Journal of Clinical Endocrinology & Metabolism
, doi:10.1210/jc.2008-0458
The Journal of Clinical Endocrinology & Metabolism Vol. 93, No. 9 3282-3283
Copyright © 2008 by The Endocrine Society
Corticotrophin-Dependent Cushing Syndrome Due to Sacrococcygeal Teratoma Detected by [18F]Fluorodeoxyglucose Positron Emission Tomography
J. Moreno-Fernández,
C. Gutiérrez-Alcántara,
M. A. Gálvez Moreno,
L. Jiménez-Reina,
J. P. Castaño and
P. Benito-López
Service of Endocrinology and Nutrition (J.M.-F., C.G.-A., M.A.G.M., P.B.-L.), Reina Sofía Hospital. E-14004 Córdoba, Spain; and Departments of Morphological Sciences (L.J.-R.) and Cell Biology, Physiology, and Immunology (J.P.C.), University of Córdoba, E-14014 Córdoba, Spain
Address all correspondence and requests for reprints to: J. Moreno-Fernández, M.D., Service of Endocrinology and Nutrition., Reina Sofía Hospital., Avenida Menendez Pidal s/n., Córdoba. 14004. Spain. E-mail: mirendjmf1976{at}hotmail.com.
A 33-yr-old woman was evaluated for ACTH-dependent Cushing syndrome (CS). The routine protocol study did not localize a source for ACTH hypersecretion. Findings on 18F-fluorodeoxyglucose positron emission tomography (18FDG-PET) (Fig. 1
) were consistent with a sacrococcygeal neoplasm, subsequently removed and identified as mature extragonadal teratoma.
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FIG. 1. A–D, 18FDG-PET coronal, transaxial, sagittal and volumetric sections showing a 1.5-cm-diameter lesion with a standard uptake value of 5.1 (normal, <2.5) in sacrococcygeal area (black arrows) that was subsequently identified as the ACTH-producing tumor; E, histological appearance and immunohistochemical detection of ACTH in the removed mature teratoma. ACTH immunostaining in the tumor appears brown. Hematoxylin and eosin counterstain; magnification, x400.
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Diagnosis and treatment of CS currently constitute challenging problems in clinical endocrinology. Different approaches to search ACTH-secreting tumors can be used including computed tomography, magnetic resonance imaging, whole-body [111In] diethylenetinamine penta-actic acid (DTPA)-pentetreotide and venous sampling for ACTH (1, 2, 3). Nevertheless, none of these techniques, including catheterizations of the inferior petrosal sinus and inferior cava vein, identified the ACTH-secreting tumor in our patient. Given the severity of CS, bilateral adrenalectomy was performed to cure the hypercortisolism. Subsequent imaging with 18FDG-PET was consistent with a sacrococcygeal neoplasm (Fig. 1
), which was subsequently removed and confirmed to be a mature teratoma with positive immunostaining for ACTH (Fig. 1E), resulting in successful normalization in serum ACTH levels.
Teratomas are known to produce several hormones, mainly human chorionic gonadotropin, but may rarely cause CS (4, 5). To our knowledge, this is the first case of ACTH CS caused by a mature sacrococcygeal teratoma. In addition, we suggest that 18FDG-PET may have a role in the evaluation of some patients with ectopic ACTH CS.
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Footnotes
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Disclosure Statement: The authors (Moreno-Fernández J., Gutiérrez-Alcántara C., Gálvez Moreno M.A., Jiménez-Reina L., Castaño J.P. and Benito-López P.) have nothing to disclose.
Support for this work was provided in part by Grants CVI-139 and CTS-0175 (Plan Andaluz de Investigación, Junta de Andalucía, Spain), and BFU2007-60180/BFI (Ministerio de Educación y Ciencia, Spain/FEDER).
Abbreviation: 18FDG-PET, [18F]Fluorodeoxyglucose positron emission tomography.
Received February 28, 2008.
Accepted June 6, 2008.
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References
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