ClinicoPathological Conference 25 Dec' 2002

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 gtfemales, 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
(No Transcript)
17
TREATMENT

• MEDICAL

• SURGICAL

• DIETARY

18
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

19
NEW DRUG TRAILS

• Calcitonin

• Acetazolamide

• Calcium-channel blockers

• Sodium diphosphonates

20
SURGICAL CARE

• Indication for surgical removal include
• Pain
• Recurrent infection
• Ulceration
• Functional impairment

• Surgical trauma may stimulate calcification

• Recurrence is common

21
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

22
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