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)

Hereditary pituitary tumor syndromes: genetic and clinical aspects

ABSTRACT The pituitary gland is responsible for the synthesis and secretion of various hormones that play a key role in regulating endocrine function and homeostasis. Pituitary adenomas (PA) are benign epithelial tumors arising from the endocrine cells of the anterior pituitary gland. Clinically relevant PA are relatively common and they occur in 0.1% of the general population. They are mostly benign monoclonal neoplasms that arise from any of the five hormone-secreting cell types of the anterior pituitary gland. PA are categorized as either functioning or non-functioning, depending on whether or not they produce a hormonal hypersecretion syndrome. Both functioning and non-functioning adenomas can produce symptoms or signs resulting from compression of the optic chiasm or invasion of cavernous sinuses. Only 5% of PA occur within the context of hereditary syndromes with reasonably well-defined oncogenic mechanisms. The vast majority of PA are sporadic, and their etiopathogenesis remains largely unknown. Pituitary tumor oncogenesis involves several mechanisms that eventually lead to abnormal cell proliferation and dysregulated hormone production. Among these factors, we found inactivating mutations of tumor suppressor genes, activating mutation of oncogenes and the participation of hormonal signals coming from the hypothalamus, all resulting in cell-cycle regulation abnormalities. In this review, we summarize the clinical and pathophysiological aspects of the different hereditary pituitary tumor syndromes.

IRM des pathologies sellaires et supra sellaires à Abidjan: à propos de 65 cas

Objectif : Déterminer la prévalence des pathologies hypophysaires retrouvées au décours des IRM réalisées au service de Radiodiagnostic et Imagerie Médicale du CHU de Treichville.Matériel et méthode : il s'agissait d'une étude rétrospective descriptive effectuée sur une période de 48 mois allant de janvier 2011 à décembre 2014 dans le service de Radiodiagnostic et Imagerie Médicale du CHU de Treichville. Cette étude était basée sur l'analyse des comptes rendus d'IRM hypophysaire effectués chez des patients qui étaient admises dans le service.Résultats : Ces patients étaient majoritairement de sexe féminin avec un sex ratio de 0,51. Leur âge variait de 18 à 61 ans avec une moyenne de 32,5 ans. Sur 638 IRM crânio encéphaliques qui avaient été réalisées, nous avions identifié 65 cas de pathologies hypophysaires soit 10,18% de l'ensemble des pathologies crânio encéphaliques. Il s'agissait essentiellement d'adénomes dans 92% des cas. Parmi les adénomes hypophysaires, les macroadénomes prédominaient soit 57%. Les céphalées étaient les indications les plus rencontrées. L'extension des adénomes hypophysaires se faisait au niveau des sinus sphénoïdaux, des sinus caverneux et au niveau du chiasma optique. Le sinus caverneux était atteint dans 57,14% et le chiasma optique dans 71,43% des cas.Conclusion : Les pathologies hypophysaires représentaient environ 10% des pathologies crânio encéphaliques et étaient dominées par les causes tumorales notamment les adénomes. L'IRM reste la méthode d'Imagerie de référence dans l'exploration des pathologies sellaires et supra sellaires notamment pour le diagnostic positif et pour le bilan d'extension

Incidence and prevalence of clinically relevant pituitary adenomas: retrospective cohort study in a Health Management Organization in Buenos Aires, Argentina

ABSTRACT Objectives The main purpose of this study was to estimate the incidence rate and prevalence of clinically relevant pituitary adenomas (PAs) within the Hospital Italiano Medical Care Program (HIMCP), a well-defined population of 150,000 members living in the urban and suburban area of the city of Buenos Aires. We defined clinically relevant PAs as those associated with endocrine dysfunction and/or mass effect. Subjects and methods A retrospective open cohort study was conducted, including all members of the HIMCP over 18 years old, with active memberships during the period of the study, from January 1st 2003, to January 1, 2014. The incidence rates (IRs) were standardized (SIR) to the World Health Organization (WHO) 2000 standard population and were expressed per 100,000 members/year. Prevalence was estimated at January 1, 2014, and was expressed per 100,000 persons. The clinical records have been electronically managed since 2001. All lab and imaging studies were done in-house. Results The overall SIR was 7.39/100,000/year (95% CI 4.47-10.31). Female patients had a specific IR significantly higher than male patients (5.85 vs.1.54) and represented 73% of the affected members. Regarding tumor size, 61.4% were microadenomas, and the mean age at diagnosis was 46.4 years. Prolactinomas had the highest SIR (5.41), followed by acromegaly (Acro) and non-functioning adenomas (NFAs) with overlapping 95% CIs (0.44-1.41 and 0.31-0.99, respectively). Microprolactinomas were more frequent in female (72.6%) (p < 0.01) and younger members (38 vs.60 years; p < 0.04). The overall prevalence rate was 97.76/100,000. Prolactinomas had the highest prevalence (56.29), followed by NFAs (21.48), Acro (14.07) and CD (5.93). Conclusion Our results demonstrate that clinically relevant PAs are more common than usually suspected, especially prolactinomas and growth-hormone secreting PAs. These data highlight the need to increase the awareness of PAs, thereby enabling early diagnosis and treatment.

