RESUMO
Pheochromocytomas and paragangliomas (PPGL) are neuroendocrine tumors characterized by the excessive production of catecholamines. This study aims to describe the clinical characteristics of PPGL cases in Argentina over recent decades. A multicenter retrospective cross-sectional analysis was carried out using a database comprising both pediatric and adult patients with confirmed PPGL diagnoses based on pathological reports. A cohort of 486 patients with PPGL was recruited. Women represent 58.4% of the patients, with a mean age of 38.3 years old at the time of diagnosis and 15.2% of the patients were under the age of 18. Hypertension, as well as classic signs and symptoms, were present in 80.9% of the patients. The adrenal incidentaloma, as a mode of presentation, increased in the last two decades rising from 3.9% (1953-2000) to 21.8% (2001-2022), p<0.001. Most tumors were located within the adrenal glands, accounting 83.0% of the cases, with bilateral occurrences noted in 20.0%. The median tumor size was 4.8cm. Local recurrence and metastases were observed in 10.9% and 12.2%. Out of 412 patients, 87.0% exhibited urinary excretion elevation of catecholamines and/or their metabolites. Furthermore, 148 patients, representing 30.4% of the study population, displayed a distinct genetic profile indicative of hereditary syndromes. The distribution of hereditary syndromes revealed that MEN2, VHL, and PGL4 constituted the most prevalent syndromes. This population-based study, spanning seven decades, offers valuable insights into the demographic and clinical characteristics of PPGL patients in Argentina.
Assuntos
Neoplasias das Glândulas Suprarrenais , Bases de Dados Factuais , Paraganglioma , Feocromocitoma , Humanos , Feocromocitoma/patologia , Feocromocitoma/epidemiologia , Argentina , Neoplasias das Glândulas Suprarrenais/epidemiologia , Neoplasias das Glândulas Suprarrenais/patologia , Feminino , Masculino , Estudos Retrospectivos , Adulto , Paraganglioma/patologia , Estudos Transversais , Pessoa de Meia-Idade , Adolescente , Adulto Jovem , Criança , Idoso , Recidiva Local de Neoplasia/epidemiologia , Pré-Escolar , Hipertensão/epidemiologiaRESUMO
Background: Pituitary incidentalomas are an occurrence documented in 10.6% of post-mortem examinations, 4%-20% of computed tomography (CT) scans, and 10%-38% of magnetic resonance imaging (MRI) cases, primarily consisting of microincidentalomas (<1 cm in size). However, the prevalence of pituitary incidentalomas in Uruguay remains unexplored. This study aimed to ascertain the prevalence of pituitary incidentalomas at our hospital. Methods: In this investigation, we retrospectively identified patients who underwent brain CT and MRI at our hospital over a 1-year span due to conditions other than suspected or known pituitary disorders. The time frame covered was from 1 January to 31 December 2017. Our analysis encompassed all scans, and we conducted interviews with patients discovered to have pituitary incidentalomas. Furthermore, we conducted biochemical assessments in accordance with clinical and imaging traits. Results: During the study period, a total of 3,894 patients underwent imaging procedures. Of these, 1,146 patients underwent MRI scans, and 2,748 underwent CT scans. The mean age was 53.1 ± 19 years, with a relatively even distribution between genders (50.6% women). The majority of imaging requisitions originated from the emergency department (43%), followed by outpatient clinics (29%), and inpatient wards (28%). Common reasons for imaging requests included trauma (20.4%), headaches (11.3%), and stroke (10.9%). Among these cases, two pituitary incidentalomas were detected, resulting in a prevalence of 5 cases per 10,000 individuals annually (0.051%). Both of these cases were initially identified through CT scans, with subsequent MRI scans performed for further assessment. The final diagnoses were a vascular aneurysm and a sellar meningioma, with the latter patient also exhibiting secondary hypothyroidism. Notably, no instances of pituitary adenomas were encountered. Conclusions: The prevalence of pituitary incidentalomas within our hospital was notably low. Further research is necessary to more comprehensively investigate the occurrence of pituitary incidentalomas in our country.
