Utility of the continuous intraoperative neuromonitoring in the prevention of the recurrent laryngeal nerve paralysis during thyroid surgery. A prospective observational study / Utilidad de la neuromonitorización continua intraoperatoria en la prevención de la parálisis del nervio laríngeo recurrente durante la cirugía tiroidea. Un estudio observacional prospectivo

Background: The continuous intraoperative neuromonitoring (C-IONM) of the recurrent laryngeal nerve (RLN) could help reducing the incidence of nerve paralysis after thyroid surgery, in comparison with the mere anatomical visualization of the RLN. The objective is to assess the efficacy and utility of C-IONM as a predictive test for recurrent laryngeal nerve paralysis after thyroidectomy. Methods: A prospective observational study was performed in 248 patients who underwent thyroid surgery where C-IONM was applied, between September 2018 and December 2019, in a high-volume center. A previous and later laryngoscopy was performed, which allowed to evaluate the reliability of the C-IONM as a predictive test for recurrent paralysis. Sensitivity (SE), specificity (SP), positive predictive value (PPV), negative predictive value (NPV) were studied. Results: A total number of 171 thyroidectomies, 62 hemithyroidectomies, 15 totalization thyroidectomies and 27 thyroidectomy with cervical dissections were performed. Postoperative laryngoscopy was altered in 40 patients (16.12%). The SE, SP, PPV and NPV values ​​were 65%, 94.7%, 70.2% and 93.4% respectively. Conclusions: C-IONM is a safe technique that provides real-time information about anatomical and functional integrity of the RLN and can improve the results of thyroid surgery. (AU)

Introducción: La neuromonitorización continua intraoperatoria (C-IONM) del nervio laríngeo recurrente (RLN) puede contribuir a la reducción de la incidencia de parálisis nerviosa tras una cirugía tiroidea, en comparación con la mera visualización anatómica del RLN. El objetivo de este estudio es evaluar la fiabilidad de la NCIO de los nervios laríngeos recurrentes como prueba predictora de parálisis vocal postoperatoria. Material y métodos: Se trata de un estudio observacional prospectivo realizado en 248 pacientes intervenidos de patología tiroidea en los que se aplicó la C-IONM, entre Septiembre de 2018 y Diciembre de 2019 en un único centro de alto volumen. Se realizó una laringoscopia pre y post operatoria a todos los pacientes, lo que pudo determinar la fiabilidad de la C-IONM como test predictivo de la parálisis recurrencial tras cirugía tiroidea. Se hallaron los valores de sensibilidad (SE), especificidad (SP), valor predictivo positivo (PPV) y valor predictivo negativo (NPV) de dicha técnica. Resultados: Se realizaron un total de 171 tiroidectomías totales, 62 hemitiroidectomías, 15 tiroidectomías de totalización y 27 tiroidectomías con disección cervical. Se hallaron alteraciones en la laringoscopia postoperatoria en 40 pacientes (16,12%). Los valores de SE, SP, PPV y NPV fueron 65%, 94.7%, 70.2% y 93.4% respectivamente. Conclusiones: La C-IONM es una técnica segura que ofrece información anatómica y funcional del NLR en tiempo real y puede contribuir a mejorar los resultados de la cirugía tiroidea. (AU)

Evaluation of a perioperative steroid coverage after pituitary surgery.

INTRODUCTION: Serum cortisol levels within the first days after pituitary surgery have been shown to be a predictor of post-surgical adrenal insufficiency. However, the indication of empirical glucocorticoids to avoid this complication remains controversial. The objective is to assess the role of cortisol in the early postoperative period as a predictor of long-term corticotropic function according to the pituitary perisurgical protocol with corticosteroid replacement followed in our center. METHODS: One hundred eighteen patients who underwent surgery in a single center between December 2012 and January 2020 for a pituitary adenoma were included. Of these, 54 patients with previous adrenal insufficiency (AI), Cushing's disease, or tumors that required treatment with high-dose glucocorticoids (GC) were excluded. A treatment protocol with glucocorticoids was established, consisting of its empirical administration at rapidly decreasing doses, and serum cortisol was determined on the third day after surgery. Subsequent adrenal status was assessed through follow-up biochemical and clinical evaluations. RESULTS: Out of the 64 patients treated, there were 56 macroadenomas and 8 microadenomas. The incidence of adrenal insufficiency after pituitary surgery was 4.7%. The optimal cut-off value that predicted an adequate corticotropic reserve, taking into account the best relationship of specificity and sensitivity, was ≥4.1 µg/dl for serum cortisol on the third day (sensitivity 95.1%, specificity 100%). CONCLUSION: Serum cortisol on the third day predicts the development of adrenal insufficiency. We suggest a cortisol cut-off point of ≥4.1 µg/dl on postoperative on the third day after surgery as a predictor of the adrenal reserve in the long-term.