Pituicitoma hipofisario, un hallazgo diagnóstico inusual: reporte de caso / Pituitary pituicytoma, finding of an unusual diagnosis: a case report

Los pituicitomas son tumores infrecuentes de la región selar y supraselar, originados de la neurohipófisis, que suelen ser confundidos con otros tumores al compartir características imaginológicas. Se reporta el caso de una mujer de 36 años de edad, con un pituicitoma en la región selar, que fue diagnosticado como un adenoma hipofisario debido a los hallazgos clínicos e imaginológicos preoperatorios y se realiza una revisión de la literatura.

The Pituicytomas are rare tumors of the sellar and suprasellar region originated of the neurohypophysis and are usually confused with other tumors when sharing imagining features. It is reported a case of a 36 years old woman with pituicytoma in the sellar region that was diagnosed as a pituitary adenoma due to the presurgical clinical and imaging findings and, it is revised the literature.

Historical distribution of central nervous system tumors in the Mexican National Institute of Neurology and Neurosurgery / Distribución histórica de neoplasias del sistema nervioso central en el Instituto Nacional de Neurología y Neurocirugía de México

Abstract Objective: To determine the frequency of central nervous system (CNS) tumors in the first fifty years of the National Institute of Neurology and Neurosurgery of Mexico Manuel Velasco Suárez (Instituto Nacional de Neurología y Neurocirugía de México, INNN) from 1965 to 2014. Materials and methods: A total of 16 116 institutional records of CNS tumors were analyzed. The frequency and distribution of CNS tumors were evaluated by tumor type, patient age and patient gender. The annual relationship between CNS tumors and surgical discharges (SD) over the last 20 years was estimated. Results: The frequencies of most CNS tumors were consistent with those found worldwide, and the most common tumors were neuroepithelial tumors (33%), particularly astrocytic tumors (67%); meningeal tumors (26%); and pituitary tumors (20%). The incidence of pituitary tumors in these data was twice as high as that reported in other regions of the world, and the relationship between CNS tumors and SD was consistent over time (0.22-0.39). Conclusion: This study summarizes the largest sample of CNS tumor cases analyzed in Mexico and provides an important reference of the frequency of this tumor type in the country. This work will serve as a basis for conducting studies evaluating factors associated with the presence of CNS tumors and for identifying adequate public health interventions.

Resumen Objetivo: Determinar la frecuencia de neoplasias del sistema nervioso central (NSNC) en los primeros 50 años del Instituto Nacional de Neurología y Neurocirugía de México (INNN). Material y métodos: Se analizaron 16 116 registros institucionales de las NSNC, atendidas en el INNN de 1965 a 2014; se estimó su frecuencia y distribución por tipo de neoplasia, edad y género, y se determinó la relación anual de NSNC y egresos quirúrgicos (EQ) en un período de 20 años. Resultados: Las frecuencias de la mayoría de NSNC fueron consistentes con las encontradas a nivel mundial. Las más frecuentes fueron las neuroepiteliales (33%), entre las cuales destacaron las astrocíticas (67%); meníngeas (26%), e hipofisiarias (20%). El número de neoplasias hipofisiarias en esta serie fue dos veces mayor al reportado en otras regiones del mundo y la relación NSNC/EQ fue similar a través del tiempo (0.22-0.39). Conclusión: Ésta es la mayor serie de casos de NSNC analizados en México y proporciona un referente importante sobre la frecuencia de este tipo de neoplasias en el país. Este trabajo servirá de base para llevar a cabo estudios de los factores asociados a la presencia de NSNC e identificar intervenciones de salud pública adecuadas.

Prevalence of pituitary incidentaloma in the Iranian cadavers.

