Calcified Tissue International

, Volume 32, Issue 1, pp 1–6 | Cite as

Tumoral calcinosis: Evidence for concurrent defects in renal tubular phosphorus transport and in 1α,25-dihydroxycholecalciferol synthesis

  • Joseph E. Zerwekh
  • Lou A. Sanders
  • Judith Townsend
  • Charles Y. C. Pak
Clinical Investigation


A 50-year-old Latin American man with tumoral calcinosis presented with hyperphosphatemia (6.62±1.04 SD mg/dl), elevated renal threshold phosphorus concentration (TmP) (7.3 mg/GFR), and 1,25-dihydroxyvitamin D [1,25-(OH)2D] (69 pg/ml) hypercalciuria (239 mg/day), and a high fractional intestinal calcium (Ca) absorption (0.74). Sodium cellulose phosphate therapy (20 g/day) lowered urinary Ca, and partially reduced serum phosphorus (P) and TmP to 5.91±0.63 mg/dl and 6.2 mg/GFR, respectively. Serum 1,25-(OH)120D remained elevated at 58–64 pg/ml. Amphojel therapy (4 oz/day) decreased urinary P to 23±21 mg/day and lowered serum P to 5.75±0.36 mg/dl (P<0.05). TmP increased to a value of 8.0 mg/GFR while serum 1,25-(OH)2D continued to remain elevated at 53 pg/ml.

This case illustrates the probable operation of dual abnormalities in tumoral calcinosis represented by augmented renal conservation of P and an elevation in the circulating concentration of 1,25-(OH)2D.

Key words

Ectopic calcification 1,25-dihydroxyvitamin D hyperphosphatemia Bone Renal tubule 


