Journal of Yeungnam Medical Science

Review article

Screening and treatment of endocrine hypertension focusing on adrenal gland disorders: a narrative review: Seung Min Chung; J Yeungnam Med Sci. 2024;41(4):269-278. Published online September 19, 2024; DOI: https://doi.org/10.12701/jyms.2024.00752

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Abstract PDF: Most cases of high blood pressure have no identifiable cause, termed essential hypertension; however, in approximately 15% of cases, hypertension occurs due to secondary causes. Primary aldosteronism (PA) and pheochromocytoma and paraganglioma (PPGL) are representative endocrine hypertensive diseases. The differentiation of endocrine hypertension provides an opportunity to cure and prevent target organ damage. PA is the most common cause of secondary hypertension, which significantly increases the risk of cardiovascular disease compared to essential hypertension; thus, patients with clinical manifestations suggestive of secondary hypertension should be screened for PA. PPGL are rare but can be fatal when misdiagnosed. PPGL are the most common hereditary endocrine tumors; therefore, genetic testing using next-generation sequencing panels is recommended. Herein, we aimed to summarize the characteristic clinical symptoms of PA and PPGL and when and how diagnostic tests and treatment strategies should be performed.

Case reports

Ruptured triple hormone-secreting adrenal cortical carcinoma with hyperaldosteronism, hypercortisolism, and elevated normetanephrine: a case report: Sin Yung Woo, Seongji Park, Kun Young Kwon, Dong-Mee Lim, Keun-Young Park, Jong-Dai Kim; J Yeungnam Med Sci. 2024;41(4):306-311. Published online September 6, 2024; DOI: https://doi.org/10.12701/jyms.2024.00626

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Abstract PDF: We report a case of a ruptured triple hormone-secreting adrenal mass with hyperaldosteronism, hypercortisolism, and elevated normetanephrine levels, diagnosed as adrenal cortical carcinoma (ACC) by histology. A 53-year-old male patient who initially presented with abdominal pain was referred to our hospital for angiocoagulation of an adrenal mass rupture. Abdominal computed tomography revealed a heterogeneous 19×11×15 cm right adrenal mass with invasion into the right lobe of the liver, inferior vena cava, retrocaval lymph nodes, and aortocaval lymph nodes. Angiocoagulation was performed. Laboratory evaluation revealed excess cortisol via a positive 1-mg overnight dexamethasone suppression test, primary hyperaldosteronism via a positive saline infusion test, and plasma normetanephrine levels three times higher than normal. An adrenal mass biopsy was performed for pathological confirmation to commence palliative chemotherapy because surgical management was not deemed appropriate considering the extent of the tumor. Pathological examination revealed stage T4N1M1 ACC. The patient started the first cycle of adjuvant mitotane therapy along with adjuvant treatment with doxorubicin, cisplatin, and etoposide, and was discharged. Clinical cases of dual cortisol- and aldosterone-secreting ACCs or ACCs presenting as pheochromocytomas have occasionally been reported; however, both are rare. Moreover, to the best of our knowledge, a triple hormone-secreting ACC has not yet been reported. Here, we report a rare case and its management. This case report underscores the necessity of performing comprehensive clinical and biochemical hormone evaluations in patients with adrenal masses because ACC can present with multiple hormone elevations.

Interleukin-6-producing paraganglioma as a rare cause of systemic inflammatory response syndrome: a case report: Yin Young Lee, Seung Min Chung; J Yeungnam Med Sci. 2023;40(4):435-441. Published online March 7, 2023; DOI: https://doi.org/10.12701/jyms.2022.00766

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1 Web of Science
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Abstract PDF

