recurrent infection Ulceration Functional impairment
Surgical trauma may stimulate calcification
Recurrence is common
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
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