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6 "ACTH syndrome"
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Thyroid
Medullary Thyroid Carcinoma with Ectopic Adrenocorticotropic Hormone Syndrome
Hong Seok Choi, Min Joo Kim, Chae Ho Moon, Jong Ho Yoon, Ha Ra Ku, Geon Wook Kang, Im Il Na, Seung-Sook Lee, Byung-Chul Lee, Young Joo Park, Hong Il Kim, Yun Hyi Ku
Endocrinol Metab. 2014;29(1):96-100.   Published online March 14, 2014
DOI: https://doi.org/10.3803/EnM.2014.29.1.96
  • 4,760 View
  • 43 Download
  • 5 Web of Science
  • 6 Crossref
AbstractAbstract PDFPubReader   

Ectopic adrenocorticotropic hormone (ACTH) syndrome is caused most frequently by a bronchial carcinoid tumor or by small cell lung cancer. Medullary thyroid carcinoma (MTC) is a rare etiology of ectopic ACTH syndrome. We describe a case of Cushing syndrome due to ectopic ACTH production from MTC in a 48-year-old male. He was diagnosed with MTC 14 years ago and underwent total thyroidectomy, cervical lymph node dissection and a series of metastasectomies. MTC was confirmed by the pathological examination of the thyroid and metastatic mediastinal lymph node tissues. Two years after his last surgery, he developed Cushingoid features, such as moon face and central obesity, accompanied by uncontrolled hypertension and new-onset diabetes. The laboratory results were compatible with ectopic ACTH syndrome. A bilateral adrenalectomy improved the clinical and laboratory findings that were associated with Cushing syndrome. This is the first confirmed case of ectopic ACTH syndrome caused by MTC in Korea.

Citations

Citations to this article as recorded by  
  • Medullary thyroid carcinoma with ACHT-dependent Cushing's syndrome: Therapeutic possibilities
    Dušan Ilić, Sanja Ognjanović, Bojana Popović, Valentina Elezović-Kovačević, Milica Opalić-Palibrk, Lena Radić, Katarina Krstić, Đuro Macut
    Medicinski glasnik Specijalne bolnice za bolesti štitaste žlezde i bolesti metabolizma.2024; 29(93): 46.     CrossRef
  • Endocrine Perspective of Cutaneous Lichen Amyloidosis: RET-C634 Pathogenic Variant in Multiple Endocrine Neoplasia Type 2
    Alexandru-Florin Florescu, Oana-Claudia Sima, Claudiu Nistor, Mihai-Lucian Ciobica, Mihai Costachescu, Mihaela Stanciu, Denisa Tanasescu, Florina Ligia Popa, Mara Carsote
    Clinics and Practice.2024; 14(6): 2284.     CrossRef
  • Medullary Thyroid Cancer with Ectopic Cushing's Syndrome: A Case Report and Systematic Review of Detailed Cases from the Literature
    Andrea Corsello, Vittoria Ramunno, Pietro Locantore, Giovanni Pacini, Esther Diana Rossi, Francesco Torino, Alfredo Pontecorvi, Carmela De Crea, Rosa Maria Paragliola, Marco Raffaelli, Salvatore Maria Corsello
    Thyroid.2022; 32(11): 1281.     CrossRef
  • Mucosal Neuroma Cues for Endocrine Emergency Treatment
    Gyu Gang Choi, Hwan Jin Lee, Hyo Jin Han, Young Beom Jeong, Heung Bum Lee, Ji Hyun Park
    Endocrinology and Metabolism.2021; 36(6): 1312.     CrossRef
  • Articles in 'Endocrinology and Metabolism' in 2014
    Won-Young Lee
    Endocrinology and Metabolism.2015; 30(1): 47.     CrossRef
  • Rapid response of hypercortisolism to vandetanib treatment in a patient with advanced medullary thyroid cancer and ectopic Cushing syndrome
    Fabián Pitoia, Fernanda Bueno, Angélica Schmidt, Sabrina Lucas, Graciela Cross
    Archives of Endocrinology and Metabolism.2015; 59(4): 343.     CrossRef
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Ectopic ACTH Syndrome with Bilateral Pheochromocytoma in Multiple Endocrine Neoplasia Type 2A.
Ji Mi Moon, Yoon Jung Kim, Young Jin Seo, Hye Yoon Choi, Joo Hyong Kim, Ju Ri Park, Yun Jeong Lee, Hee Young Kim, Sin Gon Kim, Dong Seop Choi
J Korean Endocr Soc. 2009;24(4):265-271.   Published online December 1, 2009
DOI: https://doi.org/10.3803/jkes.2009.24.4.265
  • 2,564 View
  • 34 Download
  • 4 Crossref
AbstractAbstract PDF
Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant syndrome characterized by the presence of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN 2A arises due to a germline missense mutation of the RET proto-oncogene. Specific RET mutation analysis has revolutionized the diagnosis and therapy of this disorder, and early thyroidectomy may have lowered the morbidity and mortality associated with these diseases. Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of ACTH outside of the pituitary gland; the most common causes are malignancies, but rarely adrenal pheochromocytoma may be the cause.