Assuntos
Neoplasias Hipofisárias , Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Prevalência , Estudos Retrospectivos , Uruguai , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/complicações , Hospitais de EnsinoRESUMO
Background: Malakoplakia is a rare disorder 75% of the reported cases affect mainly the genitourinary tract, its occurrence in the adrenal gland is extremely rare. Case presentation: A 65-year-old female patient presented to the emergency department for chronic abdominal pain. Radiographic and biochemical studies revealed a left adrenal incidentaloma and left adrenalectomy was performed. Histological examination showed the presence of Michaelis-Gutmann bodies, compatible with a malakoplakia of the adrenal gland. Conclusions: Malakoplakia is a rare disorder, with non-standardized treatment, medical and surgical therapies appear to be effective in treating the condition.
RESUMO
Resumen Durante la realización de informes de resonancia magnética (RM) de columna, pueden encontrarse hallazgos extrarraquídeos, normales o patológicos, que no deben ser omitidos por el médico imagenólogo. Estas imágenes casuales, conocidas como incidentalomas, pueden ser la causa que originó la realización de la RM o bien ser totalmente asintomáticas. Se describen hallazgos extrarraquídeos habituales en las RM de columna.
Abstract When reading spinal MR examinations normal or abnormal extra spinal changes can be found but they should be described in the report.These findings, known as incidentalomas, can be related to the patients symptoms or can be asymptomatic. We describe frequent extraspinal findings found on MR of the spine.
RESUMO
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)
Assuntos
Humanos , Masculino , Feminino , Adulto , Adulto Jovem , Neoplasias Hipofisárias/epidemiologia , Acromegalia/epidemiologia , Prolactinoma/epidemiologia , Adenoma/epidemiologia , Achados Incidentais , Hipersecreção Hipofisária de ACTH/epidemiologia , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/diagnóstico por imagem , Adenoma/patologia , Adenoma/diagnóstico por imagem , Relevância ClínicaRESUMO
Objective: We investigated the prevalence of adrenal incidentalomas (AIs) in a nonselected Brazilian population in chest computed tomography (CT) performed during the COVID-19 pandemic. Materials and methods: This was a retrospective cross-sectional observational study using chest CT reports from a tertiary in- and outpatient radiology clinic from March to September 2020. AIs were defined by changes in the shape, size, or density of the gland initially identified in the released report. Individuals with multiple studies were included, and duplicates were removed. Exams with positive findings were reviewed by a single radiologist. Results: A total of 10,329 chest CTs were reviewed, and after duplicate removal, 8,207 exams were included. The median age was 45 years [IQR 35-59 years], and 4,667 (56.8%) were female. Thirty-eight lesions were identified in 36 patients (prevalence 0.44%). A higher prevalence was observed with age, with 94.4% of the findings in patients aged 40 years and over (RR 9.98 IC 2.39-41.58, p 0.002), but there was no significant difference between the sexes. Seventeen lesions (44.7%) had more than 10 HU, and five lesions (12.1%) were more than 4 cm. Conclusion: The prevalence of AIs in an unselected and unreviewed population in a Brazilian clinic is low. The impact on the health system caused by AIs discovered during the pandemic should be small regarding the need for specialized follow-up.
Assuntos
Neoplasias das Glândulas Suprarrenais , COVID-19 , Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Masculino , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/epidemiologia , COVID-19/diagnóstico por imagem , COVID-19/epidemiologia , Brasil/epidemiologia , Pandemias , Prevalência , Estudos Retrospectivos , Estudos Transversais , Tomografia Computadorizada por Raios X/métodos , Achados IncidentaisRESUMO
OBJECTIVE: Although there is growing evidence associating nonfunctioning adrenal incidentalomas (NFAI) with cardiovascular risk factors, there are limited data whether NFAI and autonomous cortisol secretion (ACS) groups have similar or different clinical and metabolic features. The aim of this study is to compare cardiometabolic clinic parameters among patients with ACS and NFAI, as well as controls. DESIGN: Cross-sectional study. PATIENTS: Eighty nine NFAI, 58 ACS and 64 controls were evaluated. MEASUREMENTS: Diagnosis of NFAI (1 mg dexamethasone suppression test [1 mg-DST] ≤50 nmol/L [≤1.8 µg/dl]) and ACS (1 mg-DST > 50 nmol/L [> 1.8 µg/dl]) was established according to current guidelines. The control group was selected based on a normal adrenal imaging exam. RESULTS: There were no differences between groups regarding age, gender, ethnicity, menopause or body mass index. Patients with adrenal incidentaloma presented higher frequency of hypertension (74.1 vs. 57.8%; p = .02), resistant hypertension (45.4 vs. 9.4%; p < .001), dyslipidemia (80.1 vs. 63.9%; p = .01), as well as metabolic syndrome (84.2 vs. 61.7%; p = .001) compared to the controls, respectively. NFAI and ACS patients presented similar frequency of arterial hypertension (70.8 vs. 79.3%) and resistant hypertension (41.3 vs. 51.1%), dyslipidemia (79.3 vs. 81.5%) and metabolic syndrome (83.3 vs. 85.7%); also, levels of HbA1c were similar between the groups. Binary logistic regression showed that NFAI (p = .004) and ACS (p = .001) were independent predictors for resistant hypertension (p = .003); also, ACS was an independent predictor for metabolic syndrome (p = .04). CONCLUSIONS: NFAI and ACS presented a higher frequency of cardiometabolic morbidities in comparison with individuals with normal adrenal glands. Additionally, we demonstrated that both ACS and NFAI groups have similar cardiometabolic conditions.
