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)

Endoscopic transsphenoidal endonasal surgery in pituitary adenomas / Cirugía endoscópica vía endonasal transesfenoidal en adenomas de hipófisis

The pituitary adenomas comprise the largest part of pituitary neoplasms, representing between 9 and 12% of primary brain tumors. Currently, the treatment of election is the surgical, the trans-sphenoidal endonasal path has provided a broader panoramic view, being feasible more delicate and safe procedures. The objective of this study was to describe the management of pituitary adenomas by endoscopic surgery trans-sphenoidal endonasal path. This is a study in a series of cases, with a sample of 17 patients who met the selection criteria and were attended at the José Carrasco Hospital Arteaga, Cuenca - Ecuador, period 2018 - 2019. All patients presented informed consent for the performance of the surgery and the subsequent dissemination of the results. The 53% of the population belonged to the male gender, 47% were patients between 40 and 60 years old, 65% were detected with macroadenomas, and 29% presented complications such as nasal obstruction, sphenoiditis and headache, the residual tumor stage was due 24%.Endoscopic surgery trans-sphenoidal endonasal path is a safe, minimally invasive tool, a surgical alternative with a high rate of tumor excision, better control and fewer complications, which reduces the morbidity of patients.

Los adenomas de hipófisis comprenden la mayor parte de neoplasias hipofisiarias, representando entre el 9 y 12% de los tumores cerebrales primarios. En la actualidad el tratamiento de elección es el quirúrgico, la vía endonasal transesfenoidal ha proporcionado una visión panorámica más amplia, siendo factibles procedimientos más delicados y seguros. El objetivo de este estudio fue describir el abordaje de adenomas de hipófisis mediante cirugía endoscópica vía endonasal transesfenoidal. Diseñamos un estudio de corte longitudinal, con una muestra de 17 pacientes que cumplieron con los criterios de selección y fueron atendidos en el Hospital José Carrasco Arteaga de la ciudad de Cuenca - Ecuador, periodo 2018 - 2019. Todos los pacientes presentaron consentimiento informado para la realización de la cirugía y la posterior difusión de los resultados. El 53% de la población pertenecieron al sexo masculino, 47% fueron pacientes entre 40 y 60 años, al 65% se le detectó macroadenomas, el 29% presentaron complicaciones como obstrucción nasal, esfenoiditis y cefalea, la tasa de tumor residual fue del 24%. La cirugía endoscópica vía endonasal transesfenoidal es una herramienta segura, mínimamente invasiva, una alternativa quirúrgica con una elevada tasa de exéresis tumoral, mejor control y menos complicaciones, que reduce la morbilidad de los pacientes.

Nasal Mucoepidermoid Carcinoma after Radiotherapy: Case Report

Introduction Mucoepidermoid carcinoma (MEC) is a tumor originated from the epitheliumof the glandular excretory ducts and has highly variable biological potential. It is the most prevalent cancer of the salivary glands. The present report aims to describe a case of nasal mucoepidermoid carcinoma that developed after adjuvant radiotherapy (RT) treatment of a recurrent pituitary macroadenoma. Case Report Male patient, 62 years old, presented with recurrent nasal epistaxis on the right, associated with intense pulsatile headache, visual analogical scale (VAS) 10/10, with improvement only with the use of opioids andmorphine. After undergoing oncological screening and study by imaging exams, the presence of an expansive seal lesion with suprasellar extension was seen, involving the medial wall of the cavernous segment of the right carotid artery and the anterior cerebral artery, as well as the presence of a new expansive lesion in the right nasal cavity, with ethmoid bone invasion superiorly and medial orbit wall invasion laterally, compressing the ipsilateral optic nerve canal. Discussion Sinonasal neoplasms represent a small portion of all malignancies of the upper aerodigestive tract, accounting for<5% of these neoplasms. The development of MEC involves risk factors such as occupational issues, history of trauma and surgery involving the nasal area, and radiation exposure, as in previous RT. Conclusion Mucoepidermoid carcinoma is an uncommon neoplasia and can be associated with RT treatment, as used in cases of recurrent pituitary macroadenoma. In general, surgical resection to obtain free margins of neoplastic tissue is the aimed treatment, seeking better prognosis.