Aim: Although one-fourth of the pituitary tumors are inactive and silent, increased total volume of the pituitary gland exerts pressure on sella turcica and corrodes the clinoid processes, resulting in several problems. Therefore, determination of the prevalence of the concealed pituitary tumors is of major concern among clinical practitioners. This study was performed to determine the prevalence of these tumors in cadavers referred to the Iranian Legal Medicine Organization (Kahrizak, Tehran). Materials and Methods: This cross-sectional descriptive study was performed between June 2007 and February 2008. A total of 485 cadavers were selected by simple random sampling method. Pituitary glands were removed and then horizontally cut and four slides were prepared from each of them, stained by hematoxylin and eosin, and evaluated by a light microscope. For statistical analysis, SPSS software (version 16), Mann-Whitney U test, Kolmogorov-Smirnov nonparametric test (K-S test), and Chi-square test were used. Results: Of the 485 investigated cadavers, 365 (75.3%) were males with an average age of 42 ± 20.5 years and 120 (24.7%) were females with an average age of 44 ± 22 years. A total of 61 (12.6%) had concealed pituitary masses. No statistically significant difference was found between the mean age, sex, and body mass index (BMI) of the cadavers with and without concealed tumors (P=0.380 P=0.450, and P=0.884, respectively). Conclusions: In the present study, the prevalence of the concealed pituitary adenomas was 12.6%. There was no correlation between age, sex, and BMI and the prevalence of the concealed masses.

Frequency of central nervous system tumors in delta region, Egypt.

Introduction and Aim of Work: Central nervous system (CNS) tumors represent a major public health problem, and their epidemiological data in Egypt have been rather incomplete except for some regional reports. There are no available frequency-based data on CNS tumors in our locality. The objective of this study was to estimate the frequency of CNS tumors in east delta region, Egypt. Materials and Methods: The data were collected during the 8-year period from January 1999 to December 2007 from Pathology Department, Mansoura University, and other referred pathology labs. Examination of HandE stained sections from retrieved paraffin blocks were done in all cases for histopathologic categorization of C.N.S. tumors. Immunohistochemical studies were applied to confirm final histopathologic diagnosis in problematic cases. Results: Intracranial tumors represented 86.7% of cases in comparison to only 13.3% for spinal tumors. Gliomas were the CNS tumors of the highest frequency (35.2%), followed by meningioma (25.6%), pituitary adenoma (11.6%) and nerve sheath tumors (6.6%). 10.25% of tumors were of children <15 years. Conclusion: This study provides the largest series of the relative frequency of CNS tumors in Delta region in Egypt till now and may help to give insight into the epidemiology of CNS tumors in our locality.

Tumores hipofisarios en la tercera edad / Pituitary tumors in the elderly

La prevalencia de los tumores hipofisarios en la tercera edad se encuentra en aumento en relación a la mayor expectativa de vida de la población general. Nosotros evaluamos retrospectivamente, 36 pacientes de la tercera edad, con media de 71 años, (65 -85), 20 de ellos varones, con tumores hipofisarios. El adenoma hipofisario no funcionante (ANF) se observó en el 64% de los casos, seguido por la acromegalia, el prolactinoma, y por un caso de enfermedad de Cushing. Se suman 3 casos de hipofisitis linfocitiaria, craneofaringioma y quiste de la bolsa de Rathke respectivamente. El 89% de los tumores fueron macroadenomas y la forma de presentación más frecuente, los trastornos visuales, seguidos por el hallazgo incidental y la hipersecreción hormonal. En la evaluación bioquímica la mayoría presentaba diferentes grados de hipopituitarismo, y los pacientes con macroprolactinoma tenían PRL > 800 ng/ml. Se operaron 21 pacientes (58%), 9 recibieron radioterapia. En 3 pacientes que no recibieron ningún tipo de tratamiento hubo modificación del tamaño del adenoma (2 aumentaron y 1 disminuyó) durante el seguimiento de 3.6 años. Se indicó tratamiento médico en aquellos que persistieron con la enfermedad o como tratamiento primario si las comorbilidades imposibilitaban la cirugía. No hubo mejoría de la función hipofisaria luego de la cirugía y el 62% agregaron uno o más déficit, el campo visual mejoró en el 67%. La diabetes insípida definitiva como complicación sólo se observó en dos pacientes y hubo un caso de fístula y meningitis. En conclusión, nosotros reportamos una tasa menor a la bibliografía mundial en la prevalencia de adenomas hipofisarios no funcionantes y similar para el resto, con una mayor tasa de presentación incidental. La cirugía no tuvo más complicaciones por la edad de los pacientes, el seguimiento clínico y con imágenes de aquellos que no se operan y/o el tratamiento médico puede ser una opción en este grupo etario.

