Multiple and hereditary renal tumors: a review for radiologists.

80% of renal carcinomas (RC) are diagnosed incidentally by imaging. 2-4% of "sporadic" multifocality and 5-8% of hereditary syndromes are accepted, probably with underestimation. Multifocality, young age, familiar history, syndromic data, and certain histologies lead to suspicion of hereditary syndrome. Each tumor must be studied individually, with a multidisciplinary evaluation of the patient. Nephron-sparing therapeutic strategies and a radioprotective diagnostic approach are recommended. Relevant data for the radiologist in major RC hereditary syndromes are presented: von-Hippel-Lindau, Chromosome-3 translocation, BRCA-associated protein-1 mutation, RC associated with succinate dehydrogenase deficiency, PTEN, hereditary papillary RC, Papillary thyroid cancer- Papillary RC, Hereditary leiomyomatosis and RC, Birt-Hogg-Dubé, Tuberous sclerosis complex, Lynch, Xp11.2 translocation/TFE3 fusion, Sickle cell trait, DICER1 mutation, Hereditary hyperparathyroidism and jaw tumor, as well as the main syndromes of Wilms tumor predisposition. The concept of "non-hereditary" familial RC and other malignant and benign entities that can present as multiple renal lesions are discussed.

Tumores renales múltiples y hereditarios. Revisión por y para radiólogos / Multiple and hereditary renal tumors: A review for radiologists

El 80% de los carcinomas renales (CR) se diagnostican incidentalmente por imagen. Se aceptan un 2-4% de multifocalidad «esporádica» y un 5-8% de síndromes hereditarios, probablemente con infraestimación. Multifocalidad, edad joven, historia familiar, datos sindrómicos y ciertas histologías hacen sospechar un síndrome hereditario. Debe estudiarse individualmente cada tumor y multidisciplinarmente al paciente, con estrategias terapéuticas conservadoras de nefronas y un abordaje diagnóstico radioprotector. Se revisan los datos relevantes para el radiólogo en los síndromes de von Hippel-Lindau, translocación de cromosoma-3, mutación de proteína-1 asociada a BRCA, CR asociado a déficit en succinato-deshidrogenasa, PTEN, CR papilar hereditario, cáncer papilar tiroideo-CR papilar, leiomiomatosis hereditaria y CR, Birt-Hogg-Dubé, complejo esclerosis tuberosa, Lynch, translocación Xp11.2/fusión TFE3, rasgo de células falciformes, mutación DICER1, hiperparatoridismo y tumor mandibular hereditario, así como los principales síndromes de predisposición al tumor de Wilms.(AU)

80% of renal carcinomas (RC) are diagnosed incidentally by imaging. 2-4% of “sporadic” multifocality and 5-8% of hereditary syndromes are accepted, probably with underestimation. Multifocality, young age, familiar history, syndromic data, and certain histologies lead to suspicion of hereditary syndrome. Each tumor must be studied individually, with a multidisciplinary evaluation of the patient. Nephron-sparing therapeutic strategies and a radioprotective diagnostic approach are recommended. Relevant data for the radiologist in major RC hereditary syndromes are presented: von-Hippel-Lindau, Chromosome-3 translocation, BRCA-associated protein-1 mutation, RC associated with succinate dehydrogenase deficiency, PTEN, hereditary papillary RC, Papillary thyroid cancer- Papillary RC, Hereditary leiomyomatosis and RC, Birt-Hogg-Dubé, Tuberous sclerosis complex, Lynch, Xp11.2 translocation/TFE3 fusion, Sickle cell trait, DICER1 mutation, Hereditary hyperparathyroidism and jaw tumor, as well as the main syndromes of Wilms tumor predisposition. The concept of “non-hereditary” familial RC and other malignant and benign entities that can present as multiple renal lesions are discussed.(AU)

Dual-energy CT: Technical considerations and clinical applications.