Tumor de hipófise em paciente com hidrocefalia: um desafio diagnóstico / Pituitary tumor in a patient with hydrocephalus: a diagnostic challenge

RESUMO A hidrocefalia é definida como a dilatação ventricular pelo aumento da pressão intraventricular e intracraniana quando não tratada ou por insucesso do tratamento. Muitas vezes, leva ao dano das vias ópticas, podendo causar atrofia óptica, devido à proximidade dessas vias com o ventrículo lateral quando ocorre a dilatação. Assim como a hidrocefalia pode levar à atrofia óptica, outras patologias também podem. Tumores hipofisários compartilham desse mesmo sinal, além de causar hemianospsia bitemporal quando o tumor comprime quiasma óptico. Ademais, a hemianopsia bitemporal é o distúrbio visual mais comum encontrado em pacientes com tumor de hipófise. Os tumores de hipófise, por exemplo, geram manifestações clínicas que podem estar relacionadas à disfunção da glândula ou aos efeitos mecânicos da expansão tumoral. Sinais e sintomas visuais estão mais ligados ao efeito mecânico do tumor. Assim, muitas vezes, o paciente procura o oftalmologista antes do endocrinologista. Neste caso, analisaremos uma paciente portadora de hidrocefalia que apresentava, concomitantemente, um tumor hipofisário, e a investigação oftalmológica fez toda a diferença no tratamento da paciente.

ABSTRACT Hydrocephalus is defined as ventricular dilation caused by increased intraventricular and intracranial pressure when untreated or due to treatment failure. Optical pathways can often cause optic atrophy due to the proximity to the lateral hazard when dilation occurs. Hydrocephalus can lead to optic atrophy, as well as other pathologies. Pituitary tumors share this same sign, in addition to causing bitemporal hemianopia when it compresses the optic chiasm. In addition, bitemporal hemianopia is the visual disturbance most commonly found in patients with pituitary tumors. Pituitary tumors, for example, have clinical manifestations that may be related to gland dysfunction, or to mechanisms of tumor expansion. Visual signs and symptoms are more linked to the mechanical effect of the tumor. Therefore, the patient usually seeks the ophthalmologist before the endocrinologist. In this case, we analyzed a patient with hydrocephalus who presented, at the same time, a pituitary tumor, and the ophthalmological investigation made all the difference in the treatment of the patient.

Apoplexy in sporadic pituitary adenomas: a single referral center experience and AIP mutation analysis

ABSTRACT Objective: To analyze the clinical, laboratory, and radiological findings and management of patients with clinical pituitary apoplexy and to screen for aryl hydrocarbon receptor-interacting protein (AIP) mutations. Subjects and methods: The clinical findings were collected from the medical records of consecutive sporadic pituitary adenoma patients with clinical apoplexy. Possible precipitating factors, laboratory data, magnetic resonance imaging (MRI) findings and treatment were also analyzed. Peripheral blood samples were obtained for DNA extraction from leukocytes, and the entire AIP coding region was sequenced. Results: Thirty-five patients with pituitary adenoma were included, and 23 (67%) had non-functioning pituitary adenomas. Headache was observed in 31 (89%) patients. No clear precipitating factor was identified. Hypopituitarism was observed in 14 (40%) patients. MRI from 20 patients was analyzed, and 10 (50%) maintained a hyperintense signal in MRI performed more than three weeks after pituitary apoplexy (PA). Surgery was performed in ten (28%) patients, and 25 (72%) were treated conservatively with good outcomes. No AIP mutation was found in this cohort. Conclusion: Patients with stable neuroophthalmological impairments can be treated conservatively if no significant visual loss is present. Our radiological findings suggest that hematoma absorption lasts more than that observed in other parts of the brain. Additionally, our study suggests no benefits of AIP mutation screening in sporadic patients with apoplexy.

