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Extra-adrenal pheochromocytomas are rare neuroendocrine tumors originating outside the adrenal glands and can pose significant diagnostic challenges due to their variable presentations. This report highlights a case of an extra-adrenal pheochromocytoma masquerading as a pancreatic head malignancy. We underscore the importance of considering extra-adrenal pheochromocytoma in the differential diagnosis of pancreatic masses, particularly when biochemical or clinical features suggest catecholamine excess.

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Clinical presentation of pheochromocytoma and screening recommendations.

Rev Clin Esp (Barc)

January 2025

Servicio de Endocrinología y Nutrición, Hospital Universitario Ramón y Cajal, Madrid, Spain; Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Madrid, Spain. Electronic address:

Pheochromocytomas are neuroendocrine tumors that derive from sympathetic adrenomedullary chromaffin tissue and produce catecholamines. Due to the excess release of catecholamines, they can produce arterial hypertension, tachycardia, sweating, headache and a large number of other clinical manifestations secondary to the stimulation of α and β adrenoreceptors. Screening for pheochromocytoma is recommended in patients with paroxysmal, resistant or early-onset arterial hypertension, in cases with symptoms suggestive of catecholamine hypersecretion, patients with hereditary syndromes associated with pheochromocytomas, diabetes mellitus of atypical presentation and in adrenal incidentalomas with radiological characteristics not typical of adenoma (with > 10 Hounsfield Units on non-contrast CT).

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Neurofibromatosis is a genetic disorder arising de novo or with an autosomal dominant transmission that typically presents either at birth or in early childhood, manifesting through distinctive clinical features such as multiple café-au-lait spots, benign tumors in the skin, bone enlargement, and deformities. This literature review aims to resume the spectrum of maternal and fetal complications encountered in pregnant women with neurofibromatosis type 1 (NF1). Thorough research was conducted on databases such as Web of Science, PubMed, Science Direct, Google Scholar, and Wiley Online Library.

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Quantifying urinary catecholamines and metanephrines is essential for the clinical screening and diagnosis of neuroendocrine tumours. HPLC with electrochemical detection (HPLC-ECD) is commonly used for this type of analysis but requires extensive sample cleanup. Simple and rapid dilute-and-shoot LC-multiple-reaction monitoring (MRM)-MS assays have been developed for quantitating these analytes in urine but have not yet been validated according to the Clinical and Laboratory Standards Institute (CLSI) guidelines.

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