Assuntos
Neoplasias das Glândulas Suprarrenais , Hipertensão , Síndrome Metabólica , Feminino , Humanos , Síndrome Metabólica/etiologia , Neoplasias das Glândulas Suprarrenais/complicações , Hidrocortisona/metabolismo , Estudos Transversais , Hipertensão/complicaçõesRESUMO
Objetivo: Brindar recomendaciones actualizadas a urólogos y profesionales de la salud involucrados en el diagnóstico y manejo del incidentaloma adrenal. Métodos: Mediante la adopción de la guía para manejo de incidentaloma adrenal de la Sociedad Europea de Endocrinología (AGREE-II y AGREE-REX) y búsqueda complementaria de literatura basada en la mejor evidencia científica disponible en definición, diagnóstico, manejo quirúrgico y seguimiento. Adicionalmente, esta guía aborda pacientes con incidentalomas bilaterales y embarazadas. Resultados: Incidentaloma adrenal se define como una lesión mayor de 1 cm localizado en la suprarrenal, detectada mediante una imagen realizada por una razón diferente a cualquier sospecha de patología adrenal. La gran mayoría son adenomas no funcionantes, que no representan riesgo y no requieren manejo adicional. Sin embargo, existen lesiones tumorales como el carcinoma adrenocortical, el feocromocitoma, adenomas productores de hormonas o metástasis. Conclusiones: Los incidentalomas adrenales son masas predominantemente benignas que no requieren adrenalectomía, no obstante se requiere estudiarlas para descartar patologías que requieran manejo específico. Enfáticamente, la intervención quirúrgica debe guiarse por la probabilidad de malignidad, grado de secreción hormonal, edad, estado de salud y preferencia del paciente.
Objective: To provide updated recommendations to urologists and health-care providers faced to diagnosis and treatment of adrenal incidentaloma. Methods: Through adoption of the adrenal incidentaloma guideline from European Endocrinology Society (AGREE-II and AGREE-REX), and complementary search of literature based on available high-quality scientific evidence for definition, diagnosis, surgical management and follow-up. Additionally, this guideline covers bilateral adrenal incidentalomas and pregnant women. Results: Adrenal incidentaloma is defined as a lesion greater than 1 cm localized in adrenal gland, detected by imaging studies which are requested for another different reason than an adrenal pathology suspicion. A great majority are non-functional adenomas, without life-threatening risk nor additional treatment necessity. However, there are tumoral lesions that demand appropriate management like adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma, or metastasis. Conclusions: Adrenal incidentalomas predominantly are benign masses that do not require adrenalectomy (specially in asymptomatic, unilateral, non-functioning adrenal mass), nevertheless, must be exclude other harmful pathologies. Emphatically, surgical treatment must be indicated by malignant probability, hormonal-secreting status, age, health condition, and patient's preference.