Pheochromocytomas and paragangliomas (PPGLs) may secrete hormones or bioactive neuropeptides such as interleukin-6 (IL-6), which can mask the clinical manifestations of catecholamine hypersecretion. We report the case of a patient with delayed diagnosis of paraganglioma due to the development of IL-6-mediated systemic inflammatory response syndrome (SIRS). A 58-year-old woman presented with dyspnea and flank pain accompanied by SIRS and acute cardiac, kidney, and liver injuries. A left paravertebral mass was incidentally observed on abdominal computed tomography (CT). Biochemical tests revealed increased 24-hour urinary metanephrine (2.12 mg/day), plasma norepinephrine (1,588 pg/mL), plasma normetanephrine (2.27 nmol/L), and IL-6 (16.5 pg/mL) levels. 18F-fluorodeoxyglucose (FDG) positron emission tomography/CT showed increased uptake of FDG in the left paravertebral mass without metastases. The patient was finally diagnosed with functional paraganglioma crisis. The precipitating factor was unclear, but phendimetrazine tartrate, a norepinephrine-dopamine release drug that the patient regularly took, might have stimulated the paraganglioma. The patient’s body temperature and blood pressure were well controlled after alpha-blocker administration, and the retroperitoneal mass was surgically resected successfully. After surgery, the patient’s inflammatory, cardiac, renal, and hepatic biomarkers and catecholamine levels improved. In conclusion, our report emphasizes the importance of IL-6-producing PPGLs in the differential diagnosis of SIRS.

Citations

Citations to this article as recorded by

Screening and treatment of endocrine hypertension focusing on adrenal gland disorders: a narrative review
Seung Min Chung
Journal of Yeungnam Medical Science.2024; 41(4): 269. CrossRef

Catastrophic catecholamine-induced cardiomyopathy rescued by extracorporeal membrane oxygenation in recurrent malignant pheochromocytoma: Daniel Min; Yeungnam Univ J Med. 2019;36(3):254-259. Published online May 22, 2019; DOI: https://doi.org/10.12701/yujm.2019.00213

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Abstract PDF

Pheochromocytoma (PCC) is a rare catecholamine-producing tumor with the incidence in hypertension of 0.1-0.6%. PCC crisis is an endocrine emergency that can lead to hemodynamic disturbance and organ failure such as catecholamine-induced cardiomyopathy. The circulatory collapse caused by it often requires mechanical support. The author reports an unusual case in which a patient who previously underwent surgery for malignant PCC developed catecholamine-induced cardiomyopathy, and successfully recovered using extracorporeal membrane oxygenation.

Citations

Citations to this article as recorded by

Successful Use of Extracorporeal Life Support and Continuous Renal Replacement Therapy in the Treatment of Cardiogenic Shock Induced by Tumor Lysis Syndrome in a Pediatric Patient With Lymphoma: A Case Report
Zhulin Wang, Fang Zhang, Long Xiang, Yinyu Yang, Wei Wang, Biru Li, Hong Ren
Frontiers in Medicine.2022;[Epub] CrossRef
Clinical characteristics and outcomes of pheochromocytoma crisis: a literature review of 200 cases
Y. Ando, Y. Ono, A. Sano, N. Fujita, S. Ono, Y. Tanaka
Journal of Endocrinological Investigation.2022; 45(12): 2313. CrossRef
Catecholamine-induced cardiomyopathy: an endocrinologist’s perspective
Aman Kumar, Joseph M Pappachan, Cornelius James Fernandez
Reviews in Cardiovascular Medicine.2021;[Epub] CrossRef

Case Reports

Pheochromocytoma-induced cardiogenic shock successfully treated by extracorporeal circulation: Min Young Lee, Sang Bae Lee, Hyun Seo Cha, Ji Hong You, Eui Young Choi, Jong Suk Park; Yeungnam Univ J Med. 2017;34(2):285-289. Published online December 31, 2017; DOI: https://doi.org/10.12701/yujm.2017.34.2.285

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Abstract PDF: Pheochromocytoma can present with various symptoms including cardiogenic shock and cardiac arrest. Particularly, in cases of cardiogenic shock of unknown origin, pheochromocytoma should be considered. A 20-year-old woman without any medical history visited our emergency department due to nausea, vomiting, headache, and chest pain. Echocardiography revealed severe left ventricular dysfunction. Mechanical ventilation and veno-arterial extracorporeal membrane oxygenation (ECMO) were implemented owing to her unstable vital signs. For unstable vital sign and cardiogenic shock in a young woman without any previous medical history, pheochromocytoma was considered and diagnosed based on elevated levels of catecholamine derivatives in a 24-hour urine sample. Cardiac function recovered and ECMO was discontinued on the 5th day of hospitalization. She later underwent an elective adrenalectomy and no recurrence was found during the follow-up period. We reported a case of pheochromocytoma which was presented with cardiogenic shock in a young woman with no concomitant disease, and successfully treated with ECMO followed by an elective adrenalectomy.

