Parathyroid-hormone-related protein-mediated hypercalcemia in benign congenital mesoblastic nephroma.

Abstract

Parathyroid hormone-related protein (PTHrP) mediated hypercalcemia of malignancy is rare in children, and even more so in the setting of a benign tumor. We report two infants with PTHrP-mediated hypercalcemia secondary to congenital mesoblastic nephroma and their outcome after removal of the benign tumor. Pre-operatively hypercalcemia was corrected with saline hydration, furosemide, calcitonin and/ or pamidronate. Following resection of the tumor serum PTHrP normalized. Immunohistochemical staining of tumor cells was positive for PTHrP. Post-operatively the infants developed elevated serum parathyroid hormone with low- normal serum Ca and P, and undetectable urinary Ca and P, probably due to their movement into bone. Children needed treatment with calcitriol, Ca and P supplementation for 6-12 weeks until PTH normalized and urinary Ca and P were detected, suggesting bone replenishment. We conclude that benign congenital mesoblastic nephroma can secrete PTHrP that can cause severe hypercalcemia; and following excision one should anticipate the development of a transient modified "hungry bone"-like condition requiring Ca, P and calcitriol therapy for several weeks accompanied by careful monitoring of mineral homeostasis.

• Authors: Tarak Srivastava, Alexander Kats, T John Martin, Suelli Pompolo, Uri S Alon
• Journal: Pediatric nephrology (Berlin, Germany) (Pediatr Nephrol) Vol. 26 Issue 5 Pg. 799-803 (May 2011) ISSN: 1432-198X [Electronic] Germany
• PMID: 21161280 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Parathyroid Hormone-Related Protein

Topics

• Humans
• Hypercalcemia (etiology, metabolism, pathology)
• Infant, Newborn
• Infant, Premature
• Kidney Neoplasms (complications, metabolism, surgery)
• Male
• Nephrectomy
• Nephroma, Mesoblastic (complications, metabolism, surgery)
• Parathyroid Hormone-Related Protein (metabolism)