Pituitary MRI Features in Acromegaly Resulting From Ectopic GHRH Secretion From a Neuroendocrine Tumor: Analysis of 30 Cases.

CONTEXT: Ectopic acromegaly is a consequence of rare neuroendocrine tumors (NETs) that secrete GHRH. This abnormal GHRH secretion drives GH and IGF-1 excess, with a clinical presentation similar to classical pituitary acromegaly. Identifying the underlying cause for the GH hypersecretion in the setting of ectopic GHRH excess is, however, essential for proper management both of acromegaly and the NET. Owing to the rarity of NETs, the imaging characteristics of the pituitary in ectopic acromegaly have not been analyzed in depth in a large series. OBJECTIVE: Characterize pituitary magnetic resonance imaging (MRI) features at baseline and after NET treatment in patients with ectopic acromegaly. DESIGN: Multicenter, international, retrospective. SETTING: Tertiary referral pituitary centers. PATIENTS: Thirty ectopic acromegaly patients having GHRH hypersecretion. INTERVENTION: None. MAIN OUTCOME MEASURE: MRI characteristics of pituitary gland, particularly T2-weighted signal. RESULTS: In 30 patients with ectopic GHRH-induced acromegaly, we found that most patients had hyperplastic pituitaries. Hyperplasia was usually moderate but was occasionally subtle, with only small volume increases compared with normal ranges for age and sex. T2-weighted signal was hypointense in most patients, especially in those with hyperplastic pituitaries. After treatment of the NET, pituitary size diminished and T2-weighted signal tended to normalize. CONCLUSIONS: This comprehensive study of pituitary MRI characteristics in ectopic acromegaly underlines the utility of performing T2-weighted sequences in the MRI evaluation of patients with acromegaly as an additional tool that can help to establish the correct diagnosis.

Clinical characteristics and prognosis of familial nonmedullary thyroid carcinoma.

INTRODUCTION: Familial non-medullary thyroid carcinoma (FNMTC) is defined by the presence of 2 or more first-degree family members with differentiated thyroid carcinoma (DTC). The aim of this study is to compare clinicopathological features and prognosis of FNMTC and sporadic carcinoma (SC). MATERIALS AND METHODS: Retrospective study of DTC included in the hospital database during the period 1990-2018. RESULTS: A total of 927 patients were analyzed, 61 of them were FNMTC, with a mean follow-up of 9.7 ±â€¯6.5 years. The prevalence of FNMTC was 6.6%, with a lower TNM staging presentation (P = 0.003) consequence of a higher proportion of tumors smaller than 2 cm (P = 0.003), combined with a greater multifocality (P = 0.034) and papillary histologic subtype (P = 0.022) compared to SC. No significant differences in age at diagnosis (P = 0.347), gender (P = 0.406), neither in other aggressiveness markers (bilaterality, extrathyroidal extension, lymph node involvement and metástasis) were detected. Rate of persistence/recurrence (P = 0.656), disease-free survival (P = 0.929) and mortality caused by the tumor itself (P = 0.666) were comparable. Families with ≥3 affected relatives, had smaller tumors (P = 0.005), more multifocality (P = 0.040) and bilaterality (P = 0.002), as well as a higher proportion of males (P = 0.020). Second generation patients present earlier FNMTC compared to those of the first generation (P = 0.001). CONCLUSION: In our study FNMTC presents a lower TNM staging, higher multifocality and papillary variant, with similar aggressiveness and prognosis compared to SC.

Thyroid disorders associated with immune control point inhibitors.