Huge Sellar Plasmacytoma as Differential Diagnosis of Invasive Pituitary Adenoma: A Case Report

Sellar plasmacytomas are rare tumors arising from plasma cells. They are often misdiagnosed as adenomas.We report the case of a 63-year-old woman with headache, cranial nerve III palsy and decreased visual acuity. Imaging revealed an extensive lesion centered on the clivus, extending to the cavernous sinus bilaterally and into the sphenoid sinus. The hormonal tests were compatible with panhypopituitarism and mild hyperprolactinemia. The first hypothesis was invasive pituitary adenoma. Partial resection was achieved, and the immunohistochemical evaluation was compatible with plasmacytoma. After a few weeks, she developed lumbar and hip pain, and the imaging confirming osteolytic lesions. The final diagnosis was multiple myeloma.

Endonasal Endoscopic Pituitary Adenoma Resection in light of the COVID-19 Pandemic: A Technical Report

Introduction The endoscopic endonasal transsphenoidal approach (EETA) is routinely used to treat sellar and suprasellar tumors. It provides safe and direct access to tumors in these locations, with wide visualization of anatomical landmarks and great surgical results. With the COVID-19 pandemic, despite the high risk of transmission involved, various surgical procedures cannot be postponed due to their emergency. Case Report A 62-year-old female presented in the previous two months with headaches, followed by bilateral severe visual loss. In 2016, she was submitted to subtotal resection of a non-secretorymacroadenoma. Because of the progressive visual deficits, the EETA was used to the resect the pituitary adenoma. Technical Note We developed a low-cost adaptation to the surgical fields, covering the patient's head and superior trunk with a regular surgicalmicroscope bag with a tiny slit to enable the endoscope and surgical instruments to enter the nose, thus protecting the personnel in the operating room from the aerosolization of particles. This makes surgery safer for the surgical team and for the patient. Conclusion In view of the lack of literature on this subject, except for some reports of experiences from some services around the world, we describe the way we have adjusted the EETA in the context of the COVID-19 pandemic.

Co-Occurrence of Pineal-Region and Pituitary-Stalk Hemangioblastomas in a Patient Presenting with Von Hippel-Lindau Disease ­ A Case Report

Introduction Hemangioblastomas of the pineal region or pituitary stalk are extremely rare. Only two cases of hemangioblastomas involving the pineal region have been reported, and four involving the pituitary stalk. The purpose of the present manuscript is to describe an unusual case of supposed hemangioblastoma found concomitantly in the pineal region and pituitary stalk of a patient diagnosed with Von Hippel-Lindau (VHL) disease. Case Report A 35-year-old female patient with a previous diagnosis of VHL complaining of occipital headaches and balance disturbances for three weeks, who previously had a cerebellar hemangioblastoma resected. The visual characteristics of the tumor suggested a friable vascular lesion with a reddish-brown surface, and an incisional biopsy was performed. The tumor consisted of a dense vascular network surrounded by fibrous stroma abundant in reticulin and composed by both fusiform and dispersed xanthomatous cells; the immunohistochemistry was immunopositive for neuronspecific enolase and immunonegative for epithelial membranous antigen. The patient has been monitored closely for 2 years, and the supratentorial masses have not presented any volume alteration. Conclusion This rare association must be taken into account in patients with VHL disease, or at least be suspected in patients who present a thickening of the pituitary stalk and a pineal-region mass. We believe a biopsy of our asymptomatic patient could have been dangerous due to inherent complications like intraoperative bleeding. We recommend close observation of asymptomatic lesions with MRIs every six months or until the lesions become symptomatic. If the pineal-region tumor does become symptomatic, gross resection via a transcallosal approach would be ideal.

Manejo preoperatorio de los adenomas hipofisarios. Lo que un residente de neurocirugía debe conocer / Preoperative management of pituitary adenomas. What a neurosurgery resident should know

Las lesiones selares son una patología con una incidencia de 3,2 a 4 / 100,000 y una prevalencia de 78 a 94 / 100,000. Un 10% son incidentalomas en la población adulta. Se cree que su prevalencia en el orden mundial actualmente va en aumento.En relación a las manifestaciones clínicas, cabe destacar que es una de las pocas enfermedades que pueden manifestarse tanto por signos y síntomas neurológicos (por ejemplo: hemianopsia bitemporal, síndrome de hipertensión endocraneana debido a hidrocefalia, entre otros), como también por síndromes endocrinológicos (por ejemplo: síndrome de Cushing, acromegalia, amenorrea-galactorrea, infertilidad).Todo paciente debe presentar un estudio clínico-radiológico completo, lo que permitirá un correcto diagnóstico y categorización del mismo.El objetivo del presente trabajo es proporcionar al neurocirujano en formación los conceptos claves que servirán de sustento para el manejo preoperatorio de un paciente con adenoma hipofisario.

