Resumen ejecutivo del documento de consenso de expertos de la Sociedad Española de Neurocirugía y de la Sociedad Española de Endocrinología y Nutrición sobre: recomendaciones clínicas en el manejo perioperatorio de los tumores hipofisarios / Executive summary of the expert consensus document from the Spanish Society of Neurosurgery and the Spanish Society of Endocrinology and Nutrition: Clinical recommendations on the perioperative management of pituitary tumors

Los tumores hipofisarios (TH) suponen el 15% de los tumores intracraneales, y afectan del 10,7 al 14,4% de la población, si bien la incidencia de los TH clínicamente relevantes es de 5,1 casos/100.000 habitantes. El tratamiento quirúrgico está indicado en los TH que cursan con hipersecreción hormonal (a excepción de los TH productores de prolactina), así como en aquellos con clínica compresiva local o neurológica global. Los pacientes con TH requieren una atención multidisciplinar, idealmente en un centro de excelencia y basada en un protocolo asistencial bien definido. Con el objetivo de facilitar y estandarizar la práctica clínica ante este tipo de tumores, el presente documento recoge el posicionamiento del ÿrea de Conocimiento de Neuroendocrinología de la Sociedad Española de Endocrinología y Nutrición (SEEN) y la Sociedad Española de Neurocirugía (SENEC) sobre el manejo y el seguimiento prequirúrgico, quirúrgico y posquirúrgico del paciente con un TH (AU)

Pituitary tumors (PT) account for 15% of intracranial tumors affect 10.7 to 14.4% of the population although the incidence of clinically relevant PT is 5.1 cases/100,000 inhabitants. Surgical treatment is indicated in PTs with hormone hypersecretion (except for prolactin-producing PTs) and those with local compressive or global neurological symptoms. Multidisciplinary care, is essential for patients with PTs, preferably delivered in a center of excellence and based on a well-defined care protocol. In order to facilitate and standardize the clinical procedures for this type of tumor, this document gathers the positioning of the Neuroendocrinology Knowledge Area of the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Society of Neurosurgery (SENEC) on the management of patients with PTs and their preoperative, surgical and postoperative follow-up (AU)

Executive summary of the expert consensus document from the Spanish Society of Neurosurgery and the Spanish Society of Endocrinology and Nutrition: Clinical recommendations on the perioperative management of pituitary tumors.

Pituitary tumors (PT) account for 15% of intracranial tumors affect 10.7%-14.4% of the population although the incidence of clinically relevant PT is 5.1 cases/100,000 inhabitants. Surgical treatment is indicated in PTs with hormone hypersecretion (except for prolactin-producing PTs) and those with local compressive or global neurological symptoms. Multidisciplinary care, is essential for patients with PTs, preferably delivered in a center of excellence and based on a well-defined care protocol. In order to facilitate and standardize the clinical procedures for this type of tumor, this document gathers the positioning of the Neuroendocrinology Knowledge Area of the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Society of Neurosurgery (SENEC) on the management of patients with PTs and their preoperative, surgical and postoperative follow-up.

Executive summary of the expert consensus document from the Spanish Society of Neurosurgery and the Spanish Society of Endocrinology and Nutrition: clinical recommendations on the perioperative management of pituitary tumors.

Pituitary tumors (PT) account for 15% of intracranial tumors affect 10.7-14.4% of the population although the incidence of clinically relevant PT is 5.1 cases/100,000 inhabitants. Surgical treatment is indicated in PTs with hormone hypersecretion (except for prolactin-producing PTs) and those with local compressive or global neurological symptoms. Multidisciplinary care, is essential for patients with PTs, preferably delivered in a center of excellence and based on a well-defined care protocol. In order to facilitate and standardize the clinical procedures for this type of tumor, this document gathers the positioning of the Neuroendocrinology Knowledge Area of the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Society of Neurosurgery (SENEC) on the management of patients with PTs and their preoperative, surgical and postoperative follow-up.

Incidentalomas o no incidentalomas: ¿cuál es la relevancia de los adenomas hipofisarios en el adulto? / Incidentalomas or Non-Incidentalomas: What is the Relevance of Pituitary Adenomas in the Adult?

Introducción: desde el punto de vista anatómico, los adenomas hipofisarios (AH) se observan en el 10% de la población. Son en su mayoría pequeños y no funcionantes. La mayoría de los incidentalomas descubiertos en estudios de imágenes con alta resolución pedidos en situaciones clínicas frecuentes, como el traumatismo craneoencefálico, el accidente cerebrovascular y las demencias, corresponden a AH indolentes. Nos preguntamos cuál es la relevancia clínica de los adenomas hipofisarios. Desarrollo: los AH clínicamente relevantes son tumores en su mayoría benignos que conllevan, en diferentes proporciones, aumento en la morbilidad y/o mortalidad de los pacientes por mecanismos relacionados con la hipersecreción hormonal, la insuficiencia hormonal y/o los efectos de masa ocupante. La prevalencia de los AH clínicamente relevantes es mayor de la que se suponía hace 20 años. Afecta aproximadamente a 1/1000 habitantes. Los más prevalentes son los prolactinomas y los adenomas no funcionantes. La acromegalia, la enfermedad de Cushing y los tumores agresivos se traducen en pacientes complejos con mayor morbimortalidad. El diagnóstico temprano y el tratamiento multimodal proveen una razonable mejoría de la sobrevida. El estudio epidemiológico de los AH clínicamente relevantes es importante para la estimación del impacto en los sistemas de salud. Conclusiones: los estudios por imágenes de mejor resolución continuarán señalando incidentalomas hipofisarios. Una evaluación cuidadosa de los pacientes podrá identificar aquellos AH clínicamente relevantes. (AU)