INTRODUCTION: Immune checkpoint inhibitors (ICPI) have improved progression-free survival in several solid tumors. Side effects are related to overstimulation of the immune system. Thyroid dysfunction (TD) is the most common endocrine immune-related adverse event of ICPI. OBJECTIVE: To describe the clinical presentation and the course of TD in cancer patients treated with ICPI referred to an endocrinology outpatient clinic. MATERIAL AND METHODS: This was a descriptive, retrospective and multicenter study of patients with TD associated with ICPI in six Spanish hospitals. RESULTS: 120 patients (50.8% women), mean age 60 ±â€¯12 years were included. The initial TD was hypothyroidism in 49% of patients and hyperthyroidism in 51%, with an average of 76 (41-140) and 43 (26-82) days respectively between the onset of ICPI and the analytical alteration. Significantly, the earlier the first analytical determination was, the greater the prevalence of hyperthyroidism. A turnover was observed in 80% of subjects during follow-up, mostly from hyperthyroidism to hypothyroidism. Twenty-one percent received double ICPI therapy. The most frequent form of presentation in monotherapy was hypothyroidism (57%), and in double therapy it was hyperthyroidism (77%) (p = 0.002). Patients under double therapy showed thyroid alterations earlier than those in the monotherapy group (p = 0.001). After a follow-up of 205 (112-360) days, half of the patients continued under levothyroxine treatment. CONCLUSIONS: Hypothyroidism and hyperthyroidism present in a similar proportion in cancer patients undergoing ICPI therapy. Our results suggest that transitory hyperthyroidism may not be detected in a relevant number of cases. In addition, TD in double therapy presents earlier. This should be taken into account in the follow-up protocols of these patients.

Clinical characteristics and prognosis of familial nonmedullary thyroid carcinoma. / Características clínicas y pronósticas del carcinoma familiar de tiroides no medular.

INTRODUCTION: Familial non-medullary thyroid carcinoma (FNMTC) is defined by the presence of 2or more first-degree family members with differentiated thyroid carcinoma (DTC). The aim of this study is to compare clinicopathological features and prognosis of FNMTC and sporadic carcinoma (SC). MATERIALS AND METHODS: Retrospective study of DTC included in the hospital database during the period 1990-2018. RESULTS: A total of 927 patients were analyzed, 61 of them were FNMTC, with a mean follow-up of 9.7±6.5 years. The prevalence of FNMTC was 6.6%, with a lower TNM staging presentation (P=.003) consequence of a higher proportion of tumors smaller than 2 centimeters (P=.003), combined with a greater multifocality (P=.034) and papillary histologic subtype (P=.022) compared to SC. No significant differences in age at diagnosis (P=.347), gender (P=.406), neither in other aggressiveness markers (bilaterality, extrathyroidal extension, lymph node involvement and metástasis) were detected. Rate of persistence/recurrence (P=.656), disease-free survival (P=.929) and mortality caused by the tumor itself (P=.666) were comparable. Families with ≥3 affected relatives, had smaller tumors (P=.005), more multifocality (P=.040) and bilaterality (P=.002), as well as a higher proportion of males (P=.020). Second generation patients present earlier FNMTC compared to those of the first generation (P=.001). CONCLUSION: In our study FNMTC presents a lower TNM staging, higher multifocality and papillary variant, with similar aggressiveness and prognosis compared to SC.

The extent of surgery for low-risk 1-4 cm papillary thyroid carcinoma: a catch-22 situation. A retrospective analysis of 497 patients based on the 2015 ATA Guidelines recommendation 35.

PURPOSE: The adequate extent of surgery for 1-4 cm low-risk papillary thyroid carcinoma (PTC) is unclear. Our objective was to analyze the applicability of the 2015 ATA Guidelines recommendation 35B (R35) for the management low-risk PTC. METHODS: This multicentre study included patients with low-risk PTC who had undergone total thyroidectomy (TT). Retrospectively we selected those who met the R35 criteria for the performance of a thyroid lobectomy (TL). The aim was to identify the proportion of low-risk PTC patients treated using TT who would have required reintervention had they had a TL in accordance with R35. RESULTS: We identified 497 patients (400 female; 80.5%). Median tumor size (mm): 21.2 (11-40). A tumor size ≥2 cm was found in 252 (50.7%). Most of them, 320 (64.4%), were in Stage I (AJCC 7th Edition). Following R35, 286 (57.5%) would have needed TT. Thus, they would have required a second surgery had they undergone TL. The indications for reintervention would have included lymph node involvement (35%), extrathyroidal extension (22.9%), aggressive subtype (8%), or vascular invasion (22.5%). No presurgical clinical data predict TT. CONCLUSIONS: The appropriate management of low-risk PTC is unclear. Adherence to ATA R35 could lead to a huge increase in reinterventions when a TL is performed, though the need for them would be questionable. In our sample, more than half of patients (57.5%) who may undergo a TL for a seemingly low-risk PTC would have required a second operation to satisfy international guidelines, until better preoperative diagnostic tools become available.

