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Neha Verma, Manisha Shah, Luma Ghalib, Lawrence Kirschner, Mayumi Endo, and Bhavana Konda

Ectopic Cushing syndrome is a rare disease process with approximately 28 – 59% of all cases being from neuroendocrine tumors (NET). Here we present a case series of well differentiated NET with Adrenocorticotropic hormone (ACTH) production. Case1: A

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Jonathan R. Strosberg, Gary D. Hammer, and Gerard M. Doherty

Edited by Kerrin G. Robinson

Adrenocortical carcinomas (ACCs) are rare tumors that arise from the cortex of the adrenal gland with an incidence 1 to 2 per million. The rarity of this tumor translates into a paucity of experience in managing patients in most medical centers. Because clinical series are small and prospective evaluation of treatment strategies is limited, the current state of knowledge is strongly influenced by expert consensus opinion from a few medical centers specializing in ACCs. This article describes the basic diagnostic and prognostic issues in adrenal cancer management, and presents detailed rationales for therapeutic management.

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Leena Gandhi and Bruce E. Johnson

system . N Engl J Med 2003 ; 349 : 1543 – 1554 . 6. Brown WH . A case of pluriglandular syndrome: ``diabetes of bearded women'' . Lancet 1928 : 1022 – 1023 . 7. Ilias I Torpy DJ Pacak K . Cushing's syndrome due to ectopic

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Manisha H. Shah, Whitney S. Goldner, Al B. Benson III, Emily Bergsland, Lawrence S. Blaszkowsky, Pamela Brock, Jennifer Chan, Satya Das, Paxton V. Dickson, Paul Fanta, Thomas Giordano, Thorvardur R. Halfdanarson, Daniel Halperin, Jin He, Anthony Heaney, Martin J. Heslin, Fouad Kandeel, Arash Kardan, Sajid A. Khan, Boris W. Kuvshinoff II, Christopher Lieu, Kimberly Miller, Venu G. Pillarisetty, Diane Reidy, Sarimar Agosto Salgado, Shagufta Shaheen, Heloisa P. Soares, Michael C. Soulen, Jonathan R. Strosberg, Craig R. Sussman, Nikolaos A. Trikalinos, Nataliya A. Uboha, Namrata Vijayvergia, Terence Wong, Beth Lynn, and Cindy Hochstetler

occur frequently. Approximately 60% of patients present with evidence of adrenal steroid hormone excess, with or without virilization. 72 Signs and symptoms associated with hypersecretion of cortisol, called Cushing syndrome, include weight gain

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Matthew H. Kulke, Al B. Benson III, Emily Bergsland, Jordan D. Berlin, Lawrence S. Blaszkowsky, Michael A. Choti, Orlo H. Clark, Gerard M. Doherty, James Eason, Lyska Emerson, Paul F. Engstrom, Whitney S. Goldner, Martin J. Heslin, Fouad Kandeel, Pamela L. Kunz, Boris W. Kuvshinoff II, Jeffrey F. Moley, Venu G. Pillarisetty, Leonard Saltz, David E. Schteingart, Manisha H. Shah, Stephen Shibata, Jonathan R. Strosberg, Jean-Nicolas Vauthey, Rebekah White, James C. Yao, Deborah A. Freedman-Cass, and Mary A. Dwyer

) seem to occur frequently. Approximately 60% of patients present with evidence of adrenal steroid hormone excess, with or without virilization. 118 , 131 - 133 Signs and symptoms associated with hypersecretion of cortisol, called Cushing syndrome

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Treatment of Cushing's Syndrome (Nieman LK, et al. J Clin Endocrinol Metab 2015;100:2807-2831). AGT-2 • Top pathway, second column was revised: “ Rule out pheochromocytoma (See NE-C). Check plasma free or 23 hour urine fractionated metanephrines (See NE

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Gregory P. Kalemkerian, Wallace Akerley, Paul Bogner, Hossein Borghaei, Laura QM Chow, Robert J. Downey, Leena Gandhi, Apar Kishor P. Ganti, Ramaswamy Govindan, John C. Grecula, James Hayman, Rebecca Suk Heist, Leora Horn, Thierry Jahan, Marianna Koczywas, Billy W. Loo Jr, Robert E. Merritt, Cesar A. Moran, Harvey B. Niell, Janis O’Malley, Jyoti D. Patel, Neal Ready, Charles M. Rudin, Charles C. Williams Jr, Kristina Gregory, and Miranda Hughes

cells sometimes produce polypeptide hormones, including vasopressin (antidiuretic hormone [ADH]) and adrenocorticotropic hormone, which cause hyponatremia of malignancy (ie, syndrome of inappropriate ADH secretion [SIADH]) and Cushing syndrome

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Kamran Aghayev, Frank Vrionis, and Marc C. Chamberlain

melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome . Am J Surg Pathol 1990 ; 14 : 206 – 222 . 95. Abul-Kasim K Thurnher MM McKeever P . Intradural spinal tumors

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R. Michael Tuttle, Douglas W. Ball, David Byrd, Gilbert H. Daniels, Raza A. Dilawari, Gerard M. Doherty, Quan-Yang Duh, Hormoz Ehya, William B. Farrar, Robert I. Haddad, Fouad Kandeel, Richard T. Kloos, Peter Kopp, Dominick M. Lamonica, Thom R. Loree, William M. Lydiatt, Judith McCaffrey, John A. Olson Jr., Lee Parks, John A. Ridge, Jatin P. Shah, Steven I. Sherman, Cord Sturgeon, Steven G. Waguespack, Thomas N. Wang, and Lori J. Wirth

calcitonin, occasionally along with other hormonally active peptides (i.e., adrenocorticotrophic hormone [ACTH] or calcitonin-gene related peptide [CGRP]), can contribute to the development of diarrhea, Cushing's syndrome, or facial flushing in many patients

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Apar Kishor P. Ganti, Billy W. Loo Jr., Michael Bassetti, Collin Blakely, Anne Chiang, Thomas A. D'Amico, Christopher D'Avella, Afshin Dowlati, Robert J. Downey, Martin Edelman, Charles Florsheim, Kathryn A. Gold, Jonathan W. Goldman, John C. Grecula, Christine Hann, Wade Iams, Puneeth Iyengar, Karen Kelly, Maya Khalil, Marianna Koczywas, Robert E. Merritt, Nisha Mohindra, Julian Molina, Cesar Moran, Saraswati Pokharel, Sonam Puri, Angel Qin, Chad Rusthoven, Jacob Sands, Rafael Santana-Davila, Michael Shafique, Saiama N. Waqar, Kristina M. Gregory, and Miranda Hughes

(antidiuretic hormone [ADH]) and adrenocorticotropic hormone (ACTH), which cause hyponatremia of malignancy (ie, syndrome of inappropriate ADH secretion [SIADH]) and Cushing syndrome, respectively. 29 , 30 In patients with SCLC, SIADH occurs more frequently