Abstract
This is apparently the first report on connatal hemihypertrophy with malignant pheochromocytoma. The coincidence of hemihypertrophy with other diseases, particularly neuroectodermal dysplasias on the one hand and the frequent association of neuroectodermal dysplasias with pheochromocytoma on the other, are emphasized. Furthermore, basically known particularities of this case as malignancy of the tumor, the unusual size of the tumor in children, and the normal catecholamine levels in serum as well as the normal excretion of vanillylmandelic acid are discussed.
Similar content being viewed by others
References
Bachmann, K. D., Ziadi, J.: Über das Phäochromocytom im Kindesalter. Fortschr. Med. 18, 743–762 (1971)
Benson, P. F., Vulliamy, D. G., Taubmann, J. O.: Congenital hemihypertrophy and malignancy. Lancet 1963 I, 468–469
Blessing, M. H., Sadoni, V., Schöfer, P., Wagner, H. E.: Riesenphäochromocytome. Med. Welt 22, 1668–1671 (1971)
Böhmer, R., Schairer, K. W.: Malignes Phäochromocytom. Herz/Kreisl. 6, 380–386 (1974)
Dean MacEwen, G., Case, J. L.: Congenital hemihypertrophy. Clin. Orthop. 50, 147–150 (1967)
Duven, H. E.: Hemihypertrophie. Mschr. Kinderheilk. 121, 721–724 (1973)
Evans, R. W.: Histological appearances of tumours, 2nd ed. Edinburgh-London: Reprint. Livingstone 1968
Fraumeni, J. F., Jr., Geiser, C. F., Manning, M. D.: Wilms' tumor and congenital hemihypertrophy: Report of five new cases and review of literature. Pediatrics 40, 886–899 (1967)
Fraumeni, J. F., Jr., Miller, R. W.: Adrenocortical neoplasm with hemihypertrophy, brain tumors, and other disorders. J. Pediat. 70, 129–138 (1967)
Gitlow, St. E., Bertani, L. M., Greenwood, St. M., Lan Wong, B., Dziedzic, St. W.: Benign pheochromocytoma associated with elevated excretion of homovanillic acid. J. Pediat. 81, 1112–1116 (1972)
Grenzmann, M., Gerhards, K. P., Thelen, M.: Chromaffine Tumoren. Diagnostik 4, 121–124 (1972)
Hedinger, Chr.: Endokrine Organe. In: Organpathologie (ed. W. Doerr), Vol. II. Stuttgart: Thieme 1974
Helson, L., Fleischer, M., Bethune, V., Murphy, M. L., Schwartz, M. K.: Urinary cystathionine, catecholamine, and metabolites in patients with neuroblastoma. Clin. Chem. 18 (7), 613–615 (1972)
Keller, H.: In: Auftrag der Klinik an das klin.-chem. Laboratorium. Merck-Symposium, Wiesbaden 1970 (eds. H. Lang, W. Rick, W.-P. Fritsch). Stuttgart-New York: Schattauer 1972
Labhart, H.: Klinik der inneren Sekretion, 2nd ed. Berlin-Heidelberg-New York: Springer 1971
Lohmann, F. W., Schüren, K. P.: Der heutige Stand der Diagnostik beim Phäochromocytom. Internist 13, 41–44 (1972)
Louis, C., Diekmann, L., Brisse, B., Müller, K.-M.: Beitrag zum Phäochromocytom im Kindesalter. Z. Kinderheilk. 11, 1–4 (1975)
Meadows, A. T., Lichtenfeld, J. L., Koop, C. E.: Wilms's tumor in three children of a woman with congenital hemihypertrophy. New Engl. J. Med. 291, 23–24 (1974)
Miller, R. W., Fraumeni, J. F., Jr.: Bethesda, National Institute of Health 1974 (personal communication)
Miller, R. W., Fraumeni, J. F., Jr., Manning, M. D.: Association of Wilms' tumor with hemihypertrophy and other congenital malformations. New Engl. J. Med. 270, 922–927 (1964)
Neithardt, M.: Personal communication 1973
Palmieri, G., Ikkos, D., Luft, R.: Malignant phäochromocytoma. Acta endocr. 36, 549 (1961)