Comparación de las características clínicas en pacientes con carcinoma folicular de tiroides y carcinoma de células de Hürthle / Comparison of clinical characteristics of patients with follicular thyroid carcinoma and Hürthle cell carcinoma

Introducción: El carcinoma de células de Hürthle (CCH) es un tipo de cáncer de tiroides infrecuente considerado históricamente una variante del carcinoma folicular de tiroides (CFT). El objetivo de este estudio fue conocer las diferencias que existen entre estos grupos en cuanto a los factores clínicos y pronósticos. Pacientes y métodos: Se incluyeron 230 pacientes (153 CFT y 77 CCH) con un seguimiento mediano de 13,4 años. Se compararon las diferentes características utilizando el programa estadístico SPSS versión 20. Resultados: Los pacientes con CCH tenían mayor edad (57,3±13,8 años vs. 44,6±15,2 años; p<0,001). También se observaron estadios TNM más avanzados en los CCH, con una mayor tendencia a presentar metástasis a distancia (7,8% vs. 2,7%; p=0,078). El porcentaje de persistencia/recurrencia al finalizar el seguimiento del estudio fue mayor entre los pacientes con CCH (13% vs. 3,9%; p=0,011). Sin embargo, en el análisis multivariante, solo la edad (hazard ratio [HR]: 1,10; intervalo de confianza [IC]: 1,04-1,17; p=0,001), el tamaño (HR: 1,43; IC: 1,05-1,94; p=0,021) y el subtipo histológico (HR: 9,79; IC: 2,35-40,81; p=0,002) se asociaron de forma significativa con el pronóstico, pero no el presentar un CCH. Conclusión: El CCH se diagnostica en pacientes de mayor edad y en estadios más avanzados que el CFT. Sin embargo, si la edad, el tamaño y el subtipo histológico son similares, la supervivencia libre de enfermedad no difiere en ambos grupos (AU)

Introduction: Hürthle cell carcinoma (HCC) is an uncommon thyroid cancer historically considered to be a variant of follicular thyroid carcinoma (FTC). The aim of this study was to assess the differences between these groups in terms of clinical factors and prognoses. Patients and methods: A total of 230 patients (153 with FTC and 77 with HCC) with a median follow-up of 13.4 years were studied. The different characteristics were compared using SPSS version 20 statistical software. Results: Patients with HCC were older (57.3±13.8 years vs. 44.6±15.2 years; P<.001). More advanced TNM stages were also seen in patients with HCC and a greater trend to distant metastases were also seen in patients with HCC (7.8% vs. 2.7%, P=.078). The persistence/recurrence rate at the end of follow-up was higher in patients with HCC (13% vs. 3.9%, P=.011). However, in a multivariate analysis, only age (hazard ratio [HR] 1.10, confidence interval [CI] 1.04-1.17; P=.001), size (HR 1.43, CI 1.05-1.94; P=.021), and histological subtype (HR 9.79, CI 2.35-40.81; P=.002), but not presence of HCC, were significantly associated to prognosis. Conclusion: HCC is diagnosed in older patients and in more advanced stages as compared to FTC. However, when age, size, and histological subtype are similar, disease-free survival is also similar in both groups (AU)

Comparison of clinical characteristics of patients with follicular thyroid carcinoma and Hürthle cell carcinoma. / Comparación de las características clínicas en pacientes con carcinoma folicular de tiroides y carcinoma de células de Hürthle.

