Emma Anda Apiñániz’s research while affiliated with Universidad de Navarra and other places

Lingual thyroid ectopia is a rare congenital abnormality affecting embryogenesis of the gland descent from the foregut through the pre-tracheal region in the neck. Most cases have an asymptomatic course but may occasionally produce local obstructive symptoms such as dysphagia, dysphonia, dyspnea, pain, or a complication such as bleeding tissues. Diagnostic methods include 99mTc, ¹²³I radionuclide scan, computed tomography scan, magnetic resonance, and ultrasound. No treatment is needed for euthyroid asymptomatic patients unless they present hypothyroidism, which will be treated with LT4. The conventional surgery approach is the elective treatment for cases presenting complaints or complications. Radioactive iodine ablation is the alternative option for patients who refuse surgical intervention or are unfit for anesthesia. We report the case of a 49-year-old woman with an ectopic lingual thyroid presented as oral hemoptysis. Due to the high surgical risk associated with the patient's comorbidities, she successfully received treatment with 592 MBq of ¹³¹I.

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.

Context Ectopic acromegaly is a consequence of rare neuroendocrine tumors (NET) that secrete growth hormone releasing hormone (GHRH). This abnormal GHRH secretion drives growth hormone (GH) and insulin-like growth factor 1 (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 its rarity, the imaging characteristics of the pituitary in ectopic acromegaly have not been analyzed in depth in 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 30 ectopic acromegaly patients due to 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 to 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.

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.

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 ± 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.

Resumen Introducción La determinación de cortisol sérico los primeros días tras la cirugía hipofisaria ha demostrado predecir la insuficiencia adrenal (IA) posquirúrgica. Sin embargo, es controvertida la conveniencia de administrar empíricamente glucocorticoides (GC) para prevenirla. El objetivo es analizar la utilidad del cortisol en el postoperatorio temprano como predictor de la función corticotropa a largo plazo, siguiendo el protocolo periquirúrgico hipofisario con reemplazo de corticoides establecido en nuestro centro. Métodos Se incluyeron 118 pacientes intervenidos en un único centro entre diciembre 2012 y enero 2020 por un adenoma hipofisario. De estos, se excluyeron 54 pacientes por IA previa, enfermedad de Cushing o aquellos que precisaron tratamiento con GC a altas dosis. Se estableció un protocolo de tratamiento con GC que consistía en su administración empírica a dosis rápidamente descendentes y se determinó el cortisol sérico al tercer día poscirugía. Se realizaron sucesivas reevaluaciones de la función adrenal según criterios clínicos y bioquímicos. Resultados De 64 pacientes, 56 presentaban macroadenomas y 8 microadenomas. La incidencia de IA tras cirugía hipofisaria fue del 4,7%. El valor de corte óptimo que predijo una adecuada reserva corticotropa, teniendo en cuenta la mejor relación de especificidad y sensibilidad, fue de ≥ 4,1 μg/dl para el cortisol sérico al tercer día (sensibilidad 95,1%, especificidad 100%). Conclusión El cortisol sérico al tercer día predice el desarrollo de IA. Sugerimos un punto de corte de cortisol sérico al tercer día de la cirugía de ≥ 4,1 μg/dl como predictor de una adecuada reserva adrenal a largo plazo.

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 ± 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.

Resumen Introducción El carcinoma familiar de tiroides no medular (FNMTC, familial non-medulary thyroid carcinoma) se define por la presencia de 2 o más miembros familiares de primer grado con carcinoma diferenciado de tiroides (CDT). El objetivo de este estudio es analizar las características clínicas, anatomopatológicas y pronósticas del FNMTC respecto al carcinoma esporádico (CE). Materiales y métodos Estudio retrospectivo de los casos de CDT registrados en nuestro centro hospitalario durante el periodo 1990-2018. Resultados Se analizó a un total de 927 pacientes, de los cuales 61 son FNMTC, con un seguimiento medio de 9,7 ± 6,5 años. La prevalencia del FNMTC es del 6,6%, presentando un estadio TNM inicial más bajo (p = 0,003) debido a la mayor proporción de tumores inferiores a 2 cm (p = 0,003), además de ser más multifocales (p = 0,034) y del subtipo histológico papilar (p = 0,022), respecto al CE. No se detectaron diferencias significativas en la edad al diagnóstico (p = 0,347), género (p = 0,406), ni en otros marcadores de agresividad (bilateralidad, extensión extratiroidea, afectación ganglionar y metástasis). La tasa de persistencia/recurrencia (p = 0,656), supervivencia libre de enfermedad (p = 0,929) y mortalidad ocasionada por el propio tumor (p = 0,666) fueron comparables. En las familias con ≥ 3 familiares afectados, los tumores son más pequeños (p = 0,005), más multifocales (p = 0,040) y bilaterales (p = 0,002), además de haber una mayor proporción de hombres (p = 0,020). Los pacientes de la segunda generación presentaron al diagnóstico el FNMTC a una edad más precoz respecto a los de la primera (p = 0,001). Conclusión El FNMTC presenta en nuestro estudio, en comparación con el CE, una estadificación TNM más baja, mayor multifocalidad y representación del subtipo papilar, con similares datos de agresividad y sin diferencias en el pronóstico.