Although dual-energy CT was initially described by Hounsfield in 1973, it remains underused in clinical practice. It is therefore important to emphasize the clinical benefits and limitations of this technique. Iodine mapping makes it possible to quantify the uptake of iodine, which is very important in characterizing tumors, lung perfusion, pulmonary nodules, and the tumor response to new treatments. Dual-energy CT also makes it possible to obtain virtual single-energy images and virtual images without iodinated contrast or without calcium, as well as to separate materials such as uric acid or fat and to elaborate hepatic iron overload maps. In this article, we review some of the clinical benefits and technical limitations to improve understanding of dual-energy CT and expand its use in clinical practice.

Adrenal pheochromocytoma: Keys to radiologic diagnosis.

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.

Feocromocitoma adrenal. Claves para el diagnóstico radiológico / Adrenal pheochromocytoma: keys to radiologic diagnosis

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.(AU)

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% to 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.(AU)

Review of imaging techniques for evaluating morphological and functional responses to the treatment of bone metastases in prostate and breast cancer

Bone metastases are very common complications associated with certain types of cancers that frequently negatively impact the quality of life and functional status of patients; thus, early detection is necessary for the implementation of immediate therapeutic measures to reduce the risk of skeletal complications and improve survival and quality of life. There is no consensus or universal standard approach for the detection of bone metastases in cancer patients based on imaging. Endorsed by the Spanish Society of Medical Oncology (SEOM), the Spanish Society of Medical Radiology (SERAM), and the Spanish Society of Nuclear Medicine and Molecular Imaging (SEMNIM) a group of experts met to discuss and provide an up-to-date review of our current understanding of the biological mechanisms through which tumors spread to the bone and describe the imaging methods available to diagnose bone metastasis and monitor their response to oncological treatment, focusing on patients with breast and prostate cancer. According to current available data, the use of next-generation imaging techniques, including whole-body diffusion-weighted MRI, PET/CT, and PET/MRI with novel radiopharmaceuticals, is recommended instead of the classical combination of CT and bone scan in detection, staging and response assessment of bone metastases from prostate and breast cancer.

Review of imaging techniques for evaluating morphological and functional responses to the treatment of bone metastases in prostate and breast cancer.

Bone metastases are very common complications associated with certain types of cancers that frequently negatively impact the quality of life and functional status of patients; thus, early detection is necessary for the implementation of immediate therapeutic measures to reduce the risk of skeletal complications and improve survival and quality of life. There is no consensus or universal standard approach for the detection of bone metastases in cancer patients based on imaging. Endorsed by the Spanish Society of Medical Oncology (SEOM), the Spanish Society of Medical Radiology (SERAM), and the Spanish Society of Nuclear Medicine and Molecular Imaging (SEMNIM) a group of experts met to discuss and provide an up-to-date review of our current understanding of the biological mechanisms through which tumors spread to the bone and describe the imaging methods available to diagnose bone metastasis and monitor their response to oncological treatment, focusing on patients with breast and prostate cancer. According to current available data, the use of next-generation imaging techniques, including whole-body diffusion-weighted MRI, PET/CT, and PET/MRI with novel radiopharmaceuticals, is recommended instead of the classical combination of CT and bone scan in detection, staging and response assessment of bone metastases from prostate and breast cancer.Clinical trial registration: Not applicable.

SARS-CoV-2: what it is, how it acts, and how it manifests in imaging studies. / SARS-CoV-2: cómo es, cómo actúa y cómo se expresa en la imagen.

COVID-19 is a disease with many clinical, biochemical, and radiological signs that has a predilection for the lungs, probably because of the high number of ACE-2 receptors in this organ. The infection of cells activates proinflammatory substances, causing diffuse alveolar damage, which is the histopathological basis of ARDS. The exudative phase would manifest as ground-glass opacities and consolidation, and the proliferative phase would manifest as a tendency toward a more linear morphology. Both CT and PET/CT findings support the inflammatory character of the lung lesions in the initial phase of the disease and in patients with mild-moderate disease. Severe cases have pulmonary hypoperfusion that is likely due to abnormal alveolar ventilation and perfusion. On the other hand, a prothrombotic state increases the risk of thromboembolic disease through the activation of coagulation and platelet pathways with the production of fibrin degradation products (D-dimer) and consumption of platelets.