Introduction: from the anatomical point of view, pituitary adenomas (HA) are observed in 10% of the population. They are mostly small and non-functioning. Most incidentalomas discovered in high-resolution imaging studies ordered in frequent clinical situations, such as head trauma, stroke and dementia, correspond to indolent HA. We wonder what is the clinical relevance of pituitary adenomas. Development: clinically relevant HAs are mostly benign tumors that lead, in different degrees, to an increased morbidity and/or mortality in patients by mechanisms related to hormone hypersecretion, hormone insufficiency and/or occupying mass effects. The prevalence of clinically relevant HA is higher from what was assumed 20 years ago. It affects approximately 1/1000 of the population. The most prevalent are prolactinomas and non-functioning adenomas. Acromegaly, Cushing's disease and aggressive tumors make for complex patients with increased morbidity and mortality. Early diagnosis and multimodal treatment provide a reasonable improvement in survival. Epidemiological study of clinically relevant HAs is important for estimating the impact on health systems. Conclusions: Higher-resolution imaging studies will continue to highlight pituitary incidentalomas. Careful evaluation of patients will identify clinically relevant HAs. (AU)

Radiographic findings in acromegaly: pictorial essay / Achados radiográficos da acromegalia: ensaio iconográfico

Abstract Acromegaly is an uncommon metabolic disorder, often diagnosed after a long delay. One symptom seen in many patients with acromegaly is arthralgia, a finding that calls for the use of conventional radiography, which can reveal subtle changes that can go unnoticed. The objective of this pictorial essay is to portray the radiographic aspects of acromegaly, seeking to demonstrate the importance of conventional radiography, which, despite its simplicity, can suggest the diagnosis, even in the early stages, thus altering the clinical course of the disease.

Resumo A acromegalia é um distúrbio metabólico pouco frequente, muitas vezes apresentando grande atraso diagnóstico. A artralgia é um dos sintomas mais comumente encontrados nos pacientes acromegálicos, motivando a realização de radiografias convencionais, as quais podem apresentar achados sutis que podem passar despercebidos. O objetivo deste ensaio é retratar os achados radiográficos da acromegalia, buscando demonstrar a importância deste método que, apesar de simples, pode sugerir o diagnóstico, mesmo em fases iniciais, alterando assim o curso clínico da doença.

[Left ventricular deformation: similarities and dissimilarities in elite sport activity- and acromegaly-related abnormalities. Results from the three-dimensional speckle-tracking echocardiographic MAGYAR-Sport and MAGYAR-Path Studies] / Bal kamrai deformitás: az aktív élsporthoz és az acromegaliához kötheto eltérések hasonlóságai és különbségei. Eredmények a háromdimenziós speckle-tracking echokardiográfiás MAGYAR-Sport és MAGYAR-Path Tanulmányokból

INTRODUCTION: Acromegaly is a chronic endocrine disorder, which produces a significant amount of human growth hormone and consequently insulin-like growth factor in adulthood due to a tumor in the pituitary gland. If left untreated, it can have a significant effect on the cardiovascular system. It is also known that elite sport activity is also associated with physiologic cardiac transformation, the so-called athletes' heart, in which volumetric and functional adaptation of the heart cavities can be observed. OBJECTIVE: In accordance with the above facts, the question may rightly arise as to what differences can be observed in left ventricular morphology and function in acromegaly, and what similarities and dissimilarities do the obtained results show compared to the values of healthy non-athlete adults compared to the values of the left ventricle of young elite athletes. METHOD: The present study comprised 21 elite athletes playing high dynamic sports (mean age: 31.2 ± 6.4 years, 13 males) and 18 acromegaly patients (mean age: 47.9 ± 8.9 years, 9 males). Their results were compared to those of 22 negative controls (mean age: 47.7 ± 10.6 years, 13 males). RESULTS: Left ventricle is dilated, but its function is preserved in both elite athletes and patients with acromegaly. While increased longitudinal and circumferential left ventricular strains could be seen in elite athletes primarily due to the more pronounced contractility of left ventricular apical segments, increased radial left ventricular strain was detected in acromegaly due to increased function of the left ventricular basal region. Left ventricular rotational mechanics show different patterns as well: while basal left ventricular rotation is decreased in elite athletes, apical left ventricular rotation showed a reduction leading to the significant deterioration of left ventricular twist in acromegaly. CONCLUSION: Both elite athletes playing high dynamic sports and acromegaly patients have dilated left ventricle, more pronounced regional left ventricular contractility and left ventricular rotational abnormalities with differences in nature and extent of these alterations as compared to those of non-sporting healthy subjects. Orv Hetil. 2023; 164(8): 308-316.