World population has been growing and aging; and pituitary tumors prevalence among the elderly has also increased, summing up to 10-15% of all pituitary tumors. Thirty six elderly patients with pituitary tumors, 20 of them male, were evaluated restrospectively (average age 71 years, range 65-85). A non-functioning adenoma (NFA) prevalence was reported in 64% of the cases, followed by acromegaly, prolactinoma and one each of Cushing´s disease, lymphocytic hypophysitis, craniopharyngioma, and Rathke's pouch cyst. Macroadenomas accounted for 89% of tumors and the most frequent presentation was through visual disorders symptoms followed by incidental finding and hormone hypersecretion. During biochemical evaluation, most patients presented different degrees of hypopituitarism, and patients with macroprolactinoma had PRL levels>800ng/ml. Twenty one patients (58%) underwent surgery, while 9 underwent radiotherapy. During the 3.6 years follow-up, changes in adenoma size were reported in 3 patients who received no treatment (in 2 cases size increased, whereas in another it decreased). Medical treatment was indicated for those whose pathology persisted or as primary treatment if comorbilities prevented from performing a surgery. No pituitary function improvement was detected after surgery and the appearance of one or more deficiency was noticed in 62% of patients, though visual field did improve in 67%. Definitive diabetes insipidus as a complication was only detected in two patients, and a case of fistula and meningitis was observed. As a conclusion, we reported a NFA prevalence rate smaller than that reported in the world literature, though similar for the other pituitary tumors, and a higher rate of incidental presentation. No complications attributable to patients' age group were detected during surgery. For elderly patients who do not undergo surgery, clinical and imaging follow-up and/or medical treatment could be an option of choice.

Tumores de hipófise: considerações sobre 120 casos e revisão de literatura / Pituitary tumors: considerations about 120 cases and literature review

Objetivo: Estudo retrospectivo dos pacientes com adenoma hipofisário atendidos no Serviço de Neurocirurgia do Conjunto Hospitalar do Mandaqui de São Paulo, SP. Pacientes e métodos: Foram estudados, retrospectivamente, 120 casos de tumores de hipófise atendidos entre janeiro de 1993 e maio de 2006. Os dados analisados foram idade, sexo, queixa principal, apresentação clínica, classificação radiológica, tipo histológico e complicações. Resultados: A idade dos pacientes variou de 5 a 69 anos (média de 40,2 anos). Foram 89 pacientes do sexo feminino e 31 do sexo masculino. O déficit visual foi a queixa principal e presente em 52% dos pacientes. Com relação à classificação radiológica: 5,4%foram classificados como Hardy II; 8,1% como Hardy III; 86,5% como Hardy IV. A via de acesso foi transesfenoidal sublabial em 113 pacientes. Apenas três pacientes necessitaram reabordagem do tumor por craniotomia e sete pacientes foram submetidos à craniotomia sem tentativa transesfenoidal.Os tumores foram diagnosticados como não-secretores em 21,6% dos pacientes, prolactinomas em70,2% dos pacientes, secretor de GH em 5,4% e de ACTH e LH em 2,7% cada um. As principais complicações do tratamento cirúrgico foram diabetes insípido e fístula liquórica. Houve quatro óbitos no pós-operatório. Conclusões: A média etária foi de 40,2 anos; o sexo feminino foi mais acometido;a perda visual foi a principal queixa; a maioria dos pacientes se apresentou em grau IV da classificação de Hardy; o principal tipo histológico foi o prolactinoma; a via transesfenoidal sublabial foi suficiente para a maioria dos tumores.

