Adrenal incidentaloma: a diagnostic challenge

Panagiotis Anagnostis, Asterios Karagiannis, Konstantinos Tziomalos, Anna I. Kakafika, Vasilios G. Athyros, Dimitri P. Mikhailidis

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The widespread application of abdominal imaging procedures has resulted in an increased frequency of clinically silent adrenal masses. Adrenal incidentaloma (AI) is a term applied to an accidentally discovered adrenal mass on imaging performed for the investigation of an unrelated complaint …

Genotype-phenotype relationship in multiple endocrine neoplasia type 2. Implications for clinical management

Friedhelm Raue, Karin Frank-Raue

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Multiple endocrine neoplasia type 2 (MEN2) is an autosomal dominant tumour syndrome caused by germline activating mutations of the RET proto-oncogene. It has a strong penetrance of medullary thyroid carcinoma (MTC) and can be associated with bilateral pheochromocytoma and primary hyperparathyroidism (MEN2A) within a single patient or family…

Familial pheochromocytoma

Zoran Erlic, Hartmut P.H. Neumann

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Pheochromocytomas and Paragangliomas (PGL) form the group of paraganglial tumours which can occur in any paraganglia from the skull base to the pelvic floor. The terminology is not uniform. While the World Health Organization (WHO) applies pheochromocytoma exclusively to adrenal tumours, many clinicians use the term pheochromocytoma also for extra-adrenal abdominal and thoracic tumours, since by tradition …

Diagnosis of pheochromocytoma with special emphasis on MEN2 syndrome

Karel Pacak, Graeme Eisenhofer, Ioannis Ilias

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Pheochromocytomas/paragangliomas(PHEOs/PGLs) are rare but treacherous catecholamine-producing tumors which, if overlooked or improperly treated, will almost invariably prove fatal. Patients with MEN2 PHEOs have a high incidence of paroxysmal attacks …

Insulin resistance in pheochromocytoma improves more by surgical rather than by medical treatment

Evanthia Diamanti-Kandarakis, Evangelia Zapanti, Maria-Helen Peridis, Panayiotis Ntavos, George Mastorakos

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Pheochromocytoma, a neuroendocrine tumor, is often associated with hyperglycemia. To investigate the underlying pathogenetic mechanisms, five patients (3 women and 2 men, aged 49±2.5, mean±SD) with benign adrenal pheochromocytoma were studied with an oral glucose tolerance test (OGTT) and the euglycaemic clamp technique …

Surgery for pheochromocytoma: A 20-year experience of a single institution

Chrysanthi Aggeli, Alexander M. Nixon, Christos Parianos, Georgios Vletsis, Labrini Papanastasiou, Athina Markou, Theodora Kounadi, Georgrios Piaditis, Georgios N. Zografos

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OBJECTIVE: Resection of pheochromocytomas is a challenging procedure due to hemodynamic lability. Our aim was to evaluate surgical outcomes in 67 patients with pheochromocytoma and to validate the role of laparoscopic surgery in the treatment of these tumors. DESIGN: This study is a retrospective review. A total of 68 procedures for pheochromocytoma …

A rare missense variant in RET exon 8 in a Portuguese family with atypical multiple endocrine neoplasia type 2A

Ana Filipa Martins, João Martin Martins, Sónia do Vale, Teresa Dias, Catarina Silveira, Inês Rodrigues da Silva, Maria Carmo-Fonseca

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BACKGROUND AND OBJECTIVE: Multiple Endocrine Neoplasia type 2 (MEN2) is a rare genetic disorder characterized by medullary thyroid carcinoma (MTC), pheochromocytoma and primary hyperparathyroidism. MEN2 is an autosomal dominant syndrome caused by mutations in the RET proto-oncogene …

Symptomatic pheochromocytoma with normal urinary catecholamine metabolites

Dimitra Zianni, Marinella Tzanela, Serafim Klimopoulos, N.C. Thalassinos

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A 61-year old female presented with paroxysmal hypertension and a 4.5cm left adrenal mass on CT scan. Repeated measurements of 24-hour urinary fractionated metanephrines, total catecholamines and vanillylmandelic acid (VMA) were within normal range. A further scintigraphic study …

Adrenal cortical oncocytoma mimicking pheochromocytoma

Andreas Kiriakopoulos, Dimitrios Papaioannou, Dimitrios Linos

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Objective: Adrenal tumors present with clinical features and signs unique to their specific hormonal hypersecretion. However, there have been cases in which the clinical expression has been in conflict with the histologic features of the tumor. In this communication we report an unusual clinical presentation of an adrenal cortical tumor with histologic features …

Adrenal malignant melanoma masquerading as a pheochromocytoma in a patient with a history of a multifocal papillary and medullary thyroid carcinoma

Maria E. Barmpari, Christos Savvidis, Anastasia D. Dede, Haridimos Markogiannakis, Christina Dikoglou, Paraskevi Xekouki, Constantine A. Stratakis, Andreas Manouras, Sofia Malaktari-Skarantavou

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Objective: Adrenal masses usually represent benign and nonfunctional adrenal adenomas; however, primary or metastatic malignancy should also be considered. Discovery of an adrenal mass needs further evaluation in order to exclude malignancy and hormonal secretion. We present a rare case of a possibly primary adrenal malignant melanoma with imaging and biochemical features of a pheochromocytoma. Case report: A 61-year-old male farmer was referred for evaluation …

Landmarks in the history of adrenal surgery

Marios Papadakis, Andreas Manios, Georgios Schoretsanitis, Constantinos Trompoukis

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The last 100 years have seen a revolution in the understanding of adrenal disease and its surgical treatment. The isolation of its hormones, the detailed study of the adrenal medulla and the cortex together with the enormous expansion of surgical methods served as catalysts to this revolution. The Greek word for adrenal (epinephridio, from the Greek epi, upon, and nephros, kidney) dates back to the age of Homer who mentions the adrenal glands …