Complicaciones cardiovasculares asociadas a la acromegalia. Estado del arte / Cardiovascular complications associated with acromegaly. A state-of-the-art

Resumen La acromegalia es una enfermedad rara, causada principalmente por un tumor hipofisiario secretor de hormona de crecimiento (GH), se caracteriza por tener progresión lenta y asociarse a un compromiso multisistémico, siendo el aparato cardiovascular uno de los más comprometidos, llegando, incluso, desde hace más de 10 años a representar la principal causa de muerte. Las complicaciones cardiovasculares se desarrollan como resultado de las concentraciones sanguíneas elevadas de la GH y del factor de crecimiento similar a la insulina tipo 1 (IGF-1), los cuales ejercen efectos directos e indirectos sobre el endotelio, grandes vasos, riñón y los cardiomiocitos; produciendo hipertensión arterial, enfermedad valvular, arritmia cardiaca y una cardiopatía propia de la acromegalia denominada miocardiopatía acromegálica. Luego de la revisión bibliográfica actualizada relacionada con la fisiopatología, manifestaciones clínicas, diagnóstico y tratamiento del compromiso cardiovascular, encontramos que desde el punto de vista cardiovascular los pacientes con acromegalia pueden variar desde un estado asintomático hasta presentar disfunción cardiaca severa, siendo los marcadores bioquímicos y exámenes de imagen herramientas diagnósticas que permiten evaluar el grado de afección cardiovascular para poder brindar un tratamiento individualizado. La normalización de los valores de la GH y del IGF-1 mejora los parámetros cardiovasculares y, por tanto, su pronóstico.

Abstract Acromegaly is a rare disease, mainly caused by a pituitary tumor secreting growth hormone. It is characterized by slow progression and is associated with a multisystemic involvement, being the cardiovascular system, one of the most involved, even reaching, more than 10 years ago, to represent the main cause of death. Cardiovascular complications develop as a result of elevated blood concentrations of growth hormone and insulin-like growth factor 1, which exert direct and indirect effects on the endothelium, large vessels, kidney and cardiomyocytes; causing arterial hypertension, valve disease, cardiac arrhythmia and a specific heart disease called acromegalic cardiomyopathy. After the literature overview related to the pathophysiology, clinical manifestations, diagnosis and treatment of cardiovascular involvement, we found that from a cardiovascular point of view, patients with acromegaly can range from an asymptomatic state to severe cardiac dysfunction, being the biochemical markers and imaging studies diagnostic tools that allow assessment the degree of cardiovascular disease in order to provide individualized treatment. The normalization of growth hormone and insulin-like growth factor 1 levels improves cardiovascular parameters, and therefore its prognosis.

Complejo de Carney: reporte de un caso y revisión de la literatura / Carney complex: a case report and literature review

Resumen El complejo de Carney es una enfermedad caracterizada por lesiones en la piel, tumores endocrinos, cardiacos, gonadales y en otros órganos, que está asociada con mutaciones del gen PRKAR1A. Presentamos el caso clínico de una paciente con varias de las manifestaciones más características de este síndrome. Finalmente, se hace una revisión de la literatura.

Abstract Carney complex is a disease characterized by skin lesions, endocrine, cardiac, gonadal and other organ tumors, associated with mutations of the PRKAR1A gene. We present the clinical case of a patient with several of the most characteristic manifestations of this syndrome. Finally, there will be a review of the literature.

Acromegalia por macroadenoma hipofisiario en paciente con síndrome de McCune-Albright. Reporte de caso y revisión de la literatura / Acromegaly due to pituitary macroadenoma in a patient with McCune-Albright syndrome. Case report and literature overview

Introducción: El síndrome de McCune-Albright (SMA) es una enfermedad rara caracterizada por la triada: manchas cutáneas de color café con leche, displasia fibrosa poliostótica y pubertad precoz. Puede afectar a diversos ejes hormonales, entre ellos el de la hormona de crecimiento (GH), pudiendo asociarse a acromegalia. Reporte de caso: describimos el caso de una mujer de 44 años, con pubertad precoz periférica, hemorragia uterina anormal, crecimiento de manos y pies, prognatismo, prominencia frontal, manchas café con leche y tumoraciones pétreas en cara y antebrazos. Resultados: Apoyados con exámenes laboratoriales y de imágenes, se llegaron a los diagnósticos de acromegalia, hipogonadismo hipogonadotropo y síndrome de McCune-Albright. La paciente fue sometida a tratamiento quirúrgico con persistencia de enfermedad clínica y laboratorial. Conclusión: El diagnóstico y tratamiento oportunos de la acromegalia y sus complicaciones brindará un mejor pronóstico a los pacientes con SMA.

Background: McCune-Albright syndrome (MAS) is a rare disease characterized by the triad: café-au-lait skin spots, polyostotic fibrous dysplasia, and precocious puberty. It can affect various hormonal axes, including growth hormone (GH), and may be associated with acromegaly. We describe the case Case report:of a 44-year-old woman with peripheral precocious puberty, abnormal uterinebleeding, growthof thehands and feet, prognathism, frontal prominence, café-au-lait spots, and stony tumorsonthefaceandforearms.Supportedby Results:laboratory and imaging tests, the diagnoses of acromegaly, hypogonadotropic hypogonadism and McCune-Albright syndrome were reached. The patient underwent surgical treatment with persistence of clinical and laboratory disease. Conclusion: Timely diagnosis and treatment of acromegaly and its complications will provide a better prognosis for patients with MAS

Disfunção Ventricular e do Átrio Esquerdo Subclínica em Pacientes com Acromegalia: Um Estudo de Ecocardiograma de Rastreamento de Manchas / Subclinical Left Atrial and Ventricular Dysfunction in Acromegaly Patients: A Speckle Tracking Echocardiography Study