Sellar lesions are a pathology with an incidence of 3.2 to 4 / 100.000 and a prevalence of 78 to 94 / 100.000. Normally, 10% of them are incidentalomas and adult patients are in the highest risk group. Because it ́s prevalence in the world is currently increasing, it is of extremely importance to study and understand this pathology. In relation to the clinical manifestations, it should be noted that it is one of the few diseases that can manifest through neurological signs and symptoms like bitemporal hemianopsia, endocranial hypertension syndrome due to hydrocephalus, as well as endocrinological syndromes like Cushing's, acromegaly, amenorrhea-galactorrhea and infertility. One of the most important things to notice is that the treatment success in this pathology comes with the correct diagnosis and characterization of it, for what all patients should have a complete clinical-radiological evaluation.In this study, we establish a guide with concepts and key tools to support the medical personal during a pre-surgical preparation of patients with pituitary adenoma.

Proteína moduladora de la actividad del receptor de aril hidrocarburos (AIP): genética, bioquímica e impacto clínico / Aryl hydrocarbon receptor interacting protein (AIP): genetics, biochemistry and clinical impact

El gen AIP (proteína moduladora de la actividad del receptor de aril hidrocarburos) se localiza en la región 11q13.2 y codifica para una proteína de 330 aminoácidos que interactúa con el factor de transcripción AhR (receptor para aril hidrocarburos). Las mutaciones en este gen se han asociado con adenomas pituitarios aislados de tipo familiar (APAF). Se caracterizan por una presentación temprana (alrededor de 20 años), por lo regular producen hormona de crecimiento y/o prolactina, tienen un comportamiento clínico agresivo y poca respuesta a análogos de somatostatina.

The AIP gene (aryl hydrocarbon receptor interacting protein) is located on chromosome 11q13.2 and encodes a 330 amino acid protein which interacts with the aryl hydrocarbon receptor (AHR) transcription factor. Mutations in the AIP gene have been associated with familial isolated pituitary adenomas (FIPA). They characterize by an early-onset (around the age of 20 years old) and for being aggressive, growth hormone and/or prolactin-secreting tumors, with poor response to somatostatin analogues.

Growth hormone-releasing hormone-secreting pulmonary neuroendocrine tumor associated with pituitary hyperplasia and somatotropinoma

SUMMARY Acromegaly caused by ectopic growth hormone-releasing hormone (GHRH)-secreting tumor is exceedingly rare. We report a case of acromegaly secondary to GHRH secretion by an incidentally diagnosed pulmonary neuroendocrine tumor (NET) and review 47 similar cases in literature. A 22-year-old male patient presented with symptoms of pituitary apoplexy. Magnetic resonance imaging (MRI) showed apoplexy of a pituitary adenoma. Routinely prior to surgery, a chest radiography was performed which revealed a mass in the left lung. During investigation, the patient was diagnosed with metastatic GHRH-secreting pulmonary NET. In retrospect, it was noted that the patient had pituitary hyperplasia 20 months prior to the MRI which showed the presence of a pituitary adenoma. The histological findings confirmed somatotroph hyperplasia adjacent to somatotropinoma. This case suggests that GHRH secretion can be associated with pituitary hyperplasia, which may be followed by pituitary adenoma formation.