Assuntos
Humanos , Achados IncidentaisRESUMO
ABSTRACT Objective: We investigated the prevalence of adrenal incidentalomas (AIs) in a nonselected Brazilian population in chest computed tomography (CT) performed during the COVID-19 pandemic. Materials and methods: This was a retrospective cross-sectional observational study using chest CT reports from a tertiary in- and outpatient radiology clinic from March to September 2020. AIs were defined by changes in the shape, size, or density of the gland initially identified in the released report. Individuals with multiple studies were included, and duplicates were removed. Exams with positive findings were reviewed by a single radiologist. Results: A total of 10,329 chest CTs were reviewed, and after duplicate removal, 8,207 exams were included. The median age was 45 years [IQR 35-59 years], and 4,667 (56.8%) were female. Thirty-eight lesions were identified in 36 patients (prevalence 0.44%). A higher prevalence was observed with age, with 94.4% of the findings in patients aged 40 years and over (RR 9.98 IC 2.39-41.58, p 0.002), but there was no significant difference between the sexes. Seventeen lesions (44.7%) had more than 10 HU, and five lesions (12.1%) were more than 4 cm. Conclusions: The prevalence of AIs in an unselected and unreviewed population in a Brazilian clinic is low. The impact on the health system caused by AIs discovered during the pandemic should be small regarding the need for specialized follow-up.
RESUMO
Up to 40% of Pheochromocytoma/paraganglioma syndromes are associated with germline mutations. Therefore, they are considered familial and heritable. We report a 65 year old woman with hypertension, bilateral adrenal nodules found in the CT scan and elevated urinary metanephrines. Her genetic testing showed a c.117_120delGTCT TMEM127 gene mutation. She was subjected to a laparoscopic bilateral adrenal excision. After five years of follow up, no recurrence of the disease has been recorded.
Assuntos
Humanos , Feminino , Idoso , Feocromocitoma/cirurgia , Feocromocitoma/genética , Feocromocitoma/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Mutação em Linhagem Germinativa , Predisposição Genética para Doença , Proteínas de Membrana/genética , MutaçãoRESUMO
Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.
Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications
Assuntos
Humanos , Masculino , Feminino , Adulto , Idoso , Mielolipoma/diagnóstico , Neoplasias das Glândulas Suprarrenais/diagnóstico , Mielolipoma/cirurgia , Mielolipoma/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/diagnóstico por imagem , MéxicoRESUMO
CONTEXT: Incidentally discovered adrenal adenomas are common. Assessment for possible autonomous cortisol excess (ACS) is warranted for all adrenal adenomas, given the association with increased cardiometabolic disease. OBJECTIVE: To evaluate the discriminatory capacity of 3-dimensional volumetry on computed tomography (CT) to identify ACS. METHODS: Two radiologists, blinded to hormonal levels, prospectively analyzed CT images of 149 adult patients with unilateral, incidentally discovered, adrenal adenomas. Diameter and volumetry of the adenoma, volumetry of the contralateral adrenal gland, and the adenoma volume-to-contralateral gland volume (AV/CV) ratio were measured. ACS was defined as cortisol ≥ 1.8 mcg/dL after 1-mg dexamethasone suppression test (DST) and a morning ACTHâ ≤â 15. pg/mL. RESULTS: We observed that ACS was diagnosed in 35 (23.4%) patients. Cortisol post-DST was positively correlated with adenoma diameter and volume, and inversely correlated with contralateral adrenal gland volume. Cortisol post-DST was positively correlated with the AV/CV ratio (râ =â 0.46, Pâ <â 0.001) and ACTH was inversely correlated (râ =â -0.28, Pâ <â 0.001). The AV/CV ratio displayed the highest odds ratio (1.40; 95% CI, 1.18-1.65) and area under curve (0.91; 95% CI, 0.86-0.96) for predicting ACS. An AV/CV ratio ≥ 1 (48% of the cohort) had a sensitivity of 97% and a specificity of 70% to identify ACS. CONCLUSION: CT volumetry of adrenal adenomas and contralateral adrenal glands has a high discriminatory capacity to identify ACS. The combination of this simple and low-cost radiological phenotyping can supplement biochemical testing to substantially improve the identification of ACS.