Resumo Fundamento Embora se saiba que a fração de ejeção (FE) do ventrículo esquerdo (VE) medida por eletrocardiograma seja preservada em pacientes com acromegalia, não há informação suficiente sobre deformação longitudinal global e deformação do átrio esquerdo (SLG-VE e SAE). Objetivo O objetivo deste estudo foi avaliar as funções do ventrículo esquerdo (VE) e do átrio esquerdo (AE) por ecocardiograma strain (ES) em pacientes com acromegalia. Métodos Este estudo incluiu 50 pacientes com acromegalia na forma ativa da doença e 50 controles saudáveis com idade, sexo e área de superfície corporal similares. Além dos ecocardiogramas de rotina, medições de SLG-VE e SAE foram realizadas com o ES. Resultados Os valores dos SAE e SLG-VE foram significativamente mais baixos em pacientes com acromegalia (p<0,05 para todos). Na análise bivariada, a pressão arterial sistólica, o pró-hormônio N-terminal do peptídeo natriurético cerebral, o fator de crescimento semelhante à insulina tipo 1, e detectou-se que os níveis de IMVE tinham correlação positiva com SAE e SLG-VE (p<0,05). O nível de IGF-1 tinha forte correlação com SAE e SLG-VE (p<0,001 e β=0,5 vs. p<0,001 e β=0,626, respectivamente); 48% dos pacientes com acromegalia têm SLG-VE reduzido (<20%). O índice de massa do ventrículo esquerdo (IMVE) determina independentemente a presença de SLG-VE reduzido, e cada 1g/m2 de aumento no nível de IMVE aumenta a probabilidade de redução de SLG-VE em 6%. Conclusão Embora a fração de ejeção de VE seja normal em pacientes com acromegalia, os valores de SAE e SLG-VE são significativamente mais baixos. Além do aumento em IMVE, outro achado do envolvimento cardíaco pode ser a redução de SAE e SLG-VE. Portanto, além do ecocardiograma de rotina, SAE e SLG-VE podem ser úteis para avaliar os sinais iniciais de envolvimento cardíaco antes da ocorrência de alterações cardíacas irreversíveis.

Abstract Background Although it is known that the left ventricular (LV) ejection fraction (EF) measured by echocardiography is preserved in patients with acromegaly, there is not enough information about the LV and left atrial strain (LV-GLS and LAS). Objective This study aimed to evaluate the left ventricular (LV) and left atrial (LA) functions with strain echocardiography (SE) in patients with acromegaly. Methods This study included 50 acromegaly patients with active disease and 50 healthy controls with similar age, gender, and body surface area. In addition to routine echocardiography examinations, LV-GLS and LAS measurements were performed with SE. Results LAS and LV-GLS values were significantly lower in patients with acromegaly (p<0.05 for all). In bivariate analysis, systolic blood pressure, N-terminal prohormone of brain natriuretic peptide, Insulin-like growth factor-1, LA diastolic diameter, and LVMI levels were found to be positively correlated with both LAS and LV-GLS (p <0.05). IGF-1 level was strongly correlated with LAS and LV-GLS (p<0.001 and β=0.5 vs. p<0.001 and β=0.626, respectively); 48% of patients with acromegaly have reduced LV-GLS (<20%). Left ventricular mass-index (LVMI) independently determines the presence of reduced LV-GLS and each 1g/m2increase in LVMI level increases the likelihood of reduced LV-GLS by 6%. Conclusion Although LV ejection fraction is normal in patients with acromegaly, LAS and LV-GLS values were significantly reduced. Apart from LVMI increase, another finding of cardiac involvement may be LAS and LV-GLS decrease. Therefore, in addition to routine echocardiography, LAS and LV-GLS may be useful to evaluate early signs of cardiac involvement before the occurrence of irreversible cardiac changes.

Gigantismo acromegaloide. Presentación de un caso

RESUMEN Tanto el gigantismo como la acromegalia pueden deberse a una secreción excesiva de hormona del crecimiento por parte de la hipófisis anterior. Cuando los afectados son jóvenes en edades próximas al cierre de los cartílagos se manifiestan signos y síntomas de gigantismo y de acromegalia. Se presenta el caso de un paciente masculino de 20 años de edad atendido en el Servicio de Endocrinología del Hospital Gustavo Aldereguia Lima de Cienfuegos con manifestaciones clínicas de alta talla, crecimiento acral e hipertensión arterial. Se le realizó prueba de tolerancia oral a la glucosa que midió hormona de crecimiento y confirmó el exceso hormonal. La resonancia magnética contrastada con gadolinio informó macroadenoma hipofisario. Se diagnosticó como gigantismo acromegaloide por un tumor hipofisario productor de hormona del crecimiento. Se realizó exéresis del macroadenoma, con evolución favorable. Es indiscutible la importancia del diagnóstico precoz de esta enfermedad, pues acorta la esperanza de vida, aumenta la mortalidad global no solo por la hipersecreción hormonal, sino también por el efecto de masa del tumor.

ABSTRACT Both gigantism and acromegaly can be due to excessive secretion of growth hormone by the anterior pituitary. When those affected are young, close to cartilage closure, signs and symptoms of gigantism and acromegaly appear. The case of a 20-year-old male patient treated in the Endocrinology Service of the Gustavo Aldereguia Lima Hospital in Cienfuegos with clinical manifestations of tall stature, acral growth and arterial hypertension is presented. An oral glucose tolerance test was performed that measured growth hormone and confirmed the excess of hormones. Gadolinium-enhanced MRI reported pituitary macroadenoma. It was diagnosed as acromegaloid gigantism due to a growth hormone-producing pituitary tumor. The macroadenoma was excised, with a favorable evolution. The importance of early diagnosis of this disease is indisputable, since it shortens life expectancy, increases global mortality not only due to hormonal hypersecretion, but also due to the mass effect of the tumor.

