Endocrine diseases and pregnancy
Hung N. Winn, Frank A. Chervenak, Roberto Romero in Clinical Maternal-Fetal Medicine Online, 2021
Pheochromocytoma is generally suspected based on the presence of maternal symptoms, hypertension, or identification of a family history of MEN, von Hippel–Lindau, or neurofibromatosis. Elevated plasma catecholamines and metanephrines are strikingly elevated and are reflected in 24-hour urinary samples for catecholamines (epinephrine and norepinephrine) and metanephrines (metanephrine and normetanephrine). Most surgically confirmed pheochromocytomas have been associated with 24-hour urinary secretion three or more times the upper limit of normal for one or more of these hormones (83). When clinical suspicion is heightened by the finding of one or more extremely elevated urinary catecholamines or metanephrines (epinephrine, norepinephrine, metanephrine, or normetanephrine), adrenal imaging with non-gadolinium-enhanced MRI is accomplished, to investigate whether there is a unilateral adrenal tumor. Characteristically, pheochromocytomas image very bright on T2-weighted MRI and may be very helpful in the pregnant patient, for whom imaging modality options are limited (84).
Clinical Considerations
Stephen W. Carmichael, Susan L. Stoddard in The Adrenal Medulla 1986 - 1988, 2017
Four laboratories assessed the value of a clonidine-suppression test in the diagnosis of pheochromocytoma. Karlberg, Hedman, Lennquist et al. (1986) concluded that the test is a safe and simple diagnostic procedure for verification or exclusion of pheochromocytomas, especially in patients with normal baseline catecholamine levels. Misleading results of a clonidine-suppression test were reported by Taylor, Mayes and Anton (1986); examples of a false-negative and false-positive result were presented. Mulinari, Zanella, Guerra et al. (1987) demonstrated that urinary metanephrine levels, when monitored by the clonidine-suppression test, are useful for the diagnosis of pheochromocytoma. This combination permits the differentiation of patients with tumor from those with essential hypertension. Gross, Shapiro, Sisson et al. (1987) found that use of the clonidine-suppression test to separate normal from abnormal adrenal medulla function was only possible in those patients with abnormal adrenal anatomy and in whom adrenal medulla dysfunction could be documented by other diagnostic means. This suggests that the clonidine test alone may not be useful to identify the earliest stages of adrenal medullary dysfunction.
Surgical Endocrine Emergencies
Stephen M. Cohn, Matthew O. Dolich, Kenji Inaba in Acute Care Surgery and Trauma, 2016
Evaluation begins with measurement of plasma or urinary metanephrines in symptomatic patients, or those with an adrenal mass. The diagnosis is made biochemically. Metanephrine levels are typically elevated fourfold [39,40]. CT or MRI is the initial imaging modalities of choice to localize tumors. Pheochromocytomas will often appear hyperintense on a T2-weighted MRI, and hypointense on T1-weighted images [41]. On CT, pheochromocytomas may be homogenous, heterogeneous or cystic, and typically have attenuation greater than 10 Hounsfield units (HU) on noncontrast CT. Functional imaging with 131I-radiolabeled MIBG may be performed, in the setting of a negative CT or MRI, or in cases of suspected bilateral tumors or metastatic disease [42]. Fdg-Pet CT may also be used to assess extent of disease in the setting of known malignant pheochromocytoma, or when 131I MIBG is negative.