Orbital supratrochlear fistula secondary to anaesthetic block in cataract surgery. / Fístula orbitaria supratroclear secundaria a la realización de un bloqueo para anestesia en una cirugía de catarata.

A case is described of a woman with history of right peribulbar nerve block prior to cataract surgery that, within a few days, presented with a trochlear intraorbital arteriovenous fistula. This was resolved without sequelae after three months by performing intermittent homolateral carotid massages. This is the first case of supratrochlear intraorbital arteriovenous fistula. There are only 8 cases reported of traumatic intraorbital arteriovenous fistula, and only one was with post-peribulbar anaesthesia.

The radiologist's role in the management of papillary renal cell carcinoma. / Carcinoma papilar de células renales: el papel del radiólogo en su manejo.

Papillary carcinoma is the second most common renal cell carcinoma. It has a better prognosis than the more frequent clear cell carcinoma, although this does not hold true for advanced cases, because no specific treatment exists. It presents as a circumscribed peripheral tumor (small and homogeneously solid or larger and cystic/hemorrhagic) or as an infiltrating lesion that invades the veins, which has a worse prognosis. Due to their low vascular density, papillary renal cell carcinomas enhance less than other renal tumors, and this facilitates their characterization. On computed tomography, they might not enhance conclusively, and in these cases they are impossible to distinguish from hyperattenuating cysts. Contrast-enhanced ultrasonography and magnetic resonance imaging are more sensitive for detecting vascularization. Other characteristics include a specific vascular pattern, hypointensity on T2-weighted images, restricted water diffusion, and increased signal intensity in opposed phase images. We discuss the genetic, histologic, clinical, and radiological aspects of these tumors in which radiologists play a fundamental role in management.

Esophageal cancer: anatomic particularities, staging, and imaging techniques. / Cáncer de esófago: particularidades anatómicas, estadificación y técnicas de imagen.

Cancer of the esophagus is a tumor with aggressive behavior that is usually diagnosed in advanced stages. The absence of serosa allows it to spread quickly to neighboring mediastinal structures, and an extensive lymphatic drainage network facilitates tumor spread even in early stages. The current TNM classification, harmonized with the classification for gastric cancer, provides new definitions for the anatomic classification, adds non-anatomic characteristics of the tumor, and includes tumors of the gastroesophageal junction. Combining endoscopic ultrasound, computed tomography, positron emission tomography, and magnetic resonance imaging provides greater accuracy in determining the initial clinical stage, and these imaging techniques play an essential role in the selection, planning, and evaluation of treatment. In this article, we review some particularities that explain the behavior of this tumor and we describe the current TNM staging system; furthermore, we discuss the different imaging tests available for its evaluation and include a diagnostic algorithm.

Función sistólica del ventrículo izquierdo: cómo comprenderla y analizarla / Left ventricular systolic function: How to understand it and analyze it

En los estudios de resonancia magnética cardíaca, la función sistólica del ventrículo izquierdo suele calcularse automáticamente. Entender cómo se obtiene cada parámetro de la función y el por qué de las diferencias que pueden existir entre los valores obtenidos con dos técnicas, por ejemplo ecografía y resonancia magnética, es fundamental para comprenderlas e interpretarlas adecuadamente. En este artículo se detalla el análisis habitual de la función sistólica desde un punto de vista cuantitativo y cualitativo, y se explican otros métodos que no requieren un software específico. Hemos diseñado (y lo aportamos para que pueda usarse libremente) un fichero que, empleando el programa Microsoft Excel®, permite analizar la función sistólica sencilla e intuitivamente (AU)

In cardiac magnetic resonance imaging studies, left ventricular systolic function is usually calculated automatically. To understand and interpret parameters of left ventricular systolic function correctly, it is fundamental to understand how each parameter is obtained and why values obtained with different techniques, for example, ultrasonography and magnetic resonance imaging, can differ. This article provides details about the usual analysis of systolic function from the quantitative and qualitative points of view; it also explains other methods that do not require specific software. Moreover, we provide a file that we designed for use with Microsoft Excel® to enable simple, intuitive analysis of systolic function. Readers can use this file freely (AU)