INTRODUCTION: Hürthle cell carcinoma (HCC) is an uncommon thyroid cancer historically considered to be a variant of follicular thyroid carcinoma (FTC). The aim of this study was to assess the differences between these groups in terms of clinical factors and prognoses. PATIENTS AND METHODS: A total of 230 patients (153 with FTC and 77 with HCC) with a median follow-up of 13.4 years were studied. The different characteristics were compared using SPSS version 20 statistical software. RESULTS: Patients with HCC were older (57.3±13.8 years vs. 44.6±15.2 years; P<.001). More advanced TNM stages were also seen in patients with HCC and a greater trend to distant metastases were also seen in patients with HCC (7.8% vs. 2.7%, P=.078). The persistence/recurrence rate at the end of follow-up was higher in patients with HCC (13% vs. 3.9%, P=.011). However, in a multivariate analysis, only age (hazard ratio [HR] 1.10, confidence interval [CI] 1.04-1.17; P=.001), size (HR 1.43, CI 1.05-1.94; P=.021), and histological subtype (HR 9.79, CI 2.35-40.81; P=.002), but not presence of HCC, were significantly associated to prognosis. CONCLUSION: HCC is diagnosed in older patients and in more advanced stages as compared to FTC. However, when age, size, and histological subtype are similar, disease-free survival is also similar in both groups.

Aumento de la incidencia de cáncer de tiroides en Navarra. Evolución y características clínicas, 1986-2010 / Increased incidence of thyroid cancer in Navarra (Spain). Evolution and clinical characteristics, 1986-2010

Introducción: En los últimos estudios publicados se observa un aumento de la incidencia de cáncer de tiroides a nivel mundial. El objetivo del presente estudio fue analizar los cambios en la incidencia de cáncer de tiroides en Navarra, y su presentación en cuanto a sexo, subtipo histológico y tamaño a lo largo de los últimos 25 años. Métodos: Se calcularon las tasas de incidencia de cáncer de tiroides a partir de los datos del Registro de Cáncer de Navarra durante el periodo 1986-2010. Los datos clínicos se obtuvieron de la cohorte histórica del Registro Hospitalario de Cáncer de Navarra, que incluye todos los nuevos casos de carcinoma diferenciado de tiroides diagnosticados y tratados en la red sanitaria pública de esta comunidad en dicho periodo. Resultados: La incidencia global de cáncer de tiroides en Navarra ha aumentado en estos 25 años con un incremento en la tasa ajustada en varones de 2,24 (1986-1990) a 5,85 (2006-2010) por 100.000 habitantes/año (p<0,001), y en mujeres de 9,05 a 14,04, respectivamente (p<0,001). Este aumento se produjo únicamente a expensas del carcinoma papilar. En el ámbito hospitalario, se estudiaron 739 pacientes con cáncer diferenciado de tiroides. La edad media al diagnóstico aumentó a lo largo de los años y el predominio de mujeres (alrededor del 80%) se mantuvo estable. El tamaño tumoral medio disminuyó a lo largo de los quinquenios de 30,9 a 22,5mm (p<0,001), la proporción de microcarcinomas (T1a) aumentó de 8,8% a 30% (p<0,001) y, a pesar de este aumento, no hubo diferencias estadísticas en el estadio TNM al diagnóstico durante el periodo de estudio. La distribución de las variantes histológicas de carcinoma papilar y folicular no se modificó a lo largo de los 25 años. Conclusiones: Durante el período estudiado la incidencia de cáncer de tiroides ha aumentado en Navarra en ambos sexos. El aumento se ha producido a expensas del carcinoma papilar, sin cambios en la distribución de las variantes histológicas. Destaca el aumento en la proporción de tumores T1a, pero se mantiene la distribución por estadio TNM. Estos resultados sugieren un incremento del diagnóstico de microcarcinomas tiroideos por cambios en la práctica clínica, sin descartar además un incremento real de la incidencia del carcinoma papilar en Navarra (AU)

