Pheochromocytoma
Demetrius Pertsemlidis, William B. Inabnet III, Michel Gagner in Endocrine Surgery, 2017
The debate over the need for expansion of the circulating blood volume in the preoperative phase is plagued by basic misconception. The notion that patients with pheochromocytoma are hypovolemic [41] is not valid. Measurements of plasma and red cell volumes in these patients have proven to be within the normal range [42], and in fact, pheochromocytoma patients are hypervolemic relative to the capacity of the constricted vascular system. Attempts to expand the circulating volume with prolonged preoperative adrenergic blockade do not prevent intraoperative cardiovascular crises and the hypotension that follows tumor removal [43]. The proposed 4- to 6-week delay for volume expansion serves only to increase the time period during which these patients are at continued risk from physical or emotional stress-induced paroxysmal crises. The surgical treatment of pheochromocytoma must be expeditious and should be performed immediately after diagnosis and localization. The appropriate timing of volume restoration is immediately after tumor removal.
Endocrine hypertension
Philip E. Harris, Pierre-Marc G. Bouloux in Endocrinology in Clinical Practice, 2014
The classic triad of symptoms in a patient with pheochromocytoma are episodic headache, sweating, and tachycardia.69 Only half have paroxysmal hypertension, and the rest can be normotensive (5%–15%) or present with apparent essential hypertension.70 Headache is the commonest clinical feature and can be found in up to 90% of patients.70 Other clinical features include palpitations, tremor, pallor, abdominal pain, chest pain, and anxiety. Broadly, symptoms and signs depend upon the location of the tumor as well as the relative amounts of catecholamine secreted. More rarely, other clinical features can be found due to cosecretion of other substances in addition to catecholamines. For example, ACTH cosecretion can lead to development of Cushingoid features and hypoglycemia can occur due to production of insulin-like growth factor-2.
Complications of Adrenal Gland Surgery
Stephen M. Cohn, Matthew O. Dolich in Complications in Surgery and Trauma, 2014
Pheochromocytoma is a tumor of the adrenal medulla or of other sympathetic ganglion cells of the peripheral nervous system. Approximately 10% of these tumors are malignant, 10% are bilateral, 10% are familial, and 10% are extra-adrenal. The symptoms caused by these tumors fall into three general groups. One-third of patients experience an episode of palpitations, diaphoresis, headache, and a “feeling of impending doom.” An additional one-third of patients exhibit normotension with transient symptomatic episodes in conjunction with episodic hypertension. The remaining one-third of these patients exhibit asymptomatic chronic hypertension, which is often misdiagnosed as essential hypertension. Diagnostic testing includes determination of plasma and urinary catecholamine levels, as well as urinary levels of catecholamine degradation products (metanephrines and vanillylmandelic acid). The location of the tumors is determined by abdominal computed tomography (CT) or magnetic resonance imaging (MRI) or by 131-iodine metaiodobenzylguanidine (131I-MIBG) scanning when necessary.
Clinical presentation and diagnostic evaluation of pheochromocytoma: case series and literature review
Published in Clinical and Experimental Hypertension, 2023
Panagiota Anyfanti, Κonstantinos Mastrogiannis, Αntonios Lazaridis, Κonstantinos Tasios, Despoina Vasilakou, Αnastasia Kyriazidou, Fotios Aroutsidis, Olga Pavlidou, Εleni Papoutsopoulou, Athina Tiritidou, Vasileios Kotsis, Αreti Triantafyllou, Ιoannis Zarifis, Stella Douma, Εugenia Gkaliagkousi
Pheochromocytomas are neuroendocrine tumors producing catecholamines that derive from the chromaffin cells and develop within the adrenal medulla and autonomic nerve ganglia. Neoplasms that develop from chromaffin tissue outside the adrenal gland are called extra-adrenal pheochromocytomas or paragangliomas. The same term is used to describe tumors deriving from head or neck parasympathetic tissue, which, usually, don’t produce catecholamines. Almost 80–85% of pheochromocytomas develop within the adrenal gland medulla and only 10–15% derive from extra-adrenal tissue. The tumor is rare, with an estimated incidence of 2–8 cases for every million people and prevalence ranging from 0.2–0.6% of the general hypertensive population (1,2). Mean age of diagnosis is 40–50 years, although it can occur from childhood to late in adulthood (3,4). In around one-third (25–33%) of cases, pheochromocytoma occurs in the context of familial syndromes such as multiple endocrine neoplasia syndromes (MEN 2A, MEN 2B), type I neurofibromatosis, von Hippel-Lindau disease, Sturge-Webber syndrome and other paraganglioma syndromes. In these cases, the diagnosis is usually made at a younger age, typically before the 4th decade of life (5).