Enfermedad valvular cardíaca. Valoración con tomografía computarizada multidetector / Valvular heart disease: Multidetector computed tomography evaluation

La enfermedad valvular cardíaca es un problema clínico que se ha estudiado con técnicas de imagen clásicas como la ecocardiografía o la RM. El avance tecnológico de la TC permite obtener imágenes estáticas y dinámicas con las que hacer un análisis morfológico y, en muchas ocasiones, funcional. A pesar que actualmente está solo indicada en los pacientes en los que la ecocardiografía o la RM no son concluyentes o están contraindicadas, la TC multidetector permite diagnosticar la estenosis o insuficiencia por planimetría, valorar y cuantificar el calcio valvular, y mostrar las repercusiones funcionales en el resto de estructuras cardíacas. Teniendo en cuenta que la TC multidetector es una técnica cada día más utilizada para el diagnóstico de la enfermedad isquémica, creemos interesante que el radiólogo conozca la potencialidad que esta técnica tiene en el estudio de la afección valvular (AU)

Heart valve disease is a clinical problem that has been studied with classical imaging techniques like echocardiography and MRI. Technological advances in CT make it possible to obtain static and dynamic images that enable not only a morphological but also a functional analysis in many cases. Although it is currently indicated only in patients with inconclusive findings at echocardiography and MRI or those in whom these techniques are contraindicated, multidetector CT makes it possible to diagnose stenosis or regurgitation through planimetry, to evaluate and quantify valvular calcium, and to show the functional repercussions of thesephenomena on the rest of the structures of the heart. Given that multidetector CT is being increasingly used in the diagnosis of ischemic heart disease, we think it is interesting for radiologists to know its potential for the study of valvular disease (AU)

[Left ventricular systolic function: how to understand it and analyze it]. / Función sistólica del ventrículo izquierdo: cómo comprenderla y analizarla.

In cardiac magnetic resonance imaging studies, left ventricular systolic function is usually calculated automatically. To understand and interpret parameters of left ventricular systolic function correctly, it is fundamental to understand how each parameter is obtained and why values obtained with different techniques, for example, ultrasonography and magnetic resonance imaging, can differ. This article provides details about the usual analysis of systolic function from the quantitative and qualitative points of view; it also explains other methods that do not require specific software. Moreover, we provide a file that we designed for use with Microsoft Excel(®) to enable simple, intuitive analysis of systolic function. Readers can use this file freely.

Disección aislada y espontánea de arterias viscerales / Isolated spontaneous dissection of visceral arteries

Presentamos dos casos de disección aislada y espontánea de arterias esplácnicas en varones, diagnosticados con TC multidetector. Comenzaron con dolor agudo, más intenso y duradero en el caso de disección de tronco celiaco, que presentaba cambios periarteriales. En el caso de disección de arteria mesentérica superior se asociaba una estenosis en el origen del tronco celiaco. En ambos la sospecha clínica fue de disección aórtica. Fueron dos disecciones cortas con luz falsa permeable de forma sacular y luz verdadera de calibre reducido. Este tipo morfológico es uno de los más infrecuentes de esta de por sí rara entidad. En los últimos años, no obstante, se aprecia un aumento en el número de casos publicados, sugiriendo que pudiera ser infradiagnosticada previamente a la generalización del uso de la TC multidetector. Discutimos las dos clasificaciones morfológicas existentes y la necesidad de adecuar el manejo terapéutico a las circunstancias particulares de cada caso (AU)

We present the cases of two men with isolated spontaneous dissection of visceral arteries diagnosed by multidetector CT. In the first patient, dissection of the celiac trunk was associated with periarterial changes. In the second patient, dissection of the superior mesenteric artery was associated with stenosis at the root of the celiac trunk. Both patients presented with acute pain, which was more intense and longer lasting in the first patient. Aortic dissection was suspected clinically in both patients. Both dissections were short and had patent saccular false lumens and reduced caliber of the true lumens. This morphological type is one of the most uncommon within this rare entity. However, in recent years, the number of cases published is rising. This suggests that this entity may have been underdiagnosed before the widespread use of multidetector CT. We discuss the two morphological classifications of dissection of the visceral arteries and the need to adapt therapeutic management to the particular circumstances of each case (AU)

[Isolated spontaneous dissection of visceral arteries]. / Disección aislada y espontánea de arterias viscerales.