Citations

Citations to this article as recorded by  
  • Pheochromocytoma With High Adrenocorticotropic Hormone Production Capacity Without Pigmentation and Cushingoid Symptoms: A Case Report With a Literature Review
    Gen Mizutani, Masashi Isshiki, Eisuke Shimizu, Daigo Saito, Akira Shimada
    Cureus.2024;[Epub]     CrossRef
  • Ectopic ACTH- and/or CRH-Producing Pheochromocytomas
    Patrick F Elliott, Thomas Berhane, Oskar Ragnarsson, Henrik Falhammar
    The Journal of Clinical Endocrinology & Metabolism.2021; 106(2): 598.     CrossRef
  • Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature
    Jenan N Gabi, Maali M Milhem, Yara E Tovar, Emhemmid S Karem, Alaa Y Gabi, Rodhan A Khthir
    Journal of the Endocrine Society.2018; 2(7): 621.     CrossRef
  • Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma presented as Cushing syndrome and complicated by invasive aspergillosis
    Jae Ho Cho, Da Eun Jeong, Jae Young Lee, Jong Geol Jang, Jun Sung Moon, Mi Jin Kim, Ji Sung Yoon, Kyu Chang Won, Hyoung Woo Lee
    Yeungnam University Journal of Medicine.2015; 32(2): 132.     CrossRef
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A Case of Pneumocystis Carinii Pneumonia and Cutaneous Nocardiosis Associated with Ectopic ACTH Syndrome.
Hee Sun Jung, Seung Hwan Lee, Hee Yeon Kim, Eun Ho Chu, Kang Woo Lee, Mi Ja Kang, Ji Hyun Kim, Hyuk Sang Kwon, Kun Ho Yoon, Bong Yun Cha, Ho Young Son
J Korean Endocr Soc. 2008;23(1):44-50.   Published online February 1, 2008
DOI: https://doi.org/10.3803/jkes.2008.23.1.44
  • 2,025 View
  • 19 Download
AbstractAbstract PDF
Cushing's syndrome is characterized by hypercortisolism and impairment of cell-mediated immune function, increasing the risk of opportunistic infection, as occurs in other immunocompromised groups such as organ transplant recipients, patients with lymphoreticular malignancy, or acquired immunodeficiency syndrome. We report a case of a 68-year-old woman who was affected with pneumocystis carinii pneumonia (PCP) 8 years ago and cutaneous nocardiosis this year. The patient was diagnosed with ectopic ACTH syndrome 8 years ago, but no ectopic ACTH secreting focus was found in radiologic imaging studies. She recovered from PCP and cutaneous nocardiosis after antibiotic (trimethoprim-sulfamethoxazole) administration. After the infection resolved, she underwent laparoscopic bilateral adrenalectomy and was not longer hypercortisolemic.
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A Case of Ectopic ACTH Syndrome Associated with Small Cell Lung Cancer Presented with Hypokalemia.
Hong Jun Yang, Hea Jung Sung, Ji Eun Kim, Hyo Jin Lee, Jin Min Park, Chan Kwon Park, Eun Suk Roh, Jae Hyung Cho, Seung Hyun Ko, Ki Ho Song, Yu Bai Ahn
J Korean Endocr Soc. 2007;22(5):359-364.   Published online October 1, 2007
DOI: https://doi.org/10.3803/jkes.2007.22.5.359
  • 2,475 View
  • 29 Download
  • 4 Crossref
AbstractAbstract PDF
We report a case of a 73-year-old female patient who was diagnosed with ectopic ACTH syndrome caused by small cell lung cancer. We initially presumed that the patient was in a state of mineralocorticoid excess, because she had hypertension and hypokalemic alkalosis. This was however excluded because her plasma renin activity was not suppressed and her plasma aldosterone/plasma renin activity ratio was below 25. Moreover, her 24 hour urine free cortisol level was elevated and her serum cortisol levels after a low dose dexamethasone suppression test, were not suppressed. Furthermore, her basal plasma ACTH and serum cortisol levels increased and her serum cortisol level after a high dose dexamethasone suppression test was not suppressed. We performed studies to identify the source of ectopic ACTH syndrome and found a 3 cm-sized mass in the patient's right lower lobe of her lung, which was eventually diagnosed as small cell lung cancer following a bronchoscopic biopsy. In conclusion, Cushing's syndrome, and in particular ectopic ACTH syndrome, must be considered in the differential diagnosis of mineralocorticoid-induced hypertension. The excessive cortisol saturates the 11beta-hydroxysteroid dehydrogenase type 2 (11beta-HSD2) activity, which in turn, inactivates the conversion of cortisol to cortisone in the renal tubules. Moreover, excessive cortisol causes binding to the mineralocorticoid receptors, causing mineralocorticoid hypertension, characterized by severe hypercortisolism.

