title: 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.affiliation: University of Missouri, Kansas City, MO 64108, USA. tsrivastava@cmh.edu
authors.forename: Tarak
authors.initials: T
authors.lastname: Srivastava
authors.forename: Alexander
authors.initials: A
authors.lastname: Kats
authors.forename: T John
authors.initials: TJ
authors.lastname: Martin
authors.forename: Suelli
authors.initials: S
authors.lastname: Pompolo
authors.forename: Uri S
authors.initials: US
authors.lastname: Alon
mesh_terms: D006801:Humans; D006934:Hypercalcemia / Q000209:etiology* / Q000378:metabolism* / Q000473:pathology; D007231:Infant, Newborn; D007234:Infant, Premature; D007680:Kidney Neoplasms / Q000150:complications* / Q000378:metabolism / Q000601:surgery; D008297:Male; D009392:Nephrectomy; D018201:Nephroma, Mesoblastic / Q000150:complications* / Q000378:metabolism / Q000601:surgery; D044162:Parathyroid Hormone-Related Protein / Q000378:metabolism*
chemical_list: D044162:Parathyroid Hormone-Related Protein