JURNAL PHEOCHROMOCYTOMA PDF

Pheochromocytomas are rare catecholamine producing neuroendocrine tumors. The incidence of these tumors is estimated to affect 0. We describe a case of an year-old male who presented with cycling episodes of severe hypertension and hypotension after an elective cardiac catheterization. Of note, this is the second eldest patient known to be published to date with a pheochromocytoma. Those arising from the extra-adrenal neural ganglia are called paragangliomas. Due to a large production of catecholamines, such as epinephrine, norepinephrine, and dopamine, patients can present with the classic triad of symptoms of sweating, headaches, and tachycardia [ 3 ].

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Pheochromocytomas are rare catecholamine producing neuroendocrine tumors. The incidence of these tumors is estimated to affect 0. We describe a case of an year-old male who presented with cycling episodes of severe hypertension and hypotension after an elective cardiac catheterization.

Of note, this is the second eldest patient known to be published to date with a pheochromocytoma. Those arising from the extra-adrenal neural ganglia are called paragangliomas. Due to a large production of catecholamines, such as epinephrine, norepinephrine, and dopamine, patients can present with the classic triad of symptoms of sweating, headaches, and tachycardia [ 3 ].

However, it is important to note most patients do not present with the classic triad or during a pheochromocytoma spell [ 5 , 6 ]. The diagnosis of pheochromocytoma is made by biochemical testing in urine and serum for metanephrine MN and normetanephrine NMN end products of catecholamine metabolism, followed by imaging studies such as a computerized tomography CT scan.

In this report, we describe a case of an year-old male diagnosed with a pheochromocytoma and cycling episodes of hypertension and hypotension. We present the case of an year-old male with past medical history of hypertension, benign prostatic hyperplasia, former alcohol and cocaine abuse, chronic kidney disease stage 3A, severe malnutrition, and frequent falls who presented from a nursing home for elective left heart catheterization.

During the catheterization, the patient developed hypertensive emergency with flash pulmonary edema and the procedure had to be aborted. He was admitted to the Cardiac Critical Care Unit for hypertensive emergency and was started on antihypertensive drips. Urine drug screen was negative.

He was weaned off the drips after 36 hours. These blood pressure readings were measured in four extremities with a manual manometer and confirmed with an arterial line. During the rapid shifts in blood pressure the patient would develop blank staring episodes and nonresponsiveness that would last approximately 30 seconds to 1 minute.

An immediate head CT showed no acute ischemic changes, hemorrhagic stroke, or cerebral edema. Electroencephalogram showed generalized slowing consistent with moderate metabolic encephalopathy. In addition, the patient developed acute kidney injury with maximum creatinine of 2. Because of the labile blood pressures, the patient was on no antihypertensive medications, including alpha or beta blockers or calcium channel antagonists, at the time when then the serum and urine metanephrines were drawn.

His UDS was negative for cocaine and the patient was not on tricyclic antidepressant or other drugs that can cause false positive elevations of plasma and urinary catecholamines or metanephrines [ 7 ]. The findings confirmed the diagnosis of pheochromocytoma. The patient was started on prazosin and, however, developed worsening hypotension again, even while receiving aggressive fluid resuscitation. After the patient was euvolemic, the prazosin was able to be titrated up to goal with stabilization of his blood pressure.

Due to his advanced age, severe malnutrition, no family support, worsening mental status, and comorbidities he was deemed not an appropriate surgical candidate. Patient was discharged under hospice care. In this case report we present the second eldest patient to date, based on literature review, to be diagnosed with a pheochromocytoma at age 84 years old.

The eldest patient to date was an year-old male from Buffet et al. Previously, Chenguan et al. He was initially started on intravenous nitroglycerin that was changed to nicardipine for better blood pressure control. Per the Endocrine Clinical Practice Guidelines, patients with functional pheochromocytomas and paragangliomas should receive perioperative blockade in addition to a high-sodium diet and fluid intake prior to the surgery [ 10 ].

Perioperative blockade consists of alpha-adrenergic receptor blockers as the first-choice drug class to minimize perioperative complications and calcium channel blockers are the most often used add on drug to control blood pressure. Beta-adrenergic receptor blockers are indicated to control tachycardia, but only after alpha-adrenergic receptor blockade has been achieved. The intravenous nicardipine was discontinued. From the standpoint of the medical team, this labile blood pressure was extremely challenging to manage.

Each time the team tried to intervene on the elevated blood pressure his blood pressure suddenly plummeted and vice versa. There was concern which extreme was more harmful to the patient and was there a balance in treatment.

There are multiple cases describing such cyclic episodes of hypotension and hypertension with pheochromocytomas [ 11 — 13 ]. A review by Kobal et al. There are a few proposed mechanisms for this cyclic blood pressure; however the exact mechanism is uncertain.