Gamma knife radiosurgery in pituitary adenomas. A single-center experience / Radiocirugía gamma knife en adenomas hipofisarios: experiencia en un centro

Abstract Stereotactic radiosurgery with gamma knife (GKS) is a treatment option for persistent or recurrent pituitary adenoma. The aim of our study was to report Argentine experience in GKS, assessing the efficacy and safety in our patients with pituitary adenomas. We performed a retrospective analysis of patients with pituitary adenomas treated with GKS between 2002 and 2017 in a single institution. Patient characteristics, biochemical remission rate (for functioning tumors), tumor control rate and adverse effects with GKS were inves tigated. The study cohort comprised 99 patients with a mean follow-up of 63 months: 51 somatotropinomas, 28 non-functioning adenomas, 15 corticotropinomas, 2 prolactinomas and 3 mixed pituitary tumors. The mean radiation dose was 30.6Gy for corticotropinomas, 29.3Gy for somatotropinomas and 19.6Gy for non-secreting adenomas. Global tumor control rate was 94.2%. Biochemical remission rate was 55.9%, being higher in acromegaly than in Cushing's disease (OR4.7, 95%Ci 2.1-10.4, p<0.0001). The mean time to remission was 29.5 months (range: 6-156). Hypopituitarism occurred in 26% of patients and those with Cushing's disease were more prone to develop new hormone deficiency after GKS (OR 2.93, 95%Ci 1.2-7.2, p = 0.019). This study shows argentine experience with the use of GKS in patients with pituitary adenomas, with similar results to those reported by centers with large radiosurgical experience. We achieved biochemical remission in more than 50% of patients and global tumor control in most of them. Hypopituitarism was the most frequent adverse effect, while others were infrequent.

Resumen La radiocirugía gamma-knife (GKS) constituye una alternativa de tratamiento en adenomas hipofisarios persis tentes o recurrentes. El objetivo de nuestro trabajo fue comunicar la experiencia argentina con GKS, evaluando eficacia y seguridad en nuestros pacientes con adenomas hipofisarios. Realizamos un análisis retrospectivo de pacientes con adenomas hipofisarios tratados con GKS entre 2002 y 2017. Se investigaron: características basales, tasa de remisión bioquímica, tasa de control tumoral y efectos adversos del GKS. Estudiamos 99 pa cientes adultos con un seguimiento medio de 63 meses: 51 somatotropinomas, 28 adenomas no funcionantes, 15 corticotropinomas, 2 prolactinomas y 3 tumores mixtos. La do sis media de radiación fue 30.6 Gy para cortico tropinomas, 29.3 Gy para somatotropinomas y 19.6 Gy para adenomas no secretores. La tasa global de control tumoral fue 94.2%. La tasa de remisión bioquímica fue 55.9%, si endo mayor en acromegalia que en enfermedad de Cushing (OR4.7, 95% Ci 2.1-10.4, p < 0.0001). El tiempo medio hasta la remisión fue 29.5 meses (rango: 6-156). Se produjo hipopituitarismo en el 26% de los pacientes. Aquellos con enfermedad de Cushing fueron más propensos a desarrollar nuevas deficiencias hormonales después del GKS (OR2.93, 95% Ci 1.2-7.2, p = 0.019). Este trabajo muestra la experiencia argentina con GKS en pacientes con adenomas hipofisarios, con resultados similares a los reportados por centros con amplia experiencia radioquirúrgica. Logramos remisión bioquímica en más del 50% de los pacientes y control tumoral global en la mayoría de ellos. El hipopituitarismo fue el efecto adverso más frecuente, mientras que otros fueron infrecuentes.

Acromegalia. A propósito de un caso

RESUMEN La acromegalia es un síndrome clínico producido por la secreción excesiva de hormona del crecimiento que afecta a prácticamente todos los órganos y tejidos. Tiene una baja incidencia (30 a 60 casos por millón de habitantes) y se requiere tener un alto nivel de sospecha por lo que su diagnóstico continúa siendo tardío a pesar de que físicamente los pacientes inician los signos y síntomas entre cinco y diez años previos al diagnóstico. Se presenta el caso de una paciente femenina, de 57 años, atendida en el Servicio de Endocrinología del Hospital Gustavo Aldereguia Lima de Cienfuegos con manifestaciones clínicas de crecimiento acral, hipertensión y diabetes mellitus. Se le realizó prueba de tolerancia oral a la glucosa midiendo hormona de crecimiento que confirmó el exceso hormonal, resonancia magnética contrastada con gadolinio que informó macroadenoma hipofisario. Se diagnosticó como acromegalia por un tumor hipofisario productor de hormona de crecimiento. Es indiscutible la importancia del diagnóstico precoz, dado que su asociación a comorbilidades como la hipertensión y la diabetes es muy importante, lo que resulta en una disminución de la esperanza y la calidad de vida. Estas razones motivaron la presentación del caso.

ABSTRACT Acromegaly is a clinical syndrome caused by excessive secretion of growth hormone that affects virtually all organs and tissues. It has a low incidence (30 to 60 cases per million inhabitants) and a high level of suspicion is required, which is why its diagnosis continues to be late despite the fact that patients physically initiate signs and symptoms between five and ten years prior to the diagnosis. A 57-years-old female patient treated at the Endocrinology Service of the Gustavo Aldereguia Lima Hospital in Cienfuegos with clinical manifestations of acral growth, hypertension and diabetes mellitus it is presented. She underwent an oral glucose tolerance test measuring growth hormone that confirmed hormonal excess, magnetic resonance imaging contrasted with gadolinium that reported pituitary macroadenoma. She was diagnosed as acromegaly from a growth hormone-producing pituitary tumor. The importance of early diagnosis is undoubted, since its association with comorbidities such as hypertension and diabetes is very important, which results in a decrease in hope and quality of life.