We present the cases of two men with isolated spontaneous dissection of visceral arteries diagnosed by multidetector CT. In the first patient, dissection of the celiac trunk was associated with periarterial changes. In the second patient, dissection of the superior mesenteric artery was associated with stenosis at the root of the celiac trunk. Both patients presented with acute pain, which was more intense and longer lasting in the first patient. Aortic dissection was suspected clinically in both patients. Both dissections were short and had patent saccular false lumens and reduced caliber of the true lumens. This morphological type is one of the most uncommon within this rare entity. However, in recent years, the number of cases published is rising. This suggests that this entity may have been underdiagnosed before the widespread use of multidetector CT. We discuss the two morphological classifications of dissection of the visceral arteries and the need to adapt therapeutic management to the particular circumstances of each case.

[Valvular heart disease: multidetector computed tomography evaluation]. / Enfermedad valvular cardíaca. Valoración con tomografía computarizada multidetector.

Heart valve disease is a clinical problem that has been studied with classical imaging techniques like echocardiography and MRI. Technological advances in CT make it possible to obtain static and dynamic images that enable not only a morphological but also a functional analysis in many cases. Although it is currently indicated only in patients with inconclusive findings at echocardiography and MRI or those in whom these techniques are contraindicated, multidetector CT makes it possible to diagnose stenosis or regurgitation through planimetry, to evaluate and quantify valvular calcium, and to show the functional repercussions of these phenomena on the rest of the structures of the heart. Given that multidetector CT is being increasingly used in the diagnosis of ischemic heart disease, we think it is interesting for radiologists to know its potential for the study of valvular disease.

Análisis de la función diastólica del ventrículo izquierdo mediante resonancia magnética / Analysis of left ventricular diastolic function using magnetic resonance imaging

La insuficiencia cardíaca no siempre es debida a una alteración sistólica, y una disfunción diastólica puede explicar muchos casos de insuficiencia cardíaca con función sistólica normal. La función diastólica depende de la capacidad de llenado del ventrículo izquierdo para garantizar un volumen latido normal. Se mide rutinariamente con la ecocardiografía transtorácica, ya que se trata de una prueba no invasiva y de gran accesibilidad. La resonancia magnética (RM), utilizando secuencias de flujo, muestra una buena concordancia con la ecocardiografía, analizando la función diastólica de forma práctica, a través del flujo en la válvula mitral y las venas pulmonares. En este sentido, el análisis de la función diastólica debería añadirse como parte de un examen rutinario de RM cardíaca (AU)

Heart failure is not always due to an alteration in systolic function, and a diastolic dysfunction could explain many cases of heart failure with a normal systolic function. Diastolic function depends on the left ventricular filling capacity to ensure a normal stroke volume. It is routinely measured with transthoracic echocardiography, as it is an easily accessible non-invasive test. The MRI, using flow sequences, shows good agreement with the echocardiography, analysing the diastolic function in a practical way, by the flow into the mitral valve and pulmonary veins. In this sense, the analysis of diastolic function should be added as part of a routine cardiac MR examination (AU)

[Analysis of left ventricular diastolic function using magnetic resonance imaging]. / Análisis de la función diastólica del ventrículo izquierdo mediante resonancia magnética.

Heart failure is not always due to an alteration in systolic function, and a diastolic dysfunction could explain many cases of heart failure with a normal systolic function. Diastolic function depends on the left ventricular filling capacity to ensure a normal stroke volume. It is routinely measured with transthoracic echocardiography, as it is an easily accessible non-invasive test. The MRI, using flow sequences, shows good agreement with the echocardiography, analysing the diastolic function in a practical way, by the flow into the mitral valve and pulmonary veins. In this sense, the analysis of diastolic function should be added as part of a routine cardiac MR examination.