Objective: Retrospective study of 120 cases of pituitary adenomas treated at Serviço de Neurocirurgiado Conjunto Hospitalar do Mandaqui de São Paulo, SP, Brazil. Patients and methods: 120 cases ofpituitary tumors treated between January, 1993 and May, 2006 were studied retrospectively. The data analyzed were: age, sex, main complaint, clinical presentation, radiological classification, type of hormone and complications. Results: The age of the patients varied between five to sixty-nine years (average of 40.2 years). The female/male ratio was 89:31. The visual deficit was the main complaint and present in 52% of the patients. According to Hardy’s classification, 5.4% were classified as Hardy II; 8.1% as Hardy III and 86.5% as Hardy IV. The surgical access was the transphenoidal in 113 patients. Three patients needed a second approach by craniotomy and seven patients were submitted to craniotomy as the first choice. The tumors were diagnosed as not secretor in 21.6% patients, prolactinomas in 70.2% patients, secretor of GH in 5.4% and ACTH and LH in 2.7% each one. The main complications of the surgical treatment were diabetes insipidus and cerebrospinal fluid leak. There were four deaths in the postoperative period. Conclusions: In this series the average age at presentations was 40.2 years;females were predominant; the visual loss was the main complaint; the majority of the patients were classified as Hardy IV; prolactinoma predominated; the transphenoidal surgical access was enough for the majority of the tumors.

Craniofaringiomas: achados clínicos, epidemiológicos e anatomopatológicos de 25 casos / Craniopharyngiomas: clinical, epidemiological and pathological findings of 25 cases

Relatamos os achados clínicos, epidemiológicos e anatomopatológicos de 25 casos de craniofaringiomas. Dos 25 pacientes analisados, 14 ocorreram em pacientes do sexo masculino e 11 em pacientes femininos. As idades variaram de 3 a 64 anos, com média de 30,52 anos. Quanto à localização, 12 casos foram supra-selares, 10 ocorreram em região selar, sendo que 8 apresentaram extensão supra-sela, dois localizaram-se em lobo frontal e 1 em região ponto-cerebelar. Os sinais e sintomas observados incluiram alterações visuais (72 por cento dos pacientes), cefaléia (68 por cento), vômitos (40 por cento) e edema de papila (24 por cento), com período de evolução variando de 18 dias a 60 meses, com média de 11,66 meses. Treze pacientes foram submetidos a ressecção cirúrgica parcial, 11 a exérese total e 1 a ressecção parcial associada a quimioterapia adjuvante. Evidenciou-se taxa de recidiva tumoral total de 48 por cento. Sete recidivas tumorais ocorreram nos pacientes submetidos apenas a exérese parcial, quatro nos pacientes submetidos a tumorectomia total e 1 no submetido a tratamento cirúrgico associado a quimioterapia. Estes achados aproximam-se dos encontrados na literatura, corroborando para a compreeensão do comportamento biológico deste tumor e apontando a extensão do tumor tecidual como único fator prognóstico para recidiva desta neoplasia.

Tumores sellares y suprasellares que afectan al quiasma y nervios ópticos. I. Consideraciones generales / Sellar and suprasellar tumors affecting the chiasma and optic nerves. General considerations

Las neoplasias sellares y suprasellares representan únicamente el 7.5 por ciento de los tumores intracraneanos. Las variedades más importantes son los craneofaringiomas y los adenomas. Estas neoplasias se manifiestan clínicamente por el daño a las vías visuales anteriores. Los adenomas son neoplasias funcionales específicas mientras que los craneofanrigiomas cursan con hipopituitarismo. La resonancia nuclear magnética y la topografía axial computada substituyen con muchas ventajas a los procedimiento radiológicos anteriores. El tratamiento puede ser médico, quirúrgico, con radiaciones ionizantes y recientemente con una variante que es la radiocirugía o el bisturí de rayos gamma. Hisotológicamente tienen un patrón característico cada una de sus variedades y actualmente se puede demostrar su estirpe con toda precisión. El oftalmólogo juega el papel más importante en la detección de estas neoplasias

Análise retrospectiva da incidência de neoplasias supratentoriais da linha mediana em crianças e jovens: craniofaringeomas, neoplasias da hipótese e pineal / Retrospective analysis of the incidence of supratentorial neoplasms in the median line in children and young patients: craniopharyngiomas, hypophyseal and pineal neoplasms

Analisamos, retrospectivamente, 1.632 casos de neoplasias intracranianas, em um período de 50 anos (1931-1981), do Departamento de Patologia da Faculdade de Medicina da USP, 592 casos (36,2%) eram de indivíduos de 0 a 20 anos, grupo escolhido para este estudo. O total de craniofaringeomas nessa faixa etária foi de 21 casos (3,5%); de neoplasias de hipófise, oito (1,35%); de neoplasias da pineal, quatro (0,6%). Infiltraçöes em hipófise e pineal corresponderam a 24 casos (4,0%); näo houve nenhum caso de metástases. Descriçäo e importância destes achados e comparaçäo com os de outros autores é relatada