Assuntos
Adenoma , Neoplasias das Glândulas Suprarrenais , Adenoma Adrenocortical , Adenoma/complicações , Adenoma/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Adenoma Adrenocortical/complicações , Adenoma Adrenocortical/diagnóstico por imagem , Hormônio Adrenocorticotrópico , Adulto , Humanos , Hidrocortisona , Tomografia Computadorizada por Raios XRESUMO
PURPOSE: Latin American reports on pheochromocytomas and paragangliomas (PPGLs) are scarce. Recent studies demonstrate changes in clinical presentation and management of these patients. Herein, we assessed the main characteristics of PPGL patients in our academic center over the past 4 decades. METHODS: Demographic, clinical, biochemical, and perioperative data from 105 PPGL patients were retrospectively and prospectively collected over the 1980-2019 period. Data were organized into 4 periods by decade. RESULTS: Age at diagnosis, gender, tumor size and percentage of bilaterality, percentage of paragangliomas, and metastases remained stable across the 4 decades. The proportion of genetic testing and incidentalomas increased in recent decades (all P < 0.001). Therefore, we compared PPGLs diagnosed as incidentalomas (36%) with those clinically suspected (64%). Incidentalomas had fewer adrenergic symptoms (38 vs. 62%; P < 0.001) and lower rates of hypertension (64% vs. 80%; P = 0.01) and hypertensive crisis (28% vs. 44%; P = 0.02); also, they had lower functionality (79% vs. 100%; P = 0.01) and lower catecholamines levels (8.4-fold vs. 12.5-fold above upper cutoffs; P = 0.04). Regarding management of all PPGLs over the decades, we observed significant increases in both perioperative doxazosin dose (P = 0.003) and laparoscopic approach rates (P < 0.001), along with a decrease in the length of hospital stays (P = 0.007). CONCLUSIONS: We observed a change in the clinical presentation of PPGL in recent decades, with a marked increase in incidental cases and milder symptoms. The implementation of a multidisciplinary program for adrenal disorders in our institution has translated into more timely diagnoses, more genetic testing, and improvements in perioperative management.
RESUMO
PURPOSE: There are no data regarding periodontal derangements in patients with adrenal incidentalomas (AI). We assessed the frequency and severity of periodontitis in patients with AI [non-functioning adrenal incidentaloma (NFAI) and possible autonomous cortisol secretion (ACS)] and compared with individuals with normal adrenal. METHODS: A cross-sectional study evaluated thirty-five individuals with AI and 26 controls. NFAI and possible ACS diagnosis was based on the current guidelines: NFAI [cortisol levels after 1 mg dexamethasone suppression test (1 mg-DST) ≤ 1.8 µg/dL (≤ 50 nmol/L)]; possible ACS [cortisol levels after 1 mg-DST 1.9-5.0 µg/dL (51-138 nmol/L)]. Sociodemographic data were collected, and a full-mouth periodontal evaluation was performed. RESULTS: There was no significant difference between groups regarding age, sex, income, ethnicity, education level, smoking, body mass index, dysglycemia, and arterial hypertension. Patients with AI exhibited worse periodontal conditions than controls for the following periodontal clinical parameters: mean percentage of probing pocket depth (PPD) and clinical attachment level (CAL) ≥ 5 mm (p < 0.001 and p = 0.006, respectively). Patients with NFAI and possible ACS showed higher gingival bleeding index (p = 0.014), bleeding on probing (p < 0.001), and CAL (p < 0.001) means compared to controls. The frequencies of periodontitis were 72.7% in patients with NFAI, 84.6% in possible ACS, and 30.8% in controls (p = 0.001). Periodontitis was more severe in patients with possible ACS than NFAI and controls. Patients with NFAI and possible ACS exhibited odds ratio for periodontitis of 4.9 (p = 0.016) and 8.6 (p = 0.02), respectively. CONCLUSION: Patients with AI have higher frequency and severity of periodontitis than controls. The presence of AI was an independent predictive factor for periodontitis.
