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The Journal of Clinical Endocrinology & Metabolism Vol. 87, No. 10 4457-4461
Copyright © 2002 by The Endocrine Society

CLINICAL CASE SEMINAR

A 21-Year-Old Woman with Consumptive Hypothyroidism due to a Vascular Tumor Expressing Type 3 Iodothyronine Deiodinase

Stephen A. Huang, Stephanie A. Fish, David M. Dorfman, Domenico Salvatore, Harry P. W. Kozakewich, Susan J. Mandel and P. Reed Larsen

Division of Endocrinology (S.A.F., S.J.M.), Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 19104; Thyroid Division and Division of Endocrinology (P.R.L.) and Department of Pathology (D.M.D.), Brigham and Women’s Hospital, Boston, Massachusetts 02115; Department of Pathology (H.P.W.K.) and Division of Endocrinology (S.A.H.), Children’s Hospital Boston, Boston, Massachusetts 02115; and Dipartimento di Endocrinologia ed Oncologia Molecolare e Clinica (D.S.), Universita’degli Studi di Napoli "Federico II," 80138 Naples, Italy

Address all correspondence and requests for reprints to: Dr. P. Reed Larsen, Thyroid Division, Harvard Medical School, Room 560, Harvard Institutes of Medicine, 77 Avenue Louis Pasteur, Boston, Massachusetts 02115. E-mail: rlarsen{at}rics.bwh.harvard.edu.

Abstract

We present a 21-yr-old female with a large hepatic vascular tumor and subclinical hypothyroidism. A high level of the thyroid hormone inactivating enzyme type 3 iodothyronine deiodinase (D3) was detected in her tumor, and the TSH of 26.2 mU/liter returned to normal after surgical resection of the mass. This indicates that the vascular tumor caused this adult’s hypothyroidism as has now been documented in nine infants with this syndrome. This first example of consumptive hypothyroidism in an adult indicates that the inactivation rate of thyroid hormone by D3 in a vascular tumor can stress the secretory capacity even of the TSH-stimulated normal adult thyroid gland.




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