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Indian Pediatr 2017;54: 521 |
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Infantile Tumoral
Calcinosis
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# 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]
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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.
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Fig. 1 Boggy swellings over scalp in
an infant with hyper-phosphatemic tumoral calcinosis.
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Fig. 2 Juxtarticular calcification
seen around shoulder, hip joint and gluteal region in the infant
with Hyperphosphatemic tumoral calcinosis.
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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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