Assuntos
Neoplasias das Glândulas Suprarrenais , Glândulas Suprarrenais , Hidrocortisona , Periodontite , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/metabolismo , Neoplasias das Glândulas Suprarrenais/fisiopatologia , Glândulas Suprarrenais/metabolismo , Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/fisiopatologia , Estudos Transversais , Diagnóstico Bucal/métodos , Técnicas de Diagnóstico Endócrino , Feminino , Humanos , Hidrocortisona/biossíntese , Hidrocortisona/sangue , Masculino , Pessoa de Meia-Idade , Periodontite/diagnóstico , Periodontite/fisiopatologia , Valor Preditivo dos Testes , Prognóstico , Medição de Risco/métodos , Fatores de Risco , Índice de Gravidade de Doença , Fatores SociodemográficosRESUMO
PURPOSE: Data on endothelial derangements in patients with non-functioning adrenal incidentaloma (NFAI) are scarce. METHODS: We investigated if NFAI patients present clinical, biochemical and endothelial alterations compared to individuals without an adrenal lesion and also the associations among these variables. Forty-two NFAI and 40 controls were evaluated. NFAI diagnosis and controls were defined according to the current guidelines and based on a normal adrenal imaging exam, respectively. Body composition was evaluated by dual emission X-ray absorptiometry. Endothelial reactivity was assessed by two methods: tonometry (Endo-PAT®) and laser speckle contrast imaging (LSCI). RESULTS: There were no differences between groups regarding age, gender, ethnicity, smoking status, and statin use. The frequency of metabolic syndrome according to the International Diabetes Federation criteria was 69% and 57.9%, respectively in NFAI and controls (p = 0.36), whereas the atherosclerotic cardiovascular disease (ASCVD) risk was 63.4% and 66.7% (p = 0.81). The clinical, laboratory, and anthropometric characteristics, as well as body composition, were similar between the groups. Additionally, any differences between groups were observed on endothelial reactivity tests. Nevertheless, we noted an association between cortisol levels after 1 mg-dexamethosone suppression test (1 mg-DST) and the duration of post-occlusive reactive hyperemia tested on microcirculation (r = 0.30; p = 0.03). NFAI patients require more antihypertensive drugs to achieve blood pressure control (p = 0.04). The number of antihypertensive drugs used to control blood pressure correlated with cortisol levels after 1 mg-DST (r = 0.29; p = 0.03). CONCLUSIONS: Since both groups herein investigated had a high frequency of metabolic syndrome and ASCVD risk, it might explain similarities observed on endothelial reactivity. Nevertheless, prolonged reactive hyperemia response on microcirculation was correlated with cortisol levels under suppression.
Assuntos
Neoplasias das Glândulas Suprarrenais/complicações , Biomarcadores/sangue , Doenças Cardiovasculares/diagnóstico , Dexametasona/antagonistas & inibidores , Hidrocortisona/sangue , Hiperemia/diagnóstico , Síndrome Metabólica/diagnóstico , Doenças Cardiovasculares/sangue , Doenças Cardiovasculares/etiologia , Estudos de Casos e Controles , Estudos Transversais , Feminino , Seguimentos , Humanos , Hiperemia/sangue , Hiperemia/etiologia , Masculino , Síndrome Metabólica/sangue , Síndrome Metabólica/etiologia , Pessoa de Meia-Idade , PrognósticoRESUMO
Resumen El ganglioneuroma (Gn) es una neoplasia benigna, rara, derivada de la cresta neural, compuesta de células ganglionares maduras y células de Schwann. Solo una pequeña proporción de Gn se origina en la médula suprarrenal, siendo esta localización más frecuente en niños y adultos jóvenes. Hemos encontrado menos de 30 publicaciones reportadas en la literatura. Presentamos el caso de una paciente femenina de 37 años de edad, con dolor intermitente en fosa renal izquierda y flanco ipsilateral de 5 meses de evolución, sin otros síntomas acompañantes. Se realizó ecografía abdominal, tomografía axial computada (TAC) y resonancia magnética nuclear (RMI) abdomino pélvica, en las cuales se identificó formación expansiva solida, de 85 x 58 x 74 mm, de contornos definidos, adyacente a riñón izquierdo. Las características por imagen no permitieron descartar lesión benigna. Se realizó perfil hormonal completo para despistaje de funcionalidad. Dadas las características imagenológicas y el tamaño de la lesión, se decide tratamiento quirúrgico, considerando a feocromocitoma silente como principal diagnóstico presuntivo pre quirúrgico. La evolución postoperatoria fue excelente. Al examen microscópico, se observa proliferación nodular, de bordes delimitados, formado por células fusadas, con núcleos bipolares y citoplasmas elongados con aisladas células ganglionares. En la inmunomarcación presenta aisladas células ganglionares positivas para Cromogranina A y Sinaptofisina, proteína S100 positivo difuso, hallazgos consistentes con ganglioneuroma suprarrenal. El hallazgo de una masa suprarrenal en paciente joven, sin síntomas específicos, con características no sugestivas de adenoma, debe considerar el diagnostico de ganglioneuroma como parte de las lesiones incidentales adrenales y destacamos la importancia del diagnostico diferencial con feocromocitoma y carcinoma adrenal ya que comparten similares características imagenológicas. El tratamiento de elección es la resección quirúrgica completa, con pronóstico excelente en la mayoría de los casos.
