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Vol. 64. Issue 4.
Pages 348-367 (July - August 2022)
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Vol. 64. Issue 4.
Pages 348-367 (July - August 2022)
Radiology through images
Adrenal pheochromocytoma: Keys to radiologic diagnosis
Feocromocitoma adrenal. Claves para el diagnóstico radiológico
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1566
M.A. Corral de la Callea,
Corresponding author
migcorral@gmail.com

Corresponding author.
, J. Encinas de la Iglesiab, G.C. Fernández-Pérezc, M. Repollés Cobaledad, A. Frainoa
a Servicio de Radiodiagnóstico, Complejo Asistencial de Ávila, Ávila, Spain
b Servicio de Radiodiagnóstico, Complejo Asistencial de Salamanca, Salamanca, Spain
c Centro Radiológico Grupo Recoletas, Valladolid, Spain
d Servicio de Radiodiagnóstico, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain
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Abstract

Pheochromocytomas are adrenal paragangliomas. Potentially malignant, these tumors have a low incidence but clear importance. They can appear in various hereditary syndromes, especially in von Hippel-Lindau syndrome, multiple endocrine neoplasia-2 (MEN2), and familial paraganglioma syndromes. In sporadic cases, underlying genetic alterations are often found, and these findings are changing our understanding of the disease.

Although these tumors can manifest with a characteristic clinical presentation, in 13.1%–57.6% of cases, it is the radiologist who first suggests the diagnosis, indicating analyses for catecholamines or nuclear medicine examinations.

Radiologists should suspect a pheochromocytoma on detection of a well-delimited adrenal mass with rapid, intense enhancement that typically shows cystic and hemorrhagic phenomena, high T2 signal intensity, and the absence of macroscopic or microscopic lipids. The behavior in diffusion-weighted imaging usually does not provide very useful information. Approximately one-third of lesions show late washout similar to that seen with adenomas on CT. Percutaneous puncture should be avoided to avoid the risk of unleashing a severe hypertensive crisis.

Keywords:
MeSH terms
Pheochromocytoma
Paraganglioma
Adrenal glands
Adrenal gland tumors
Multidetector computed tomography
Magnetic resonance imaging
Diffusion-weighted imaging
Resumen

El feocromocitoma es un paraganglioma adrenal potencialmente maligno, con baja incidencia, pero relevancia evidente. Puede aparecer en varios síndromes hereditarios, especialmente von Hippel-Lindau, neoplasia endocrina múltiple 2 y paraganglioma familiar. En casos esporádicos subyacen también frecuentemente alteraciones genéticas que están cambiando el paradigma de la enfermedad.

Aunque puede tener una presentación clínica característica, en un 13,1−57,6% de los casos es el radiólogo el primero en sugerirlo, indicando determinaciones analíticas de catecolaminas o exploraciones de Medicina Nuclear.

Debe sospecharse ante una masa adrenal bien delimitada con realce intenso y rápido, mostrando característicamente fenómenos quísticos y hemorrágicos, alta señal en T2 y ausencia de lípidos macro o microscópicos. El comportamiento en difusión no suele aportar información muy relevante. Aproximadamente un tercio presentan lavado tardío similar al del adenoma en TC. Debe evitarse su punción percutánea ante su sospecha, por el riesgo de desencadenar una crisis hipertensiva grave.

Palabras clave:
Feocromocitoma
Paraganglioma
Glándulas adrenales
Tumores de las glándulas adrenales
Tomografía computarizada multidetector
Imagen por resonancia magnética
Resonancia magnética potenciada en difusión

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