Parathyroid-hormone-related protein-mediated hypercalcemia in benign congenital mesoblastic nephroma.
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.
Pediatric nephrology (Berlin, Germany)
Humans; Hypercalcemia; Infant, Newborn; Infant, Premature; Kidney Neoplasms; Male; Nephrectomy; Nephroma, Mesoblastic; Parathyroid Hormone-Related Protein
Srivastava, T., Kats, A., Martin, T. J., Pompolo, S., Alon, U. S. Parathyroid-hormone-related protein-mediated hypercalcemia in benign congenital mesoblastic nephroma. Pediatric nephrology (Berlin, Germany) 26, 799-803 (2011).