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Endocrine diseases and pregnancy
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Pheochromocytoma is a tumor of the adrenal medulla or extra-adrenal chromaffin tissue that hypersecretes catechola-mines (80). Prevalence in pregnancy is estimated to be approximately 1 in 54,000, and over 200 cases have been reported in the literature (81). Typical clinical manifestations include sustained or paroxysmal hypertension, pallor, headache, palpitations, and diaphoresis. Hypertensive crisis can occur and is a feared complication. Serious sequelae include chest or abdominal pain, which can mimic acute myocardial infarction or acute surgical abdomen, seizures, hemodynamic shock (due to volume contraction induced by the excessive vasoconstricted state, when catecholamine secretory troughs occur), and death. Approximately 10% of pheochromocytomas are familial, including the multiple endocrine neoplasia (MEN) 2a and 2b syndromes (with pheochromocytoma about half as prevalent as medullary thyroid carcinoma), von Hippel–Lindau, and neurofibromatosis syndromes. Timely evaluation and definitive treatment of pheochromocytoma in pregnancy is essential, as untreated maternal mortality may exceed 15%, and if treated, mortality may be as low as 1% (82). Abdominal palpation and labor are known provocateurs of catecholamine secretion by pheochromocytoma tumors, and hypertensive crisis may accompany a secretory surge of adrenomedullary hormones.
Mediastinal masses
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Brent R. Weil, Robert C. Shamberger
The solid neural tumors have a fusiform shape and are based in the posterior sulcus between the vertebral bodies and the ribs. The age of the patient will give some hint of the diagnosis: Neuroblastomas and ganglioneuroblastomas arise more often in infants. Ganglioneuromas occur in older children and are generally asymptomatic, but they can extend into the spinal canal and produce neurologic symptoms. Neurofibromas arise primarily in conjunction with neurofibromatosis (von Recklinghausen's disease) and are often associated with scoliosis. Paragangliomas may arise in the posterior mediastinum, although they are rare. They often present with symptoms related to catecholamine secretion, particularly paroxysmal hypertension, diaphoresis, and palpitations. Extralobar pulmonary sequestrations arise in the posterior mediastinum with arterial supply from the aorta. They generally can be distinguished by a triangular shape.
Endocrine Disorders
Published in Gozie Offiah, Arnold Hill, RCSI Handbook of Clinical Surgery for Finals, 2019
Presentation➣ Classic Triad: Episodic headache, sweating and tachycardia.➣ Sustained or paroxysmal hypertension (85-95% of patients).➣ Headache (up to 90%).➣ Generalised sweating (60-70%).➣ Other: palpitations, tremor, pallor, panic attacks, weakness and dyspnea.➣ Increased availability of imaging has led to an increase of asymptomatic presentation.
Para-aortic paraganglioma mimicking lymph node metastasis in an ovarian carcinoma: a case report
Published in Journal of Obstetrics and Gynaecology, 2018
Gozde Sahin, Ceyhan Ugurluoglu, Serra Akar, Ayhan Gul, Aysegul Kebapcilar, Cetin Celik
Typical clinical manifestations, including paroxysmal hypertension, headache, excessive perspiration and palpitations, are well-described. In the absence of this complex of symptoms, an extra-adrenal paraganglioma may be incidentally discovered during unrelated surgery, as in our patient; at autopsy or during abdominal imaging for abdominal pain or mass (Krane 1986). The preoperative diagnosis of asymptomatic paraganglioma is difficult due to the lack of specific symptoms. Unfortunately, when diagnosed during surgery, patients have high morbidity and mortality rates. Unless patients who harbour this uncommon tumour are correctly prepared and protected from the effects of excessive catecholamine release, they are at risk when undergoing any kind of surgical procedure (Hull 1986). A life-threatening reaction may be induced via the physical manipulation of the tumour or the utilisation of drugs that induce free catecholamine release. The risk of a hypertensive crisis due to surgical excision can be reduced via an alpha and beta adrenergic blockade. Once the diagnosis and drug preparation have been accomplished, a complete surgical resection can be performed. Resection is often challenging because these highly vascular tumours are located near vital blood vessels (Young 2006; Wen et al. 2010).
Characteristics and outcome of patients with pheochromocytoma at a tertiary endocrinology clinic in Durban, South Africa over 14 years
Published in Journal of Endocrinology, Metabolism and Diabetes of South Africa, 2018
Abdurraouf Esseid Zorgani, Fraser J Pirie, Ayesha A Motala
Figure 1 shows the clinical features at presentation. The three most common symptoms were headache (68.6%), palpitation (60%), and sweating (57.6%). Abdominal pain, fatigue and weight loss were also frequent symptoms. The majority of patients (n = 30; 85.7%) presented with hypertension; of these, 65.7% had sustained hypertension and 20% had paroxysmal hypertension. Clinical features of established hypertension, namely retinopathy (grade I (n = 4; 12.5%), grade II (n = 4; 12.5%) and grade III (n = 1; 3.1%)) and left ventricular hypertrophy were found in 28.1% and 34.3%, respectively. Orthostatic hypotension was a feature in seven (20%) patients. Three patients were pregnant at presentation; one in the second trimester and two in the third trimester.
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
Hypertension was defined as either a known history of hypertension, current use of antihypertensive drugs or systolic BP above or equal to 140 mm Hg or diastolic BP above or equal to 90 mm Hg on office measurements or daytime systolic BP above or equal to 135 mmHg or diastolic BP above or equal to 85 mm Hg on 24-h ambulatory BP monitoring (ABPM) [13]. Known duration of hypertension was assessed from patients interviews and defined as the period between first diagnosis and/or treatment of hypertension and study entry. Paroxysmal hypertension was defined as episodic increments of BP of >40–50 mm Hg over usual patient’s BP values.