Primary pituitary tuberculosis

Tuberculosis is an infectious disease that involves any organ. However, the primary pituitary tuberculosis is an extremely rare disease. Intracranial tuberculomas account for 0.15-5% of intracranial space-occupying lesions, of which, pituitary as the primary site is unusual, and easily misdiagnosed as pituitary adenoma. In this setting, the late diagnosis can result in permanent endocrine dysfunction. We report the case of a 50-year-old woman who presented to the neurosurgery outpatient department with complaints of progressively increasing headache and diminished vision over the last year. On the clinical examination, the patient was conscious and oriented. The routine hematological and biochemical workup showed an increased erythrocyte sedimentation rate (ESR) and increased prolactin levels. The radiological working diagnosis was consistent with pituitary macroadenoma. No other radiological and/or clinical clue that could elicit the suspicion of pulmonary or extrapulmonary lesions of tuberculosis was found. The transsphenoidal endonasal tumor excision was done. The histopathology showed numerous epithelioid cell granulomas, Langhans giant cells along with scant necrosis. Ziehl Neelsen staining demonstrated acid-fast bacilli, and the final diagnosis of pituitary tuberculoma was made. We report this rare case of pituitary lesion that may be included in the differential diagnosis of sellar lesions to avoid unnecessary surgical interventions, especially in regions where the disease is endemic.

As consequências irreversíveis do diagnóstico tardio de macroadenoma de hipófise em paciente jovem / The irreversible consequences of late diagnosis of pituitary macroadenoma in a young patient

Resumo Os tumores de hipófise representam aproximadamente 15% de todos os tumores cerebrais e dependendo do tamanho, pressionam o quiasma óptico, resultando em comprometimento da função visual que se manifesta como defeitos no campo visual, diminuição da acuidade visual e da visão das cores. O objetivo do presente estudo foi relatar um caso de macroadenoma de hipófise com compressão do quiasma óptico e defeito no campo visual, tratado inicialmente como glaucoma, levando a um diagnóstico e tratamento tardio.

Abstract Pituitary tumors represent approximately 15% of all brain tumors and depending on size, pressure optic chiasma, resulting in impaired visual func-tion that manifests itself as defective in the visual field, decreased acuity visual and color vision. The ob-jetive of the present study was to report a case of pitui-tary macroadenoma with compression of optical chiasma and visual field de-fect, initially treated as glaucoma, leading to a late diagnosis and treatment.

Non-functioning pituitary adenomas and pregnancy: one-center experience and review of the literature

SUMMARY The usual clinical presentation of non-functioning pituitary adenoma (NFPA) consists of symptoms of mass effect and hypopituitarism. NFPA is a rare condition in young women and an uncommon complication during pregnancy. We present the outcome of three patients with NFPA during pregnancy. Case 1: a 38-year-old woman was referred at 32nd week of spontaneous pregnancy because of diagnosis of a pituitary macroadenoma discovered in the context of progressive visual loss. Hormonal deficiency and hypersecretion were ruled out. Prolactin levels were high as expected. She developed diplopia and severe headache despite the use of dopamine agonists and corticosteroids, so pregnancy was interrupted at 34th week. After an uncomplicated delivery of a healthy newborn, transsphenoidal surgery was performed. The pathology was consistent with a gonadotroph adenoma. She recovered visual field, and remained with normal pituitary function. Postsurgical tumor remnant increased in size during the follow-up. Case 2: a 34-year-old woman was referred due to secondary amenorrhea and galactorrhea. A macroadenoma with suprasellar extension was discovered. Transsphenoidal surgery confirmed a gonadotroph adenoma. Two years after surgery she had a normal pregnancy. Six years after surgery a small tumor recurrence occurred. Case 3: a 23-year-old woman was referred due to a microincidental pituitary adenoma. Laboratory testing was normal. No findings on physical examination. A wait and see approach was decided. Two years after diagnosis, the patient got pregnant without complications. Image remained stable. This article may contribute new cases and provides an extensive review of NFPA during pregnancy.

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.