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  1. 1.
    Duret, M. H.: Tumeurs multiples et singulieres des bourses, Bull. Mem. Soc. Ant. Paris74:725–731, 1899Google Scholar
  2. 2.
    Inclan, A., Leon, P., Camejo, M.G.: Tumoral calcinosis, J.A.M.A.121:490–495, 1943Google Scholar
  3. 3.
    Baldursson, H., Evans, E.B., Dodge, W.F., Jackson, W.T.: Tumoral calcinosis with hyperphosphatemia: a report of a family with incidence in four siblings, J. Bone Joint Surg. [Am]51:913–925, 1969Google Scholar
  4. 4.
    Wilber, J.F., Slatopolsky, E.: Hyperphosphatemia and tumoral calcinosis, Ann. Intern. Med.68:1044–1049, 1968Google Scholar
  5. 5.
    Chaplin, R., Clark, L.D., Ropers, M.W.: Vitamin D intoxication, Am. J. Med. Sci.221:369–378, 1969Google Scholar
  6. 6.
    Holman, C.B.: Roentgenologic manifestations of vitamin D intoxication, Radiology69:805–816, 1952Google Scholar
  7. 7.
    Christensen, W.R., Liebman, C., Sosman, M.C.: Skeletal and periarticular manifestations of hypervitaminosis D, Am. J. Roentgenol. Radium Ther.65:27–41, 1951PubMedGoogle Scholar
  8. 8.
    Walker, G.S., Davison, A.M., Peacock, M., McLachlan, M.S.F.: Tumoral calcinosis: a manifestation of extreme metastatic calcification occurring with 1α, -hydroxycholecalciferol therapy, Postgrad. Med. J.53:570–573, 1977PubMedGoogle Scholar
  9. 9.
    Prince, M.J., Schaefer, P.C., Goldsmith, R.S., Chausmer, A.B.: Tumoral calcinosis: an ectopic calcification syndrome associated with elevated serum 1,25-dihydroxycholecalciferol (1,25-(OH)2D3), Program and Abstracts, Annual Meeting, Endocrine Society, Abst. 322, p. 235, 1978Google Scholar
  10. 10.
    Hacihanefioglu, U.: Tumoral calcinosis. A clinical and pathological study of eleven unreported cases in Turkey, J. Bone Joint Surg. [Am]60:1131–1135, 1978Google Scholar
  11. 11.
    Pak, C.Y.C., Stewart, A., Kaplan, R., Bone, H., Notz, C., Brown, R.: Photon absorptiometric analysis of bone density in primary hyperparathyroidism, Lancet2:7–9, 1975CrossRefPubMedGoogle Scholar
  12. 12.
    Pak, C.Y.C., Ohata, M., Lawrence E.C., Snyder, W.: The hypercalciurias: causes, parathyroid functions, and diagnostic criteria, J. Clin. Invest.54:387–400, 1974PubMedGoogle Scholar
  13. 13.
    Bone, H.E., III, Zerwekh, J., Haussler, M.R., Pak, C.Y.C.: Effect of parathyroidectomy on serum 1α,25-dihydroxyvitamin D and on intestinal calcium absorption in primary hyperparathyroidism, J. Clin. Endocrinol. Metab.48:877–879, 1979PubMedGoogle Scholar
  14. 14.
    Silox, D.C., McCarty, D.J.: Measurement of inorganic pyrophosphate in biological fluids: elevated levels in some patients with osteoarthritis, pseudogout, acromegaly, and uremia, J. Clin. Invest.52:1863–1870, 1973Google Scholar
  15. 15.
    Walton, R.J., Bijvoet, O.L.M.: Nomogram for derivation of renal threshold phosphate concentration, Lancet ii:209–210, 1975Google Scholar
  16. 16.
    Blacklock, N.J., MacLeod, M.A.: The effect of cellulose phosphate on intestinal absorption and urinary excretion of calcium, Br. J. Urol.46:385–392, 1974PubMedCrossRefGoogle Scholar
  17. 17.
    Fiske, C.H., Subbarow, Y.: The colorimetric determinations of phosphorus, J. Biol. Chem.66:375–400, 1925Google Scholar
  18. 18.
    Gilman, A.G.: A protein binding assay for adenosine 3′:5′-cyclic monophosphate, Proc. Natl. Acad. Sci. U.S.A.67:305–312, 1970PubMedGoogle Scholar
  19. 19.
    Pak, C.Y.C., Fetner, C., Townsend, J., Brinkley, L., Northcutt, C., Barilla, D.E., Kadesky, M., Peters, P.: Evaluation of calcium urolithiasis in ambulatory patients: comparison of results with those of inpatient evaluation, Am. J. Med.64:979–987, 1978CrossRefPubMedGoogle Scholar
  20. 20.
    Eisman, J.A., Hamstra, A.J., Kream, B.D., DeLuca, H.F.: A sensitive precise and convenient method for determination of 1,25-dihydroxyvitamin D in human plasma, Arch. Biochem. Biophys.176:235–243, 1976CrossRefPubMedGoogle Scholar
  21. 21.
    Zerwekh, J.E., Glass, K., Jowsey, J., Pak, C.Y.C.: A unique form of osteomalacia associated with end organ refractoriness to 1,25-dihydroxyvitamin D and apparent defective synthesis of 25-hydroxyvitamin D, J. Clin. Endocrinol. Metab.49:171–175, 1979PubMedCrossRefGoogle Scholar
  22. 22.
    Brumbaugh, P.F., Haussler, D.H., Bursac, K.M., Haussler, M.R.: Filter assay for 1α,25-dihydroxyvitamin D3: utilization of the hormone's target tissue chromatin receptor, Biochemistry13:4091–4097, 1974CrossRefPubMedGoogle Scholar
  23. 23.
    Haddad, J.G., Chyu, K.F.: Competitive protein-binding assay for 25-hydroxycholecalciferol, J. Clin. Endocrinol. Metab.33:992–995, 1971PubMedGoogle Scholar
  24. 24.
    Diaz-Buxo, J.A., Knox, F.G.: Effects of parathyroid hormone on renal function, Mayo Clin. Proc.50:537–541, 1975PubMedGoogle Scholar
  25. 25.
    DeLuca, H.F.: The kidney as an endocrine organ for the production of 1,25-dihydroxyvitamin D3, a calcium mobilizing hormone, N. Engl. J. Med.289:359–365, 1973PubMedCrossRefGoogle Scholar
  26. 26.
    Tanaka, Y., DeLuca, H.F.: The control of 25-hydroxyvitamin D metabolism by inorganic phosphorus, Arch. Biochem. Biophys.154:566–574, 1973CrossRefPubMedGoogle Scholar
  27. 27.
    Reiss, E., Canterbury, J.M., Bercovitz, M.A., Kaplan, E.L.: The role of phosphate in the secretion of parathyroid hormone in man, J. Clin. Invest.49:2146–2149, 1970PubMedCrossRefGoogle Scholar
  28. 28.
    Recker, R.R., Hassing, G.S., Lau, J.R., Saville, P.D.: The hyperphosphatemic effect of disodium ethane-1-hydroxy-1, 1-diphosphonate (EHDPTm): renal handling of phosphorus and the renal response to parathyroid hormone, J. Lab. Clin. Med.81:258–266, 1973PubMedGoogle Scholar
  29. 29.
    Francis, M.D., Russell, R.G., Fleisch, H.: Diphosphonates inhibit formation of calcium phosphate crystals in vitro and pathological calcification in vivo, Science165:1264–1266, 1969PubMedGoogle Scholar
  30. 30.
    Kaplan, R.A., Geho, W.G., Poindexter, C., Haussler, M., Dietz, G.W., Pak, C.Y.C.: Metabolic effects of diphosphonate in primary hyperparathyroidism, J. Clin. Pharmacol.17:410–419, 1977PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1980

Authors and Affiliations

  • Joseph E. Zerwekh
    • 1
  • Lou A. Sanders
    • 1
  • Judith Townsend
    • 1
  • Charles Y. C. Pak
    • 1
  1. 1.Section on Mineral Metabolism, Department of Internal Medicine, Southwestern Medical SchoolThe University of Texas Health Science Center at DallasDallasUSA

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