Introduction: The latest published studies show an increased incidence of thyroid cancer worldwide. The aim of this study was to analyze the changes in the incidence of thyroid cancer in Navarra and its clinical presentation regarding sex, histological subtype and size over the last 25 years. Methods: Thyroid cancer incidence rates were calculated on the basis of data from the Cancer Registry of Navarra during 1986-2010. Clinical data were obtained from the historical cohort of the Hospital Registry of Cancer of Navarra, which includes all the new cases of differentiated thyroid carcinoma diagnosed and treated in the public health network of this Community in that period. Results: The overall incidence of thyroid cancer in Navarra increased over the last 25 years, with an increase in the adjusted rate in men from 2.24 (1986-1990) to 5.85 (2006-2010) per 100,000 population/year (P<.001) and in women from 9.05 to 14.04, respectively (P<.001). This increase occurs only in papillary carcinoma. The clinical characteristics of 739 patients with differentiated thyroid cancer were studied. The mean age at diagnosis increased over the years and the predominance of women (about 80%) remains stable. Mean tumor size decreased over the five-year periods from 30.9 to 22.5mm (P<.001), the proportion of microcarcinomas (T1a) increased from 8.8% to 30% (P<.001) and, despite this increase, there were no statistical differences in the TNM stage at diagnosis during the study period. The distribution of histological variants of papillary and follicular carcinoma did not change over 25 years. Conclusions: During the period studied, the incidence of thyroid cancer increased in Navarra in both sexes. The increase occurred only in papillary carcinoma, without changes in the distribution of his histological variants. The increase in the proportion of T1a tumors is remarkable, but the TNM stage distribution was maintained. These results suggest an increase in the diagnosis of thyroid microcarcinomas due to changes in clinical practice, without ruling out a real increase in the incidence of papillary carcinoma in Navarra (AU)

Increased incidence of thyroid cancer in Navarra (Spain). Evolution and clinical characteristics, 1986-2010. / Aumento de la incidencia de cáncer de tiroides en Navarra. Evolución y características clínicas, 1986-2010.

INTRODUCTION: The latest published studies show an increased incidence of thyroid cancer worldwide. The aim of this study was to analyze the changes in the incidence of thyroid cancer in Navarra and its clinical presentation regarding sex, histological subtype and size over the last 25 years. METHODS: Thyroid cancer incidence rates were calculated on the basis of data from the Cancer Registry of Navarra during 1986-2010. Clinical data were obtained from the historical cohort of the Hospital Registry of Cancer of Navarra, which includes all the new cases of differentiated thyroid carcinoma diagnosed and treated in the public health network of this Community in that period. RESULTS: The overall incidence of thyroid cancer in Navarra increased over the last 25 years, with an increase in the adjusted rate in men from 2.24 (1986-1990) to 5.85 (2006-2010) per 100,000 population/year (P<.001) and in women from 9.05 to 14.04, respectively (P<.001). This increase occurs only in papillary carcinoma. The clinical characteristics of 739 patients with differentiated thyroid cancer were studied. The mean age at diagnosis increased over the years and the predominance of women (about 80%) remains stable. Mean tumor size decreased over the five-year periods from 30.9 to 22.5mm (P<.001), the proportion of microcarcinomas (T1a) increased from 8.8% to 30% (P<.001) and, despite this increase, there were no statistical differences in the TNM stage at diagnosis during the study period. The distribution of histological variants of papillary and follicular carcinoma did not change over 25 years. CONCLUSIONS: During the period studied, the incidence of thyroid cancer increased in Navarra in both sexes. The increase occurred only in papillary carcinoma, without changes in the distribution of his histological variants. The increase in the proportion of T1a tumors is remarkable, but the TNM stage distribution was maintained. These results suggest an increase in the diagnosis of thyroid microcarcinomas due to changes in clinical practice, without ruling out a real increase in the incidence of papillary carcinoma in Navarra.

Estruma ovárico maligno metastásico: 2 escenarios de una misma enfermedad / Metastatic malignant struma ovarii: Two scenarios of the same pathology

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Inédita coincidencia: feocromocitoma sobre glándula suprarrenal atrófica por enfermedad de Addison / An unusual association: pheochromocytoma on an atrophied adrenal gland due to Addison´s disease