RESUMO
CONTEXT: Data regarding high-resolution peripheral quantitative computed tomography (HR-pQCT) in patients with adrenal incidentaloma (AI) are unknown. PURPOSE: To evaluate the areal bone mineral density (aBMD), microstructure, and fractures in patients with nonfunctioning AI (NFAI) and autonomous cortisol secretion (ACS). METHODS: We evaluated 45 patients with NFAI (1 mg dexamethasone suppression test [DST] ≤1.8 µg/dL) and 30 patients with ACS (1 mg DST 1.9-5.0 µg/dL). aBMD was measured using dual-energy X-ray absorptiometry; vertebral fracture by spine X-ray; and bone geometry, volumetric bone mineral density (vBMD), and microstructure by HR-pQCT. RESULTS: Patients with ACS showed lower aBMD values at the spine, femoral neck, and radius 33% than those with NFAI. Osteoporosis was frequent in both groups: NFAI (64.9%) and ACS (75%). Parameters at the distal radius by HR-pQCT were decreased in patients with ACS compared to those with NFAI: trabecular vBMD (Tb.vBMD, P = 0.03), inner zone of the trabecular region (Inn.Tb.vBMD, P = 0.01), the bone volume/tissue volume ratio (BV/TV, P = 0.03) and trabecular thickness (P = 0.04). As consequence, a higher ratio of the outer zone of the trabecular region/inner zone vBMD (Meta/Inn.vBMD, P = 0.003) was observed. A correlation between the cortisol levels after 1 mg DST and Meta/Inn.vBMD ratio was found (r = 0.29; P = 0.01). The fracture frequency was 73.7% in patients with ACS vs 55.6% in patients with NFAI (P = 0.24). CONCLUSION: Our findings point to an association between trabecular bone microarchitectural derangement at the distal radius and ACS. Our data suggest that AI have a negative impact on bone when assessed by HR-pQCT, probably associated to subclinical hypercortisolism.
Assuntos
Neoplasias das Glândulas Suprarrenais/complicações , Osso Esponjoso/patologia , Síndrome de Cushing/diagnóstico , Fraturas Espontâneas/diagnóstico , Processamento de Imagem Assistida por Computador , Fraturas da Coluna Vertebral/diagnóstico , Tomografia Computadorizada por Raios X/métodos , Absorciometria de Fóton , Córtex Suprarrenal/patologia , Neoplasias das Glândulas Suprarrenais/sangue , Neoplasias das Glândulas Suprarrenais/patologia , Adulto , Idoso , Densidade Óssea , Osso Esponjoso/diagnóstico por imagem , Estudos Transversais , Síndrome de Cushing/sangue , Síndrome de Cushing/etiologia , Feminino , Fraturas Espontâneas/etiologia , Fraturas Espontâneas/patologia , Humanos , Hidrocortisona/sangue , Hidrocortisona/metabolismo , Masculino , Pessoa de Meia-Idade , Fraturas da Coluna Vertebral/etiologia , Fraturas da Coluna Vertebral/patologiaRESUMO
Objective: Germline ARMC5 mutations are considered to be the main genetic cause of primary macronodular adrenal hyperplasia (PMAH). PMAH is associated with high variability of cortisol secretion caused from subclinical hypercortisolism to overt Cushing's syndrome (CS), in general due to bilateral adrenal nodules and rarely could also be due to non-synchronic unilateral adrenal nodules. The frequency of adrenal incidentalomas (AI) associated with PMAH is unknown. This study evaluated germline allelic variants of ARMC5 in patients with bilateral and unilateral AI and in patients with overt CS associated with bilateral adrenal nodules. Methods: We performed a retrospective multicenter study involving 123 patients with AI (64 bilateral; 59 unilateral). We also analyzed 20 patients with ACTH pituitary independent overt CS associated with bilateral adrenal nodules. All patients underwent germline genotyping analysis of ARMC5; abdominal CT and were classified as normal, possible or autonomous cortisol secretion, according to the low doses of dexamethasone suppression test. Results: We identified only one pathogenic allelic variant among the patients with bilateral AI. We did not identify any pathogenic allelic variants of ARMC5 in patients with unilateral AI. Thirteen out of 20 patients (65%) with overt CS and bilateral adrenal nodules were carriers of pathogenic germline ARMC5 allelic variants, all previously described. The germline ARMC5 mutation was observed in only one patient with bilateral AI; it was associated with autonomous cortisol secretion and showed to be a familial form. Conclusion: The rarity of germline ARMC5 mutations in AI points to other molecular mechanisms involved in this common adrenal disorder and should be investigated. In contrast, patients with overt Cushing's syndrome and bilateral adrenal nodules had the presence of ARMC5 mutations that were with high prevalence and similar to the literature. Therefore, we recommend the genetic analysis of ARMC5 for patients with established Cushing's syndrome and bilateral adrenal nodules rather than patients with unilateral AI.