Enfermedad nodular tiroidea en personas con diagnóstico de acromegalia / Nodular thyroid disease in people diagnosed with acromegaly

La aparición de nódulos tiroideos en las personas con acromegalia es una consecuencia de la elevación crónica de la hormona de crecimiento y el factor de crecimiento similar a la insulina tipo 1. Su naturaleza varía según la zona geográfica, suficiencia de yodo y antecedentes patológicos familiares, entre otros factores. No se han publicado estudios cubanos sobre la enfermedad nodular tiroidea en estas personas. Objetivos: Describir las características clínicas, bioquímicas y ultrasonográficas de la glándula tiroidea, según la presencia o no de la enfermedad nodular tiroidea. Métodos: Estudio observacional descriptivo, transversal, que incluyó 73 pacientes con acromegalia entre enero de 2003 y diciembre de 2017. Se estudiaron las variables: edad, sexo, color de la piel, antecedentes familiares de la enfermedad nodular tiroidea, niveles de la hormona de crecimiento, hormona estimulante del tiroides, T4 libre, anticuerpos contra la peroxidasa tiroidea y contra la tiroglobulina, volumen tiroideo, patrón ecográfico nodular y estudio citológico. Resultados: La enfermedad nodular tiroidea se presentó en el 75,3 por ciento de los casos, con predominio del bocio multinodular. La edad al diagnóstico fue menor en los pacientes con la enfermedad (43,53 ± 9,67), que en los que no la tenían (49,33 ± 6,96 años) (p = 0,02). La hormona de crecimiento al diagnóstico de acromegalia, resultó menor en los pacientes con este padecimiento (18,73 ± 11,33 µg/L vs. 35,91 ± 21,68 µg/L; (p = 0,00). El volumen tiroideo mostró diferencias significativas entre ambos grupos (14,2 ± 4,5 mL en los casos positivos de la enfermedad nodular tiroidea y 10,5 ± 2,8 mL en los casos negativos; p = 0,002), siendo el nódulo de baja sospecha de malignidad el más frecuente. El resto de las variables resultaron similares entre los pacientes con y sin la enfermedad. La citología se informó como benigna en el 75 por ciento en los nódulos únicos, el 80 por ciento de los bocios nodulares y el 90 por ciento de los bocios multinodulares (p = 0,51). Conclusiones: La enfermedad nodular tiroidea fue frecuente en los casos de acromegalia, y se asoció a la menor edad y los niveles inferiores de la hormona de crecimiento al diagnóstico. El bocio multinodular constituyó la forma clínica más frecuente y los parámetros hormonales y de autoinmunidad no se asociaron al tipo de la enfermedad nodular tiroidea(AU)

The appearance of thyroid nodules in people with acromegaly is a consequence of chronic elevation of growth hormone (GH) and insulin-like growth factor type 1 (IGF-1). Its nature varies according to the geographical area, the iodine sufficiency and family pathological history, among other factors. No Cuban studies on thyroid nodular disease (TND) in these people have been published. Objectives: Describe some clinical characteristics, as well as biochemical and ultrasonographic ones related to the thyroid gland, according to the presence or not of TND, and to identify the possible association of clinical, biochemical, ultrasonographic and cytological factors with the different types of TND in patients with acromegaly. Methods: A descriptive, cross-sectional observational study that included 73 patients with acromegaly between January 2003 and December 2017. The following variables were studied: age, sex, skin color, family history of TND, GH levels, thyroid stimulating hormone, free T4, antibodies against thyroid peroxidase and thyroglobulin, thyroid volume, nodular ultrasound pattern and cytological study. Results: TND occurred in 75.3 percent of cases, with a predominance of multinodular goiter. The age at diagnosis time was lower in patients with TND (43.53 ± 9.67) than in those who did not have it (49.33 ± 6.96 years) (p=0.02). GH at diagnosis time of acromegaly was lower in patients with TND (18.73±11.33µg/L vs 35.91±21.68µg/L; (p=0.00). The thyroid volume showed significant differences between both groups (14.2±4.5mL in positive cases of TND and 10.5±2.8mL in negative cases; p=0.002), being the most frequent the nodule with low suspicion of malignancy. The rest of the variables were similar between patients with and without TNDs. Cytology was reported as benign in 75 percent in single nodules, 80 percent of nodular goiters and 90 percent of multinodular goiters (p=0.51). Conclusions: TND was frequent in cases of acromegaly, and was associated with lower age and lower GH levels at diagnosis time. Multinodular goiter was the most frequent clinical form and hormonal and autoimmunity parameters were not associated with the type of TND(AU)

Endoscopic endonasal lateral transellar approach for growth hormone-secreting adenomas with cavernous sinus invasion: Technical note and surgical results / Abordaje transelar lateral endonasal endoscópico para adenomas hipofisiarios productores de hormona del crecimiento con invasión al seno cavernoso: nota técnica y resultados quirúrgicos

