Abstract
A 51-year old man presented with the classical features of Cushing’s syndrome which had evolved over the previous 5 yr, and was found to have ectopic ACTH secretion from an atypical thymic carcinoid tumor. Tumor extract, assayed under conditions designed to prevent artefactual generation of peptides, was found to contain a wide variety of immunoreactive hormones including ACTH, α-MSH, CLIP, β-endorphin and met-enkephalin. The ACTH-related peptides were probably derived from a common precursor, pro-opiocortin, but the presence of met-enkephalin suggests the production of a separate type of precursor molecule. The tumor was locally invasive and, despite subtotal excision and radiotherapy, continued to secrete large amounts of ACTH. Hypercortisolism was controlled longterm with pharmacological adrenal blockade and steroid replacement.
Similar content being viewed by others
References
Azzopardi J.G., Williams E.D. Pathology of non-endocrine tumours associated with Cushing’s Syndrome. Cancer 22: 274, 1968.
Clement-Jones V., Corder R., Lowry P.J. Isolation of human met-enkephalin and two groups of putative precursors (2K-pro-met-enkephalin) from an adrenal medullary tumour. Biochem. Biophys. Res. Commun. 95: 665, 1980.
Clement-Jones V., Lowry P.J., Rees L.H., Besser G.M. Development of a specific extracted RIA for met-enkephalin in human plasma and cerebro-spinal fluid. J. Endocrinol. 86: 231, 1980.
Donald R.A. Application bf the coated charcoal separation method to the radioimmunoassay of plasma corticotrophin. J. Endocrinol. 41: 499, 1968.
Drekter I.J., Heisler A., Seism G.R., Stern S., Pearson S., McGavack T.H. The detection of urinary steroids, the preparation of pigment-free extracts and a simplified procedure for the estimation of total 17-ketosteroids. J. Clin. Endocrinol. Metab. 12: 55, 1952.
Few J.D. A method for the analysis of urinary 17-hydroxycorticoste-roids. J. Endocrinol. 22: 31, 1961.
Gilby E.D., Rees L.H., Bondy P.K. Biological characterization of human tumours. Excerpta Medica 375: 132, 1975.
Gilkes J.J., Bloomfield G.A., Scott A.P., Lowry P.R., Ratcliffe J.G., Landon J., Rees L.H. Development and validation of a radioimmunoassay for peptides related to/3-melanocyte stimulating hormone in human plasma: The Lipotrophins. J. Clin. Endocrinol. Metab. 40: 450, 1975.
Gilkes J.J., Rees L.H., Besser G.M. Plasma immunoreactive corticotrophin and lipotrophin in Cushing’s syndrome and Addison’s disease. Br. Med. J. 1: 996, 1977.
Imura H., Matsukura S., Yamamoto H., Hirata Y., Nakai Y., Endo J., Tanaka A., Nakamura M. Studies on ectopic ACTH-producing tumours. II. Clinical and biomedical features of 30 cases. Cancer 35: 1430, 1975.
Jeffcoate W., Rees L.H. Adrenocorticotrophin and related peptides in nonendocrine tumours. Curr. Top. Exp. Endocrinol. 3: 57, 1978.
Lerner A.B., McGuire J.A. Melanocyte-stimulating hormone and adrenal corticotrophic hormone: their relation to pigmentation. N. Engl. J. Med. 270: 539, 1964.
Leyton O., Turnbull H.M., Bratton A. Primary cancer of the thymus with pluriglandular disturbance. J. Pathol. 34: 635, 1931.
Liddle G.W., Nicholson W.E., Island D.P., Orth D.N., Abe K., Lowder S.C. Clinical and laboratory studies of ectopic humoral syndromes. Recent Prog. Horm. Res. 25: 283, 1969.
Rees LH. Human ACTH and LPH in health and disease. In: Martini L., Besser G.M. (Eds.), Clinical Neuroendocrinology. Academic Press, N. Y., 1978, p. 429.
Murphy B.E. Some studies of the protein-binding of steroids and their application to the routine micro and ultramicro measurement of various steroids in body fluids by competitive protein-binging radioassay. J. Clin. Endocrinol. Metab. 27: 973, 1967.
Pullan P.T., Clement-Jones V., Corder R., Lowry P.J., Besser G.M., Rees L.H. ACTH, LPH and related peptides in the ectopic ACTH syndrome. Clin. Endocrinol. (Oxf.). 13: 437, 1980.
Pullan P.T., Clement-Jones V., Corder R., Lowry P.J., Rees G.M., Besser G.M., Macedo M.N., Galvao-Teles A. Ectopic production of methionine enkephalin and beta endorphin. Br. Med. J. 2: 758, 1980.
Ratcliffe J.G., Knight R.A., Besser G.M., Landon J., Stansfield A.G. Tumour and plasma ACTH concentrations in patients with and without the ectopic ACTH syndrome. Clin. Endocrinol. (Oxf.) 1: 27, 1972.
Ratter S.J., Lowry P.J., Besser G.M., Rees L.H. Chromatographie characterisation of ACTH in human plasma. J. Endocrinol. 85: 359, 1980.
Rees L.H., Bloomfield G.A., Gilkes J.H., Jeffcoate W.J., Besser G.M. ACTH as a tumour marker. Am. NY. Acad. Sci. 297: 603, 1977.
Roasi J., Higa E. Mediastinal endocrine neoplasm of probable thymic origin related to carcinoid tumour; clinicopathologic study of 8 cases. Cancer 29: 1061, 1972.
Roasi J., Levine G., Weber W.R., Higa E. Carcinoid tumours and oat cell carcinomas of the thymus. Pathol. Annu. 11: 201, 1976.
Schteingart D.E., Conn J.W., Orth D.N., Harrison T.S., Fox F.F., Bookstein J.J. Secretion of ACTH and β-MSH by an adrenal medullary paraganglioma. J. Clin. Endocrinol. Metab. 34: 676, 1972.
Yalow R.S., Berson S.A. Size heterogeneity of immunoreactive human ACTH in plasma and in extracts of pituitary glands and ACTH producing thymoma. Biochem. Biophys. Res. Commun. 44: 439, 1971.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Baker, J., Holdaway, I.M., Jagusch, M. et al. Ectopic secretion of ACTH and met-enkephalin from a thymic carcinoid. J Endocrinol Invest 5, 33–37 (1982). https://doi.org/10.1007/BF03350479
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/BF03350479