Citations

Citations to this article as recorded by  
  • Emergencia hipertensiva como debut de síndrome de Cushing paraneoplásico
    E. Rubio González, M. de Valdenebro Recio, M.I. Galán Fernández
    Hipertensión y Riesgo Vascular.2024; 41(2): 135.     CrossRef
  • Management of small cell lung cancer complicated with paraneoplastic Cushing’s syndrome: a systematic literature review
    Yanlong Li, Caiyu Li, Xiangjun Qi, Ling Yu, Lizhu Lin
    Frontiers in Endocrinology.2023;[Epub]     CrossRef
  • Ectopic Cushing Syndrome in Adenocarcinoma of the Lung: Case Report and Literature Review
    Rana Al-Zakhari, Safa Aljammali, Basma Ataallah, Svetoslav Bardarov, Philip Otterbeck
    Cureus.2021;[Epub]     CrossRef
  • A Case of Ectopic Adrenocorticotropic Hormone Syndrome in Small Cell Lung Cancer
    Chaiho Jeong, Jinhee Lee, Seongyul Ryu, Hwa Young Lee, Ah Young Shin, Ju Sang Kim, Joong Hyun Ahn, Hye Seon Kang
    Tuberculosis and Respiratory Diseases.2015; 78(4): 436.     CrossRef
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2 Cases of Ectopic ACTH Syndrome due to Thymic Neuroendocrine Carcinoma Associated with Different Clinical Manifestations.
Yu Jeong Park, Moon Kyu Lee, Jong Ryeal Hahm, Jung Ho Do, Sang Taek Heo, Ju Sung Kim, Yong Ki Min, Myung Shik Lee, Kwang Won Kim, Joung Ho Han
J Korean Endocr Soc. 2001;16(1):115-122.   Published online February 1, 2001
  • 1,185 View
  • 17 Download
AbstractAbstract PDF
Primary neuroendocrine carcinoma of the thymus (formerly known as thymic carcinoids) are unusual tumors that account for 5% of all anterior mediastinal neoplasms. Thymic neuroendocrine carcinomas exhibit a much more aggressive behavior than those originating from other foregut locations and are associated with the ectopic ACTH syndrome in 38% of cases. Patients having a thymic neuroendocrine carcinoma usually present with symptoms such as chest tightness, dyspnea, a palpable neck mass and sometimes a weight gain. We report on 2 patients with thymic neuroendocrine carcinoma who showed different clinical manifestations each other. One presented with a typical. Cushingoid appearance and the other had symptoms of fatigue and weight loss which is a common symptom of malignant tumors.
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A Case of Ectopic ACTH Syndrome Caused by Thymic Carcinoid Tumor and Localized by PET.
Jong Chul Won, Sung Kwan Hong, Hyun Ju Lee, Sang Ook Kim, Yun Hee Jeong, Dae Hyuk Moon, Dong Kwan Kim, Kyung Yub Kong, Jae Sung Lee, Ki Soo Kim
J Korean Endocr Soc. 1999;14(4):771-778.   Published online January 1, 2001
  • 1,043 View
  • 17 Download
AbstractAbstract PDF
Ectopic ACTH syndrome is frequently caused by lung cancer and uncommonly by other tumors such as thymic carcinoid. For its treatment, early diagnosis and complete resection is irresponsible, but some cases are remained unlocalized in spite of all diagnostic modalities. Here we report a case of ectopic ACTH syndrome which was localized by PET but could not be localized by conventional technique. A tumor at thymic area was ACTH secreting thymic carcinoid which was operated but couldnt resect completly. Glucocorticoid hypersecretion was persisted with chemotherapy, radiotherapy, and ketoconazole treatment. Patient died of sepsis after 12 months of diagnosis.
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