Huge pheochromocytoma presented with paraaortic lymph node and spine metastases: Yeon Won Park, Han Ju Moon, Jung Suk Han, Ji Min Han, Jong Wook Park, Yun Hyi Ku; Yeungnam Univ J Med. 2017;34(2):247-253. Published online December 31, 2017; DOI: https://doi.org/10.12701/yujm.2017.34.2.247

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Abstract PDF: Approximately 10–15% of pheochromocytomas are malignant. There are insufficient histologic criteria for the diagnosis of malignant pheochromocytoma. Thus, the term malignant pheochromocytoma is restricted to tumors with local invasion or distant metastases. We experienced a case of malignant pheochromocytoma recurred with spinal metastasis 4 years after the surgery for huge benign pheochromocytoma. A 68-year-old female was admitted for trunk and back pain. The patient had a history of surgery 4 years ago for a 10.0×9.5×7.5 cm sized benign pheochromocytoma at the left adrenal gland. A thoracolumbar magnetic resonance imaging showed a tumor in the 7th thoracic vertebral body and a 24-hour urinary norepinephrine increased, suggesting metastatic recurrence of malignant pheochromocytoma. After metastasectomy in the 7th thoracic vertebral body, urine catecholamine was normalized and pain also disappeared. However, a metastatic lesion was found in the paraaortic area on a follow-up abdominal computed tomography scan and an additional metastasectomy was performed. The pathology confirmed the diagnosis of metastatic pheochromocytoma in the paraaortic lymph nodes. She is supposed to be treated with adjuvant iodine 131-meta-iodobenzylguanidine therapy. In our experience, a close follow-up should be considered in patients who had a huge benign pheochromocytoma due to the possibility of malignant metastases.

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 Univ J Med. 2015;32(2):132-137. Published online December 31, 2015; DOI: https://doi.org/10.12701/yujm.2015.32.2.132

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Abstract PDF

Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma has been rarely reported, whereas only a few cases of Cushing syndrome accompanied by opportunistic infections have been reported. We experienced a patient with pheochromocytoma with ectopic Cushing syndrome complicated by invasive aspergillosis. A 35-year-old woman presented with typical Cushingoid features. Her basal plasma cortisol, ACTH, and 24-hour urine free cortisol levels were significantly high, and 24-hour urine metanephrine and catecholamine levels were slightly elevated. The endogeneous cortisol secretion was not suppressed by either low- or high-dose dexamethasone. Abdominal computed tomography (CT) revealed a heterogeneous enhancing mass measuring approximately 2.5 cm in size in the left adrenal gland. No definitive mass lesion was observed on sellar magnetic resonance imaging. On fluorine-18 fluorodeoxyglucose positron emission tomography/CT, a hypermetabolic nodule was observed in the left upper lung. Thus, we performed a percutaneous needle biopsy, which revealed inflammation, not malignancy. Thereafter, we performed a laparoscopic left adrenalectomy, and its pathologic finding was a pheochromocytoma with positive immunohistostaining for ACTH. After surgery, the biochemistry was normalized, but the clinical course was fatal despite intensive care because of the invasive aspergillosis that included the lungs, retina, and central nervous system.

Citations

Citations to this article as recorded by

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
Ectopic adrenocorticotrophic hormone syndrome (EAS) with phaeochromocytoma: a challenging endocrine case with a happy ending
Sharifah Faradila Wan Muhamad Hatta, Leoni Lekkakou, Ananth Viswananth, Harit Buch
BMJ Case Reports.2019; 12(8): e230636. 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

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