Estudios clínicos y genéticos en tres miembros de una familia afectados con adenomas hipofisarios aislados con prolactinomas homogéneos / Clinical and genetic studies of a three-member familial isolated pituitary adenoma with homogeneous prolactinomas

La mayoría de los adenomas hipofisarios son esporádicos, pero un 3-5% puede ocurrir en un contexto familiar y hereditario. Este es el caso de la neoplasia endocrina múltiple de tipo 1 (NEM1), complejo de Carney (CNC) y adenomas hipofisarios aislados familiares (FIPA). El FIPA es una condición infrecuente, que ocurre en un contexto familiar, no asociada a NEM t ipo1 ni CNC. Los FIPA pueden ser homogéneos (todos los adenomas tienen el mismo fenotipo) o heterogéneos (diferente fenotipo tumoral). Describimos una familia congolesa en la que dos hermanas y una prima fueron diagnosticadas a los 29, 32 y 40 años, respectivamente, con un prolactinoma (FIPA homogéneo). Las pacientes presentaron macroadenomas no invasivos al momento del diagnóstico, con buena respuesta biológica y tumoral al tratamiento con cabergolina hasta una dosis máxima de 1.5 mg/semanal. De las dos hermanas, una cursó un embarazo sin complicaciones. Durante el seguimiento de 12 años, ninguna de ellas presentó elementos clínicos o biológicos compatibles con NEM1 o CNC, por lo que dichos genes no se estudiaron. El análisis genético en dos de las pacientes permitió descartar la posibilidad de una mutación germinal del gen aryl hydrocarbon receptor interacting protein (AIP). Se considera que el 80% de los pacientes con FIPA no presentan mutación del gen AIP, por lo que se requieren futuros estudios en este tipo de familias, para poder determinar otros genes afectados involucrados en su fisiopatología.

Most pituitary adenomas are sporadic, but 3-5% can occur in a family and hereditary context. This is the case of multiple endocrine neoplasia type 1 (MEN1), Carney complex (CNC) and familial isolated pituitary adenomas (FIPA). FIPA is an infrequent condition that occurs in a family context, not associated with MEN type1 or CNC. FIPA kindred can be homogeneous (all adenomas affected in the family having the same tumor phenotype) or heterogeneous (different tumor phenotypes in the affected members). We describe a Congolese family in which two sisters and a cousin were diagnosed with a prolactinoma (homogenous FIPA) at the ages of 29, 32 and 40 years, respectively. The patients presented with macroadenomas at the time of diagnosis, non-invasive tumors and good biological response to cabergoline treatment (maximum dose of 1.5 mg/weekly). Of these two sisters, one went through a pregnancy without complications. Because no MEN1 and CNC clinical and biochemical features were detected during the 12-year follow-up, these genes were not investigated. The genetic analysis of the aryl hydrocarbon receptor interacting protein (AIP) was normal. As nearly 80% of patients with FIPA do not have a mutation in the AIP gene, future studies in these families are required to identify other affected genes involved in their physiopathology.

Pituitary tumor resection in a patient with SARS-CoV-2 (COVID-19) infection. A case report and suggested airway management guidelines / Assistência perioperatória de paciente com infecção pelo SARS-CoV-2 (COVID-19) submetido a ressecção de tumor de hipófise urgente. Relato de caso e diretrizes para manejo de via aérea

Abstract The 2020 pandemic caused by the novel coronavirus, COVID-19, had its headquarters in China. It causes Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) and presents a broad spectrum of clinical manifestations, ranging from entirely asymptomatic through severe acute respiratory failure and death. Presuming a significant quantity of ventilator-dependent patients, several institutions strategically delayed elective surgeries. Particularly procedures performed involving the nasal mucosa, such as a transsphenoidal approach of the pituitary gland, considering the tremendous level of viral shedding. Nevertheless, critical cases demand expeditious resolution. Those situations are severe pituitary apoplexy, declining consciousness level, or risk of acute visual loss. This case presents a successful urgent perioperative management of a 47 year-old male COVID-19 positive patient who presented to the Emergency Department with a left frontal headache that culminated with diplopia, left eye ptosis, and left visual acuity loss after 5 days. Transsphenoidal hypophysectomy was uneventfully performed, and the patient was discharged from the hospital on postoperative day four. It additionally describes in detail the University of Mississippi Medical Center airway management algorithm for patients infected with the novel coronavirus who need emergent surgical attention.

