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Indian Pediatr 2017;54:521

Infantile Tumoral Calcinosis

#Lavleen Singh, *Amitabh Singh and Sataroopa Mishra

From Departments of #Pathology and Pediatrics, Chacha Nehru Bal Chikitsalaya, Geeta Colony, New Delhi, India.
Email: [email protected]


A 2-months-old girl was brought with complaints of multiple swellings over scalp since neonatal period. Boggy swellings, free from underlying bone and with no change in overlying skin, were noticed over scalp in left parietal and occipital region (Fig. 1). Similar swellings developed subsequently over both feet and hand. Laboratory investigations showed hyperphosphatemia (serum phosphorus 7.19 mg/dL) with normal serum calcium, alkaline phosphatase, parathormone, 25-hydroxy vitamin D, renal and liver function tests. Skeletal survey showed juxtaarticular calcification over shoulder, hip joints and gluteal region (Fig. 2). Histopathological examination of the excised wrist swelling showed features of tumoral calcinosis. The child was treated with phosphate binder and acetazolamide. At two month follow-up, phosphate levels were normal and there was no increase in the size of the lesions or appearance of new lesion.

Fig. 1 Boggy swellings over scalp in an infant with hyper-phosphatemic tumoral calcinosis.


Fig. 2 Juxtarticular calcification seen around shoulder, hip joint and gluteal region in the infant with Hyperphosphatemic tumoral calcinosis.

Hyperphosphatemic tumoral calcinosis is a familial condition characterized by hyperphosphatemia and abnormal deposits of phosphate and calcium (calcinosis) in the body’s tissues. Soft tissue calcification secondary to renal disorder can be differentiated easily by renal function test and vitamin D levels. Medical management is preferred due to metabolic nature of underlying pathology and high recurrence rate after surgical resection. Lifelong phosphate binder and low phosphate diet may be required.


 

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