Background Cavernous sinus (CS) invasion is found in 15–20% of pituitary adenomas; it represents a poor prognosis factor and a surgical challenge even in experienced pituitary centers. We present our experience and technical note description for surgical management of pituitary adenomas with CS invasion in acromegaly by the transsellar lateral approach with an endoscopic endonasal transsphenoidal route. Method prospective case series of patients who underwent endoscopic endonasal surgery for Growing Hormone (GH) producing adenomas with CS invasion treated at the Neurosurgery departments of National Institute of Neurology and Neurosurgery in Mexico City, and of Toluca Medical Center of Social Security Institute of the State of Mexico and Provinces between January 2014 and March 2019. Results Thirty-two of 94 patients with diagnosis of pituitary adenoma treated at our institutions (34%) had acromegaly; thirteen of patients with acromegaly diagnosis met the inclusion criteria for CS invasion. Postoperative images reported gross total resection in 10 patients (76.9%). Mean follow-up time was 28.3 months. Remission criteria were achieved in nine patients (69.2%), with one of these patients (11.1%) having recurrence during follow up. All patients with no biochemical remission had improvement in GH and IGF profiles. Three patients without remission underwent radiosurgery (14Gy), and one patient had remission after the procedure. Conclusions We consider this to be a safe and efficient approach for tumors invading CS, when surgical team have good experience in endoscopy of the skull base and reconstruction techniques, appropriate instruments are available, and tumor has soft consistency (AU)

Antecedentes Se encuentra invasión al seno cavernoso en el 15-20% de los adenomas hipofisiarios, y representa un factor de mal pronóstico, así como un reto quirúrgico en algunas series. En el presente trabajo describimos y evaluamos nuestra experiencia con una técnica quirúrgica para el manejo de adenomas hipofisiarios con invasión al seno cavernoso en pacientes con acromegalia a través de un abordaje transelar lateral por la vía endonasal endoscópica. Métodos Serie de casos prospectiva de pacientes tratados con cirugía endonasal endoscópica para pacientes con diagnóstico de acromegalia y adenomas hipofisiarios con invasión al seno cavernoso tratados en los Departamentos de Neurocirugía del Instituto Nacional de Neurología y Neurocirugía en la Ciudad de México y en el Centro Médico Toluca del Instituto de Seguridad Social del Estado de México y Municipios entre enero del 2014 y marzo del 2019. Resultados Treinta y dos pacientes de 94 con diagnóstico de adenoma hipofisiario fueron tratados en nuestras instituciones, cumplieron con criterios clínicos y bioquímicos para el diagnóstico de acromegalia (34%); de estos, 13 pacientes cumplieron con criterios por imagen y quirúrgicos de invasión al seno cavernoso (40,6%). Las imágenes postoperatorias mostraron resección completa en 10 pacientes (76,9%). El tiempo medio de seguimiento fue de 28,3 meses. Se alcanzó un índice de remisión bioquímica en el 69,2% de los casos, con un paciente presentando recurrencia durante el seguimiento. Tres de los pacientes sin remisión bioquímica fueron sometidos a radiocirugía, con respuesta terapéutica en uno de ellos (AU)

Endoscopic endonasal lateral transellar approach for growth hormone-secreting adenomas with cavernous sinus invasion: Technical note and surgical results.

BACKGROUND: Cavernous sinus (CS) invasion is found in 15-20% of pituitary adenomas; it represents a poor prognosis factor and a surgical challenge even in experienced pituitary centers. We present our experience and technical note description for surgical management of pituitary adenomas with CS invasion in acromegaly by the transsellar lateral approach with an endoscopic endonasal transsphenoidal route. METHOD: prospective case series of patients who underwent endoscopic endonasal surgery for Growing Hormone (GH) producing adenomas with CS invasion treated at the Neurosurgery departments of National Institute of Neurology and Neurosurgery in Mexico City, and of Toluca Medical Center of Social Security Institute of the State of Mexico and Provinces between January 2014 and March 2019. RESULTS: Thirty-two of 94 patients with diagnosis of pituitary adenoma treated at our institutions (34%) had acromegaly; thirteen of patients with acromegaly diagnosis met the inclusion criteria for CS invasion. Postoperative images reported gross total resection in 10 patients (76.9%). Mean follow-up time was 28.3 months. Remission criteria were achieved in nine patients (69.2%), with one of these patients (11.1%) having recurrence during follow up. All patients with no biochemical remission had improvement in GH and IGF profiles. Three patients without remission underwent radiosurgery (14Gy), and one patient had remission after the procedure. CONCLUSIONS: We consider this to be a safe and efficient approach for tumors invading CS, when surgical team have good experience in endoscopy of the skull base and reconstruction techniques, appropriate instruments are available, and tumor has soft consistency.

Mitral annulus is dilated with preserved function in acromegaly regardless of its activity: Insights from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

INTRODUCTION AND OBJECTIVE: Acromegaly is a rare, chronic and slowly developing endocrine disorder caused by hypersecretion of human growth hormone and consequently of insulin-like growth factor-1 during adulthood. The present study was conducted to assess mitral annular (MA) size and function between acromegalic patients and age- and gender-matched healthy controls by three-dimensional speckle-tracking echocardiography (3D-STE). It also aimed to examine whether activity of the disease has any effect on MA parameters. METHODS: This study included 27 patients with acromegaly, three of whom were excluded due to inferior image quality. The mean age of the remaining 24 patients was 55.7±14.0 years and seven were male. Complete two-dimensional Doppler echocardiography and 3D-STE were performed in all cases. RESULTS: Significantly increased end-diastolic and end-systolic MA diameter (2.81±0.36 cm vs. 2.44±0.34 cm and 2.00±0.32 cm vs. 1.65±0.37 cm, respectively), area (9.67±2.33 cm2 vs. 7.38±1.93 cm2 and 5.14±1.62 cm2 vs. 3.74±1.19 cm2, respectively) and perimeter (11.76±1.42 cm vs. 10.27±1.33 cm and 8.61±1.23 cm vs. 7.36±1.10 cm, respectively) were demonstrated in acromegalic patients compared with control subjects. MA functional parameters were not significantly altered compared to those of healthy individuals. CONCLUSIONS: MA dilation could be seen in acromegaly regardless of its activity. Acromegaly is not associated with MA functional impairment.