Resumo A pandemia de 2020 causada pelo novo coronavírus, COVID-19, teve seu epicentro na China. Causa Síndrome Respiratória Aguda Grave pelo Coronavírus 2 (SARS-CoV-2) e apresenta um amplo espectro de manifestações clínicas, que vão desde nenhum sintoma a insuficiência respiratória aguda grave e óbito. Com a expectativa de um número significativo de pacientes dependentes de ventilador, várias instituições estrategicamente adiaram cirurgias eletivas. Esse é o caso principalmente de procedimentos envolvendo a mucosa nasal, como a via transesfenoidal para a hipófise, devido ao nível imenso de disseminação de material viral. Não obstante, casos críticos requerem resolução acelerada. Essas situações são grave apoplexia hipofisária, diminuição do nível de consciência ou risco de perda visual aguda. O presente caso relata o manejo perioperatório bem sucedido de urgência de paciente do sexo masculino de 47 anos de idade com COVID-19 que chegou ao Pronto Socorro com cefaleia frontal à esquerda que culminou com diplopia, ptose do olho esquerdo e perda de acuidade visual à esquerda após 5 dias. A hipofisectomia transesfenoidal ocorreu sem intercorrências e o paciente recebeu alta do hospital no quarto dia do pós-operatório. Adicionalmente, descrevemos em detalhe o algoritmo de manejo de via aérea da University of Mississippi Medical Center para pacientes infectados pelo novo coronavírus e que necessitam de atenção cirúrgica de emergência.

Tratamiento quirúrgico de los adenomas hipofisarios en el Noroeste Argentino / Surgical treatment of pituitary adenomas in the Argentine Northwest

Introducción: Los tumores de hipófisis representan aproximadamente un 10% de los tumores intracraneales. Los adenomas hipofisarios son ampliamente la lesión selar más frecuente. Es fundamental que se conformen equipos capaces de tratar holísticamente esta patología en las distintas regiones de nuestro vasto territorio. El propósito del presente trabajo es presentar nuestra experiencia en el manejo quirúrgico de los adenomas hipofisarios en el Noroeste Argentino. Materiales y métodos: Estudio retrospectivo de pacientes operados por adenomas selares, desde enero de 2013 hasta abril de 2019, en la provincia de Tucumán, por el autor Senior. Resultados: Se analizaron 211 pacientes operados de adenomas pituitarios. En el 75% (n=158) las resecciones fueron totales y en el 25% (n=53) fueron resecciones subtotales. De los 88 pacientes con macroadenomas no funcionantes y déficit visual, el 94% (n=83) presentó mejoría visual en el postoperatorio. De los 52 pacientes con acromegalia, un 75% (n=39) presentó remisión bioquímica completa postquirúrgica. De los 26 pacientes con enfermedad de Cushing, un 81% (n=21) presentó remisión bioquímica completa postquirúrgica. De los 21 pacientes con prolactinomas, un 71% (n=15) normalizaron los niveles de prolactina en el postoperatorio. Conclusión: Los resultados en el tratamiento quirúrgico de los adenomas hipofisarios de la presente serie son comparables a los reportados por centros especializados internacionales. Debemos formar equipos capaces de tratar esta patología en las distintas regiones de la Argentina.

Background: Pituitary tumors account for 10% of intracranial tumors. Pituitary adenomas are the most common selar lesion. It is essential that teams be formed capable of treating this pathology holistically in the different regions of our vast territory. The purpose of this article is to present our experience in the surgical management of pituitary adenomas in the Northwest of Argentine. Materials and methods: A retrospective analysis was done studding patients operated from pituitary adenomas, from January 2013 to April 2019, in Tucumán, by the Senior Author. Results: We analyzed 211 patients operated from pituitary adenomas, of which 75% (n=158) had gross total resections and 25% (n=53) had subtotal resections. Of the 88 patients with non-functioning macroadenomas and visual deficit, 94% (n=83) presented visual improvement after surgery. Of the 52 patients with acromegaly, 75% (n=39) presented complete biochemical remission after surgery. Of the 26 patients with Cushing's disease, 81% (n=21) presented complete biochemical remission after surgery. Of the 21 patients with prolactinomas, 71% (n=15) normalized prolactin levels after surgery. Conclusion: Our results in the surgical treatment of pituitary adenomas are comparable to those reported by international specialized centers. We must form teams capable of treating this pathology in the different regions of Argentina

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.