An adrenal cortical adenoma with neuroendocrine-type granules mimicking pheochromocytoma
Published in Ultrastructural Pathology, 2023
Leonardo Rossi, Carlo Enrico Ambrosini, Liborio Torregrossa, Maria Margherita de Santi, Raffaella Guazzo, Tommaso Simoncini, Alessandra Bacca, Benard Gjeloshi, Francesco Pignatelli, Mattia Iachini, Elisa Loguercio, Chiara Becucci, Gabriele Materazzi
A 59-year-old woman with a medical history of hypertension, hypertensive cardiopathy, severe obesity and Obstructive Sleep Apnea Syndrome (OSAS) was under follow-up since 2017 for a left adrenal nodule. Due to the increase of catecholamine secretion (24 h urinary normetanephrine 1407.6 mcg, 24 h urinary metanephrine 8.1 mcg), in 2019 the patient underwent Metaiodobenzylguanidine (MIBG) scintigraphy that resulted negative. Notwithstanding, later on the patient complained that her blood pressure had become difficult to control and laboratory evaluation revealed a progressive increase of 24 h urinary normetanephrine (3016 mcg/24 h), with normal level of metanephrine (20.0 mcg/24 h). The patient underwent computed tomography which confirmed a 15-mm nodule of the left adrenal gland with benign contrast-enhancement features; besides, a 25-mm tumor of uterus was documented. Hysteroscopy was performed with uterine endometrial biopsy, which revealed a well-differentiated endometrioid endometrial adenocarcinoma. With the presumptive diagnosis of pheochromocytoma associated with endometrioid endometrial adenocarcinoma, a laparoscopic left adrenalectomy along with hysteroannessiectomy was scheduled.
Intrarenal hemodynamics and kidney function in pheochromocytoma and paraganglioma before and after surgical treatment
Published in Blood Pressure, 2021
Magdalena Januszewicz, Piotr Dobrowolski, Andrzej Januszewicz, Ewa Warchoł-Celińska, Katarzyna Jóźwik-Plebanek, Daria Motyl, Marek Kabat, Mariola Pęczkowska, Ilona Michałowska, Urszula Ambroziak, Sadegh Toutounchi, Zbigniew Gałązka, Louisiane Courcelles, Marco Pappaccogli, Graeme Eisenhofer, Alexandre Persu, Jacques W. M. Lenders, Jacek Kądziela, Aleksander Prejbisz
Catecholamine biochemical phenotypes were assessed based on relative tumour-derived increases in plasma concentrations of normetanephrine, metanephrine, and methoxytyramine. Tumour-derived increments were calculated by subtracting from the concentration of each metabolite in each patient with a PPGL the mean concentration of the corresponding metabolites- normetanephrine (52 pg/mL), metanephrine (26 pg/mL) and methoxytyramine (5 pg/mL) in a previously described reference group [14]. We defined adrenergic tumours as those exhibiting both an increase in plasma metanephrine above a predefined cut-off (62 pg/mL) and a tumour-derived increment of metanephrine larger than 5% of combined increments of all O-methylated metabolites. We defined all other tumours as non-adrenergic, including both noradrenergic and dopaminergic tumors [15]. Within the non-adrenergic group only one patient was characterised by solitary increased secretion of dopamine but not of noradrenaline nor adrenaline.
Impact of obesity with or without hypertension on systemic haemodynamic and renal responses to lower body negative pressure
Published in Blood Pressure, 2021
Nima Vakilzadeh, Dusan Petrovic, Marc Maillard, Lucie Favre, Eric Grouzmann, Gregoire Wuerzner
Norepinephrine (NE), epinephrine (EPI) and urinary metanephrines (normetanephrine and metanephrine) were measured using ultra high-performance liquid chromatography-tandem mass spectrometry in plasma and urine [15–17]. PRA was determined by a radioimmunoassay kit for the quantitative determination of Angiotensin I in human plasma (REN-CT2, Cisbio Bioassays, Codolet, France), and we measured aldosterone with a commercial RIA kit (Aldo-Riact; CIS Bio International, Yvette, Cedex, Saclay, France). Endogenous trace lithium, a marker of proximal sodium reabsorption, was measured by atomic absorption spectrophotometry [18]. Plasma leptin was determined using a sensitive and specific sandwich ELISA for human leptin (EZHL-81K; Linco Research, St. Charles, MO). Total plasma adiponectin levels were determined using a sensitive and specific sandwich ELISA for human adiponectin (EZHADP-61K; Linco Research, St. Charles, MO).
Related Knowledge Centers
- Adrenaline
- Metabolite
- Normetanephrine
- Pheochromocytoma
- Neoplasm
- Catechol-O-Methyltransferase
- Metanephrines