Acromegalia en Paciente con Cáncer Papilar de Tiroides. Reporte de caso / Acromegaly in a patient with papillary thyroid cancer. Case report

Introducción: El cáncer de tiroides es la neoplasia endocrina más frecuenteyel Carcinoma Papilar de tiroides representa el 80% de los casos. Por otro lado, la acromegalia es un trastorno poco diagnosticado con una incidencia estimada anual de 4 casos por millón de personas. Presentamos un caso en el que se presentan las dos entidades al mismo tiempo en un paciente y la discusión del posible nexobio químico. Caso clínico: Mujer de 23 años, con masa nodular pétrea de 3 centímetros, en polo superior de lóbulo derecho de tiroides; sometida a tiroidectomía radical y linfadenectomía cervical bilateral, reporte histopatológico de carcinoma papilar. Evoluciona con cambios visuales en ojo izquierdo, cefalea, amenorrea de 1 año, galactorrea negativa, características morfo-anatómicas de acromegalia, campimetría hemianopsia homónima izquierda. IRM de cerebro lesión sellar y suprasellar hiperintensa en T2 en relación a macroadenoma hipofisario. Bioquímicos: Prolactina 131.20, GH 2.7, ACTH 18.5, IGF1 434, IGFBP 35.8, TTOG para GH que se informa: GH 0 min: 10.7, GH 30 min: 9.24, GH 60 min: 7.9. Evolución: La paciente fue sometida a hipofisectomía transesfenoidal endoscópica parcial. En tratamiento con cabergolina 0.5 mg bisemanal, levotiroxina 225 mcg día y octreótide 20 mg mensual. Conclusión: Se concluye que siendo la acromegalia una enfermedad rara se asocia a cáncer de tiroides, implicando posibles mutaciones en la subunidad α de la proteína G.

Introduction:Thyroid cancer is the most frequent endocrine neoplasia and Papillary thyroid carcinoma represents 80% of cases. On the other hand, acromegaly is a poorly diagnosed disorder with an estimated annual incidence of 4 cases per million people. We present a case in which the two entities occur at the same time in a patient and the discussion of the possible biochemical link. Clinical case: 23-year-old woman, with a 3 cm stone nodular mass in the upper pole of the right thyroid lobe; submitted to radical thyroidectomy and bilateral cervical lymphadenectomy, histopathological report of papillary carcinoma. It evolves with visual changes in the left eye, headache, 1-year amenorrhea, negative galactorrhea, morpho-anatomical characteristics of acromegaly,left homonymous hemianopia campimetry. T2 hyperintense seal and suprasellar lesion brain MRI in relation to pituitary macroadenoma. Biochemicals: Prolactin 131.20, GH 2.7, ACTH 18.5, IGF1 434, IGFBP 35.8, TTOG for reported GH: GH 0 min: 10.7, GH 30 min: 9.24, GH 60 min: 7.9. Evolution: The patient underwent partial endoscopic transsphenoidal hypophysectomy. In treatment with cabergoline 0.5 mg twice weekly, levothyroxine 225 mcg day and octreotide 20 mg monthly. Conclusion: It is concluded that, being acromegaly a rare disease, it is associated with thyroid cancer, involving possible mutations in the α subunit of the G protein.

Endoscopic endonasal lateral transellar approach for growth hormone-secreting adenomas with cavernous sinus invasion: Technical note and surgical results.

BACKGROUND: Cavernous sinus (CS) invasion is found in 15-20% of pituitary adenomas; it represents a poor prognosis factor and a surgical challenge even in experienced pituitary centers. We present our experience and technical note description for surgical management of pituitary adenomas with CS invasion in acromegaly by the transsellar lateral approach with an endoscopic endonasal transsphenoidal route. METHOD: prospective case series of patients who underwent endoscopic endonasal surgery for Growing Hormone (GH) producing adenomas with CS invasion treated at the Neurosurgery departments of National Institute of Neurology and Neurosurgery in Mexico City, and of Toluca Medical Center of Social Security Institute of the State of Mexico and Provinces between January 2014 and March 2019. RESULTS: Thirty-two of 94 patients with diagnosis of pituitary adenoma treated at our institutions (34%) had acromegaly; thirteen of patients with acromegaly diagnosis met the inclusion criteria for CS invasion. Postoperative images reported gross total resection in 10 patients (76.9%). Mean follow-up time was 28.3 months. Remission criteria were achieved in nine patients (69.2%), with one of these patients (11.1%) having recurrence during follow up. All patients with no biochemical remission had improvement in GH and IGF profiles. Three patients without remission underwent radiosurgery (14Gy), and one patient had remission after the procedure. CONCLUSIONS: We consider this to be a safe and efficient approach for tumors invading CS, when surgical team have good experience in endoscopy of the skull base and reconstruction techniques, appropriate instruments are available, and tumor has soft consistency.