Manejo clínico y quirúrgico de los feocromocitomas y paragangliomas / Clinical and surgical management of pheochromocytomas and paragangliomas

Los feocromocitomas y paragangliomas (Feo/PGL) son tumores neurendocrinos raros con diferentes presentaciones clínicas, asociados a alta morbimortalidad. Reconocer los signos y síntomas es el paso diagnóstico inicial. Las metanefrinas fraccionadas urinarias tienen una excelente sensibilidad y especificidad. La tomografía computarizada (TC) es el método de elección para su localización. La tomografía por emisión de positrones (PET) con F18-fluordeoxiglucosa (F18-FDG) es el método funcional recomendado para detectar metástasis. La resección quirúrgica constituye la única opción curativa en estos pacientes. La adrenalectomía laparoscópica es la vía de abordaje para la mayoría los Feo/PGL. El tratamiento farmacológico, 7 a 14 días previos con alfabloqueantes y betabloqueantes, tiene como objetivo normalizar la presión arterial y prevenir complicaciones cardiovasculares periquirúrgicas. Se conoce que al menos un tercio de los pacientes presentan una mutación genética germinal. El estudio genético debe estar orientado a las características sindrómicas, formas de presentación, localización y fenotipo bioquímico del tumor. Se recomienda el abordaje interdisciplinario en centros especializados con experiencia en esta patología poco frecuente. (AU)

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors with different clinical manifestation associated with high morbidity and mortality. Recognize signs and symptoms is the first step in diagnosis. Urinary fractionated metanephrines have an excellent specificity and sensitivity. Computed Tomography (CT) is the first-choice imaging modality for location. F18-DG positron tomography (PET)/CT scanning is the functional modality of choice for metastatic disease. Surgery is the only curative treatment. Minimally invasive adrenalectomy is the surgical approach for most adrenal pheochromocytomas. Perioperative alpha and beta blockade for 7 to 14 days normalize blood pressure and prevent perioperative cardiovascular complications. Is recognize that at least one-third of the patients have disease-causing germline mutations. Genetic testing must be orientated to syndromic features, presentation, localization and biochemical profile of these tumors. Multidisciplinary teams at centers with appropriate expertise are recommended to ensure a favorable outcome. (AU)

Clinical characteristics and follow-up of Korean patients with adrenal incidentalomas

BACKGROUND/AIMS: We investigated the clinical characteristics and follow-up findings of subjects with adrenal incidentalomas in a single, tertiary-care hospital in South Korea. METHODS: The study consisted of a retrospective analysis of 282 adrenal incidentaloma patients who underwent radiographic and endocrinological evaluations at Samsung Medical Center in Seoul, South Korea, between January 2004 and July 2011. RESULTS: Most (86.2%) of the subjects were found to have nonfunctioning tumors. Functioning tumors were seen in 39 patients (13.8%). Among them, 28 (9.9%) had subclinical Cushing syndrome (SCS), six (2.1%) had pheochromocytoma, and five (1.8%) had primary hyperaldosteronism. Malignant adrenal tumors were discovered in three cases: two (0.7%) were primary adrenal cancers, and one (0.4%) was a secondary metastasis from a lung cancer. Significant risk factors for functional tumors were female gender (odds ratio [OR], 3.386; 95% confidence interval [CI], 1.611 to 7.117; p = 0.0013) and a noncontrast attenuation value of > 10 Hounsfield units (OR, 2.806; 95% CI, 1.231 to 6.397; p = 0.0141). During follow-up (mean, 22.5 months) of 72 of the patients, three (4.2%) developed hormonal changes due to functional tumors. One was confirmed as pheochromocytoma by histopathology, and the others were diagnosed with SCS and followed routinely without surgical intervention. No malignant transformation was found in these patients. CONCLUSIONS: Based on these findings, initial hormonal and radiographic evaluations for adrenal incidentalomas appear to be more important than follow-up tests because functional or malignant changes are rare.

Assessment of serum catecholamine concentrations in patients with pheochromocytoma undergoing videolaparoscopic adrenalectomy

INTRODUCTION: We analyzed the changes in serum catecholamine concentrations, i.e. adrenaline and noradrenaline, in response to surgical stress in patients with pheochromocytoma who undergone videolaparoscopic adrenalectomy. MATERIALS AND METHODS: Between January 1998 and March 2002, 11 patients underwent 12 videolaparoscopic adrenalectomies. In one case, the adrenalectomy was bilateral. Serum catecholamines were measured at 6 surgical times: T0: control before induction; T1: following the induction, laryngoscopy and intubation sequence; T2: after installing the pneumoperitoneum; T3: during manipulation-exeresis of the pheochromocytoma; T4: following ablation of the pheochromocytoma; T5: in the recovery room following intervention when the patient was extubated and was hemodynamically stable. RESULTS: Mean concentrations of serum noradrenaline were significantly different when the T0 and T2 surgical times were compared (T0: 3161 pg/mL; T2: 40440 pg/mL; p < 0.01), T0 and T3 (T0: 3161 pg/mL; T3: 46021 pg/mL; p < 0.001), T1 and T3 (T1: 5531 pg/mL; T3: 46021 pg/mL; p < 0.01), T2 and T4 (T2: 40440 pg/mL; T4: 10773 pg/mL; p < 0.01) and T3 and T5 (T3: 46021 pg/mL; T5: 2549 pg/mL; p < 0.001). Mean concentrations of serum adrenaline were significantly different when the T0 and T3 surgical times were compared (T0: 738 pg/mL; T3: 27561 pg/mL; p < 0.01). CONCLUSION: The pneumoperitoneum significantly increases serum noradrenaline concentrations, manipulation of the adrenal gland significantly increases the serum concentrations of noradrenaline and adrenaline, and the pheochromocytoma ablation significantly decreases serum noradrenaline concentrations.