One proposed mechanism is based on autoregulation by baroreceptors. A surge in catecholamines, primarily epinephrine, would cause vasoconstriction and decreased blood volume leading to decreased cardiac output triggering further release of catecholamines. The baroreceptors would then sense the vasoconstriction causing negative feedback of the sympathetic and parasympathetic nervous system resulting in hypotension [ 15 ]. Another proposed mechanism is that posture affects tumor secretions.

Oishi et al. There was discussion which alpha-blockade to initiate. The patient was on prazosin, a short acting alpha adrenergic blocker, for BPH with blood pressure control prior to admission. Phenoxybenzamine, an irreversible, long acting, and nonselective alpha receptor antagonist, is the preferred drug for preoperative preparation to control blood pressure. Doxazosin, a selective alphaadrenergic blocker, was another option [ 10 ].

However, with the labile pressures and concern for hypotension, there was concern for using a long acting antagonist and prazosin was initiated because of its ability to be up titrated. The only curative modality for pheochromocytoma is tumor excision, with a laparoscopic approach being the procedure of choice for patients with a solitary intra-adrenal pheochromocytomas without malignant radiologic features [ 10 ]. These factors also contributed to the decision to not proceed with genetic evaluation, as the Endocrine Clinical Practice Guidelines recommend all patients be engaged in shared decision-making for genetic testing [ 10 ].

In conclusion, pheochromocytoma can be seen in elderly patients. It is important to note that alpha-adrenergic antagonist medications may mask an insidious pheochromocytoma unless provoked by a stressor, like a surgery. The composition of the tumor secretions, epinephrine versus norepinephrine versus dopamine, can lead to varying presentations of patients that should trigger pheochromocytoma workup.

Lastly, patients with pheochromocytomas that present with rapid cyclic fluctuations of hypertension and hypotension may benefit from fluid repletion in addition to an alpha-adrenergic antagonist. The case report findings, including case demographics, imaging, vital signs, and lab results, used to support the findings of this report are available from the corresponding author upon request.

The authors declare that there is no conflict of interest regarding the publication of this paper. DiMaria et al. This is an open access article distributed under the Creative Commons Attribution License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Journal overview. Christina N. DiMaria , 1 Lorena I. Rasquin , 1 and Wikien A. Academic Editor: Takeshi Usui. Received 08 May Accepted 24 Jun Published 07 Jul Abstract Pheochromocytomas are rare catecholamine producing neuroendocrine tumors. Introduction Pheochromocytomas are rare catecholamine producing neuroendocrine tumors.

Case Presentation We present the case of an year-old male with past medical history of hypertension, benign prostatic hyperplasia, former alcohol and cocaine abuse, chronic kidney disease stage 3A, severe malnutrition, and frequent falls who presented from a nursing home for elective left heart catheterization. Discussion In this case report we present the second eldest patient to date, based on literature review, to be diagnosed with a pheochromocytoma at age 84 years old.

Conclusion In conclusion, pheochromocytoma can be seen in elderly patients. Data Availability The case report findings, including case demographics, imaging, vital signs, and lab results, used to support the findings of this report are available from the corresponding author upon request. Conflicts of Interest The authors declare that there is no conflict of interest regarding the publication of this paper. References C. Beard, S.

Sheps, L. Kurland, J. Carney, and J. View at: Google Scholar M. Guerrero, J. Schreinemakers, M. Vriens, I. Suh, J. Hwang, and W. View at: Google Scholar P. Stein and H. Young and D. View at: Google Scholar Y. Kudva, W. Young, G. Thompson et al. Baguet, L. Hammer, T. Mazzuco et al. Feingold, B.

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Pheochromocytomas and secreting paragangliomas

Pheochromocytoma is a rare tumor arising from chromaffin cells in adrenal medulla or other paraganglia in the body, which may be associated with many genetic syndromes and mutation. The role of endocrinologist is in biochemical diagnosis of suspected cases; its anatomic and functional localization with the help of imaging like CT, MRI, and nuclear scanning; preoperative control of hypertension; and postoperative follow-up of cases that have undergone surgical resection. Familial and genetic screening of cases and their family is important to detect occult cases. Endocrinologist will also play a role in cases with malignant pheochromocytoma in assessment of metastasis, control, chemoradiotherapy, and follow-up. Pheochromocytoma is a rare tumor arising from chromaffin cells in adrenal medulla or other paraganglia in the body.

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The Highs and Lows of an Unknown Pheochromocytoma in an Elderly Patient

Metrics details. Catecholamine-producing tumors may arise in the adrenal medulla pheochromocytomas or in extraadrenal chromaffin cells secreting paragangliomas. Their prevalence is about 0. These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease.

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