Pheochromocytoma with Acute Non-cardiac Pulmonary Edema: A Report of One Case and the Review of Literature
Published in Cancer Investigation, 2021
Yuan Liu, Ning Wang, Shi Li, Ling Jiang, Chunfang Liu, Jian Xu, Huadong He
Pheochromocytoma originates from chromaffin cells in adrenal medulla, sympathetic ganglion or other parts. It causes clinical symptoms by continuously or intermittently releasing a large amount of catecholamines (epinephrine, norepinephrine and dopamine). The classic clinical manifestations are "headache, sweating and palpitation" triad (2,3). However, due to the different amount, proportion and release mode of catecholamines secreted by the tumor, the clinical manifestations of some pheochromocytoma patients are atypical and difficult to identify (4,5). The clinical manifestations of pheochromocytoma patients are often diversified, including myocardial infarction, myocarditis, abdominal pain, fever and other symptoms, which are very easy to cause misdiagnosis and missed diagnosis clinically. Some patients may even have pheochromocytoma crisis. Pheochromocytoma crisis refers to a series of serious clinical manifestations such as sudden hypertension or hypotension, palpitation, even shock and heart failure caused by acute hypercatecholaminemia caused by epinephrine and norepinephrine in short-term endocrine of pheochromocytoma patients. The main inducements are: (1) excessive emotional excitement; (2) External extrusion or sudden increase of intra-abdominal pressure; (3) Strenuous exercise. The clinical manifestations of a very small number of pheochromocytoma patients are pulmonary edema, which progresses rapidly and even endangers life (6). Clinical reports of acute pulmonary edema caused by pheochromocytoma are rare (7).
Pheochromocytoma and sinus node dysfunction
Published in Baylor University Medical Center Proceedings, 2019
Mary Lee, Dane Langsjeon, Srikala Devabhaktuni, Greg Olsovsky
Renal ultrasound revealed a 5.5-cm soft tissue lesion near the superior medial aspect of the right kidney without evidence of renal artery stenosis (Figure 1). Computed tomography of the abdomen showed a 3.7 × 4.0 × 5.5 cm right adrenal lesion that corresponded to renal ultrasound findings (Figure 2). Enhancement washout characteristics were not consistent with adenoma, so biochemical studies were obtained. Studies revealed elevated levels of serum metanephrine, 6.30 nmol/L (normal <0.50 nmol/L); serum normetanephrine, 16 nmol/L (normal <0.90 nmol/L); 24-hour urine norepinephrine, 298 µg/24 h (normal 16–71 µg/24 h); 24-hour urine epinephrine, 124 µg/24 h (normal 1–7 µg/24 h); and 24-hour urine metanephrine, 6365 mcg/24 h (normal 30–180 mcg/24 h), confirming the diagnosis of pheochromocytoma. She was referred for endocrine surgery, started on phenoxybenzamine, and underwent a successful uncomplicated laparoscopic adrenalectomy of the right adenoma. Pathology confirmed a benign tumor (pheochromocytoma of the adrenal gland, scaled score of 2) with clear surgical margins.
Related Knowledge Centers
- Adrenal Medulla
- Chromaffin Cell
- Perspiration
- Tachycardia
- Catecholamine
- Hypertension
- Paraganglioma
- Metanephrines
- Organ of Zuckerkandl
- Signs & Symptoms