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.
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.
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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
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.
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 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.
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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