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Dr Abdelaziz Elamin, MD, PhD, FRCPCH College of Medicine Sultan Qaboos University

TUMORAL CALCINOSIS. Dr Abdelaziz Elamin, MD, PhD, FRCPCH College of Medicine Sultan Qaboos University. 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:

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Dr Abdelaziz Elamin, MD, PhD, FRCPCH College of Medicine Sultan Qaboos University

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  1. TUMORAL CALCINOSIS Dr Abdelaziz Elamin, MD, PhD, FRCPCH College of MedicineSultan Qaboos University

  2. 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

  3. 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

  4. 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

  5. METASTATIC CALCINOSIS • Occurs in the setting of abnormal calcium & phosphate metabolism • Has identifiable underlying cause • Hypercalcemia and or hyperphosphatemia are usually present

  6. Causes of Metastatic Calcinosis • Primary or Secondary Hyperparathyroidism • Paraneoplastic Hypercalcemia • Destructive Bone Disease • Milk-Alkali Syndrome • Hypervitaminosis D • Sarcoidosis • Chronic Renal Failure • Calciphylaxis

  7. 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)

  8. 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

  9. Recognized types of idiopathic Calcinosis • Tumoral calcinosis • Subepidermal calcified nodules • Milialike idiopathic calcinosis • Calcinosis of scrotum/ penis/ vulva

  10. 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

  11. 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

  12. 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

  13. CLINICAL PRESENTATIONS • Large painless masses around big joints • Subcutaneous deposits with ulceration • Compression of neural structures • Sinus tract and infection • Low grade fever & pain • Regional Lymphadenopathy

  14. 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

  15. INVESTIGATIONS • Plasma levels of albumin, ca, ph, ALP & PTH • Urea & electrolytes and full blood count • Level o vitamin D metabolites • 24 hour urinary excretion of ca & ph • CT & bone scans, US & plain x-ray films • Arterial blood gases, CK & serum amylase • Biopsy & histopathologic examination is diagnostic

  16. TREATMENT • MEDICAL • SURGICAL • DIETARY

  17. 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 • Probenecid & Colchicine • Warfarin has shown benefit in some patients

  18. NEW DRUG TRAILS • Calcitonin • Acetazolamide • Calcium-channel blockers • Sodium diphosphonates

  19. SURGICAL CARE • Indication for surgical removal include: • Pain • Recurrent infection • Ulceration • Functional impairment • Surgical trauma may stimulate calcification • Recurrence is common

  20. 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

  21. 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

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