Pecherstorfer, M., Raptis, S.: Über einen Fall eines malignen Phäochromocytoms. Wien. klin. Wschr. 25, 459 (1966)
Pfister, R. C., Weber, A. L., Smith, E. H., Wilkinson, R. H., May, D. A.: Congenital asymmetry (hemihypertrophy) and abdominal disease. Radiology 116, 685–691 (1975)
Rath, F., Rosenmayr, F., Wiltschke, H.: Phäochromocytom im Kindesalter. Bericht über zwei Fälle. Dtsch. med. Wschr. 95, 2236–2240 (1970)
Riedel, H. A.: Adrenogenital syndrome in a male child due to adrenocortical tumor. Report of case with hemihypertrophy and subsequent development of embryoma (Wilms' tumor). Pediatrics 10, 19–27 (1952)
Ringrose, R. E., Jabbour, J. T., Keele, D. K.: Hemihypertrophy. Pediatrics 36, 434–448 (1965)
Robinson, M. J., Kent, M., Stocks, J.: Pheochromocytoma in childhood. Report of 3 cases. Arch. Dis. Childh. 48, 137–144 (1973)
Robinson, M. J., Williams, A.: Clinical and pathological details of two cases of pheochromocytoma in childhood. Arch. Dis. Childh. 31, 69–74 (1956)
Sack, H., Mühlhoff, G.: Das Phäochromocytom. Dtsch. Ärztebl. 11, 769–774 (1974)
Sandritter, W., Lasch, H. G.: Phäochromocytom — malignes Phäochromocytom. Med. Welt 2, 113–117 (1967)
Sava, P., Mourot, M., Carbillet, J.-P., Gille, P.: A propos d'un cas de neurofibromatose avec hypertrophie segmentaire d'un membre. Ann. Pédiat. 22, 355–361 (1975)
Schaeffer, E.: Halbseitenriesenwuchs und Wilms' Tumor. Mschr. Kinderheilk. 108, 504–506 (1960)
Schmid, B.: Pathophysiologie, Klinik und Therapie des Phäochromocytoms. Med. Welt 21, 1424–1428 (1970)
Sprayregen, S., Strasberg, Z., Naidich, T. P.: Medullary sponge kidney and congenital total hemihypertrophy. N.Y. St. J. Med. 73, 2768–2772 (1973)
Stackpole, R. H., Melicow, M. M., Uson, A. C.: Pheochromocytoma in children. J. Pediat. 63, 315–330 (1963)
Steigleder, G. K., Gartmann, H., Jung, K.: Hautveränderungen bei Phäochromocytom. Zur Differentialdiagnose der Akrozyanose und Livedo racemosa. Z. Hautkr. 49, 647–652 (1974)
Strunge, P., Ingstrup, H. M., Lochte, J. J., Zimmermann-Nielsen, C.: Bilateral phaeochromocytoma in two brothers. Acta paediat. scand. 61, 729–732 (1972)
Ward, J., Lerner, H.: A review of the subject of congenital hemihypertrophy and a complete case report. J. Pediat. 31, 403–413 (1947)
Wenz, W., Roth, E.: Angiographische Diagnostik des Phäochromocytoms. Med. Welt 21, 1653–1660 (1970)
Werning, C., Siegenthaler, W.: Diagnostik des Phäochromocytoms. Dtsch. med. Wschr. 96, 121–124 (1971a)
Werning, C., Siegenthaler, W.: Therapie des Phäochromocytoms. Dtsch. med. Wschr. 96, 124–126 (1971b)
Wiedemann, H.-R.: Exomphalos-Makroglossie-Gigantismus-Syndrom, Berardinelli-Seip-Syndrom und Sotos-Syndrom—eine vergleichende Betrachtung unter ausgewählten Aspekten. Z. Kinderheilk. 115, 193–207 (1973)
Wiedemann, H.-R., Tolksdorf, M., Hansen, H. G., Klose, K.: Chromosomenuntersuchungen bei partiellem Riesenwuchs. Mschr. Kinderheilk. 112, 281 (1964)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Schnakenburg, K.v., Müller, M., Dörner, K. et al. Congenital hemihypertrophy and malignant giant pheochromocytoma — a previously undescribed coincidence. Eur J Pediatr 122, 263–273 (1976). https://doi.org/10.1007/BF00481506
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00481506