El síndrome pluriglandular autoinmunitario (SPGA) tipo II es el síndrome inmunoendocrinopatológico más frecuente. Se define por la aparición de2 o más de las siguientes entidades: insuficiencia suparrenal primaria(enfermedad de Addison), enfermedad de Graves, diabetes mellitus tipo1A, tiroiditis autoinmunitaria, hipogonadismo primario, enfermedad celíaca o miastenia grave. Asimismo, es frecuente que aparezcan también vitíligo, alopecia, anemia perniciosa y/o serositis. La insuficiencia suprarrenal primaria con la que cursan estos pacientes afecta a la corteza adrenal, y ésta es destruida por los auto anticuerpos contra la 21-hidroxilasa. A diferencia de las demás etiologías de la insuficiencia suprarrenal (enfermedades infecciosas, enfermedades infiltrativas, hemorragia, enfermedades tumorales), respeta la médula adrenal. Los feocromocitomas son tumores derivados de las células cromafines del sistema nervioso simpático situadas en la médula adrenal. Pueden cursar con manifestaciones clínicas muy variadas, desde una hipertensión arterial (HTA) aislada o acompañada de episodios paroxísticos, que incluyen la clásica tríada de cefalea, palpitaciones y diaforesis, hasta cuadros potencialmente graves, como edema agudo de pulmón, arritmias o muerte súbita. No hay que olvidar que hasta el 40% son asintomáticos. A continuación, presentamos el caso de una paciente diagnosticada de SPGA tipo II, que desarrolla un feocromocitoma. En esta ocasión, en una glándula adrenal cortical atrofiada se desarrolla un tumor dependiente de la médula adrenal. Esta coexistencia de endocrinopatías, sin conexión etiológica alguna, no deja de ser cuando menos un hallazgo sorprendente, no descrito hasta el momento en la literatura actual (AU)

Autoimmune polyendocrine syndrometype II (APS-II) is the most commonimmunoendocrinopathy syndrome. APS-IIis defined by the development of two or more of the following entities: primaryadrenal insufficiency (Addison’s disease),Graves’ disease, type 1A diabetes mellitus,autoimmune thyroiditis, primaryhypogonadism, celiac disease, and myasthenia gravis. Other frequent clinical findings are vitiligo, alopecia, pernicious anemia and/or serositis. Primary adrenal insufficiency in these patients affects the adrenal cortex, which is destroyed byautoantibodies against 21-hydroxylase.Unlike other causes of adrenal insufficiency (infectious diseases, infiltrative diseases, bleeding, tumors), the adrenal medulla is not involved. Pheochromocytomas are tumors arising from the chromaffin cells of the sympathetic nervous system in the adrenal medulla. The clinical symptoms of these tumors vary from isolated hypertension or hypertension accompanied by paroxysmal episodes —including the classical triad of headache, palpitations and diaphoresis—to potentially serious manifestations such as acute pulmonary edema, arrhythmias and sudden death. Nevertheless, up to 40%of affected patients are asymptomatic. We present the case of a patient diagnosed with APS-II who developed apheochromocytoma. In this patient, the adrenal gland cortex was atrophied andthe tumor was attached to the adrenal medulla. This coexistence ofendocrinopathies, with no etiologic connection, is a surprising finding, which has not previously been described in the current literatura (AU)

An unusual association: pheochromocytoma on an atrophied adrenal gland due to addison's disease.

Autoimmune polyendocrine syndrome type II (APS-II) is the most common immunoendocrinopathy syndrome. APS-II is defined by the development of two or more of the following entities: primary adrenal insufficiency (Addison's disease), Graves' disease, type 1A diabetes mellitus, autoimmune thyroiditis, primary hypogonadism, celiac disease, and myasthenia gravis. Other frequent clinical findings are vitiligo, alopecia, pernicious anemia and/or serositis. Primary adrenal insufficiency in these patients affects the adrenal cortex, which is destroyed by autoantibodies against 21-hydroxylase. Unlike other causes of adrenal insufficiency (infectious diseases, infiltrative diseases, bleeding, tumors), the adrenal medulla is not involved. Pheochromocytomas are tumors arising from the chromaffin cells of the sympathetic nervous system in the adrenal medulla. The clinical symptoms of these tumors vary from isolated hypertension or hypertension accompanied by paroxysmal episodes -including the classical triad of headache, palpitations and diaphoresis-to potentially serious manifestations such as acute pulmonary edema, arrhythmias and sudden death. Nevertheless, up to 40% of affected patients are asymptomatic. We present the case of a patient diagnosed with APS-II who developed a pheochromocytoma. In this patient, the adrenal gland cortex was atrophied and the tumor was attached to the adrenal medulla. This coexistence of endocrinopathies, with no etiologic connection, is a surprising finding, which has not previously been described in the current literature.