Assuntos
Neoplasias das Glândulas Suprarrenais/genética , Proteínas do Domínio Armadillo/genética , Síndrome de Cushing/genética , Polimorfismo de Nucleotídeo Único , Doenças das Glândulas Suprarrenais/epidemiologia , Doenças das Glândulas Suprarrenais/etiologia , Doenças das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/epidemiologia , Adulto , Alelos , Estudos de Casos e Controles , Síndrome de Cushing/complicações , Síndrome de Cushing/epidemiologia , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Mutação em Linhagem Germinativa , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Primary adrenal failure comprises an insufficient production of mineralocorticoids and glucocorticoids in the adrenal cortex. A rare manifestation of antiphospholipid syndrome (APS) is adrenal failure. The majority of patients with adrenal involvement in APS develop an irreversible cortisol deficiency and atrophy of the adrenal glands. Adrenal incidentalomas are adrenal masses larger than 1 cm that are discovered in the course of diagnostic evaluation or treatment for another medical condition. Its prevalence is calculated in 1.5-9% of individuals. We describe an exceptional case of a 23-year-old male patient with APS with persistent high levels of antiphospholipid antibodies (aPL) from the time of diagnosis, who developed Addison's disease as a manifestation of APS with atrophy of the adrenal glands, in whom an adrenal incidentaloma was developed later and was corroborated as an aldosterone-producing adenoma. Currently, the patient is asymptomatic and without manifestations of tumor recurrence. The protumoral effect of elevated and persistent aPL is discussed.
Assuntos
Doença de Addison/imunologia , Neoplasias das Glândulas Suprarrenais/imunologia , Insuficiência Adrenal/imunologia , Síndrome Antifosfolipídica/complicações , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/etiologia , Insuficiência Adrenal/diagnóstico , Insuficiência Adrenal/etiologia , Anticorpos Antifosfolipídeos/sangue , Humanos , Masculino , Adulto JovemRESUMO
Resumen Objetivo Describir el caso clínico de una paciente con neoplasia oncocitica adrenocortical, tratado quirúrgicamente en una clínica de Lima, Perú. Caso clínico Paciente mujer de 26 años ingresa a emergencia por dolor abdominal inespecífico. Se evidencia tumoración de 15x14x12 cm dependiente de glándula suprarrenal izquierda por lo que se decide tratamiento quirúrgico. Al análisis patológico se evidencia neoplasia oncocítica de potencial maligno incierto. Discusión Las neoplasias oncocíticas adrenocorticales son entidades poco frecuentes, con escasos reportes de casos de esta enfermedad. Para clasificarlas, se usa la escala de Weiss modificada. Obtenemos una neoplasia oncocítica de potencial maligno incierto, cuyo tratamiento incluye la cirugía de resección de tumor y observación. Conclusión Considerar a las neoplasias oncocíticas dentro del diagnóstico diferencial de incidentalomas adrenales.
Objective To describe a case report of a oncocytic adrenocortical neoplasm, treated surgically in a clinic in Lima, Peru. Case report A 26-year-old woman is admitted to the emergency due to nonspecific abdominal pain. A tumor measuring 15x14x12 cm dependent on left adrenal gland is evidenced, so surgical treatment is decided. Pathological analysis evidences oncocytic neoplasia of uncertain malignant potential. Discussion Oncocytic adrenocortical neoplasms are rare entities, with few case reports of this disease. To classify them, the modified Weiss scale is used. We obtain an oncocytic neoplasm of uncertain malignant potential, whose treatment includes surgery for tumor resection and observation. Conclusion Consider oncocytic neoplasms within the differential diagnosis of adrenal incidentalomas.