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