Endocrinology and Metabolism

Case Reports

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

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Abstract 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.

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

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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

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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

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

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Abstract 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.

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

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Abstract 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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