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The patient with acute endocrine problems
Published in Peate Ian, Dutton Helen, Acute Nursing Care, 2020
A pheochromocytoma is a (very rare) tumour of the adrenal medulla, whereby abnormally high levels of adrenaline and noradrenaline are secreted into the systemic circulation. The release of these catecholamines is intermittent, but results in headaches, tachycardia, hyperglycaemia, blurred vision, bowel disturbances and very severe hypertension. Following blood pressure stabilisation (usually with alpha- and beta-blockers), a pheochromocytoma will require surgical removal.
Endocrine and Neuroendocrine Tumors
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Natasha Shrikrishnapalasuriyar, P.N. Plowman, Márta Korbonits, Ashley B. Grossman
The clinical manifestations of pheochromocytomas usually result from catecholamine secretion, which may occur at rest or be precipitated by physical activity, emotion, certain drugs such as hydrocortisone, and tyramine-rich food. Hypertension, either paroxysmal or sustained (in 50% of patients) is the most common manifestation, found in about 90% of patients. Episodes of hypotension, either postural or alternating with hypertension, can also occur. Headache is common in about 80–90% of cases and truncal sweating can be seen in 60–70%. The triad of headache, sweating, and palpitations is suggestive of a diagnosis of pheochromocytoma, the major differential diagnosis being anxiety or panic attacks.62
Hereditary Pheochromocytoma and Paraganglioma Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Molecular identification of SDHA, SDHB, SDHC, SDHD, and SDHAF2 pathogenic variants further confirms the diagnosis of PPGL in the adrenal as well as extra-adrenal tissues (Table 58.2). Genetic testing is recommended for (i) patients with a personal or family history of clinical features suggestive of hereditary PPGL syndrome, (ii) patients with bilateral or multifocal tumors, (iii) patients with sympathetic or malignant extra-adrenal paragangliomas, (iv) patients diagnosed before age 40 years, and (v) patients between 40–50 years of age with a unilateral pheochromocytoma and no personal or family history suggestive of hereditary disease, but not for patients >50 years [2,23,24].
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 is a tumor originated from adrenal medulla, sympathetic ganglion or other chromaffin tissues. Among them, tumors originated from sympathetic ganglion, parasympathetic ganglion or other parts of chromaffin tissue, also known as paraganglioma or extraadrenal pheochromocytoma. Pheochromocytoma and paraganglioma (ppgl) occur in adrenal medulla and extraadrenal. At present, adrenal pheochromocytoma and extraadrenal pheochromocytoma (paraganglioma) are collectively referred to as pheochromocytoma. It can lead to sustained or paroxysm hypertension, multiple organ dysfunction and metabolic disorders due to sustained or intermittent releasing of catecholamine (1). pheochromocytoma is mainly found in adrenal gland, also in retroperitoneal paraaortic tissue. Therefore, it indicates that the occurrence of pheochromocytoma with acute non-cardiac pulmonary edema is relatively rare. Although previous studies have reported the clinic characteristics of patients with pheochromocytoma, few studies have reported the clinic characteristics of patients with pheochromocytoma and acute non-cardiac pulmonary edema. In the present study, we reported a case of pheochromocytoma combined with acute non-cardiac pulmonary edema and reviewed literatures in an attempt to improve the recognition of the disease in order to strive for early diagnosis and therapy.
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.