Current Management of Head and Neck Paragangliomas: A Multicenter Series With Long-Term Follow-Up.

OBJECTIVE, STUDY DESIGN, SETTING, METHODS: To improve our knowledge about head and neck paragangliomas (HN-PGL), the clinical characteristics of all the patients with HN-PGL included in the Spanish Registry were analyzed, as well as the treatment modalities and their outcomes. RESULTS: A total of 202 patients, 67.8% women, aged 53.1 ± 17.4, with 264 HN-PGL, from 16 Spanish hospitals, were included. Tumors were located in the carotid body in 61%, jugular bulb in 20.5%, tympanic area in 10.2%, and along the vagal nerve in 6.4%. Multiple tumors developed in 20.8% and metastatic disease in 4%. A genetic study was done in 64.4% and showed a pathogenic variant in SDHx in 50%. These patients were younger, with no sex predominance, and had more multiple and metastatic tumors. Tumors were treated by surgery in 134 patients, radiotherapy in 33, and other treatments in 20, and were observed without active treatment in 41 patients, who were older, more often asymptomatic, and had smaller tumors. Tumors treated with radiotherapy were larger and more often in locations other than the carotid body. After a median follow-up of 80 months (interquartile range: 41-136), 6.9% had died; among the survivors, 48.4% were disease-free, 42.1% stable, and 9.4% had progressed. The rate of sequelae was similar among patients submitted to surgery, radiotherapy, or observation. We could not identify any prognostic factor for progression. CONCLUSION: Most HN-PGL are localized slow-growing tumors. Long-term survival is high, even in case of metastasis. Although surgery is the most common treatment, radiotherapy and active surveillance are safe approaches.

Differences between Patients with Sporadic and Familial Pheochromocytoma-Is It Possible to Avoid Genetic Testing in Certain Patients?

BACKGROUND: Pheochromocytoma (PHEO) is a rare neuroendocrine tumour with a strong genetic link, which therefore may modify its clinical behaviour and prognosis. The aim of the study is to evaluate the epidemiological and clinical differences between patients with sporadic and familial PHEO, as well as the specific differences in the index cases. METHODS: A retrospective analysis of 136 patients in a tertiary hospital (1984-2021). Epidemiological, clinical, and histological variables were analysed. STATISTICS: SPSS 28.0 software was used. Univariate and multivariate logistic regression analyses were performed. p < 0.05 was considered statistically significant. RESULTS: 64.71% of the cases (n = 88) presented a genetic mutation (familial cases). Additionally, 32.39% (n = 23) corresponded to index cases and the rest to screening cases. The main differences between patients with familial and sporadic PHEO were age (OR = 0.93 (0.89-0.97)), blood pressure-related symptoms (OR = 0.22 (0.06-0.89)), bilaterality (OR = 15.49 (3.76-63.84)), and size (OR = 0.70 (0.54-0.92)). Among patients with sporadic PHEO and index cases, only bilaterality was significant (OR = 13.53 (1.24-144.34)). CONCLUSIONS: Patients with familial PHEO diagnosed by screening differ from sporadic cases in terms of age, clinical features, and size. However, patients with sporadic PHEO only differ from index cases by a lower presence of bilaterality, which reaffirms the importance of genetic screening of patients with PHEO and their relatives.

Improvement of mood and sleep quality in patients with primary hyperparathyroidism after parathyroidectomy: A prospective case-control study.

BACKGROUND: Primary hyperparathyroidism can impact patient quality of life with psychopathological symptoms like mood and sleep disturbances. In the latest recommendations published according to the primary hyperparathyroidism surgical consensus, these neuropsychological symptoms continue to be excluded. This study aims to assess the negative effects of mood and sleep on patients with primary hyperparathyroidism compared to healthy controls and analyze their improvement after surgery. METHODS: Prospective case-control study of patients with primary hyperparathyroidism. The Beck questionnaire and Pittsburgh Sleep Quality Index were used. The control group consisted of healthy people matched by age and sex. Preoperative results were compared with results at 3 and 12 months after surgery and compared with the control group. Statistical analysis consisted of the Kolmogorov-Smirnov test and Student's t test for correlations. RESULTS: Forty-nine patients who underwent parathyroidectomy were analyzed. In relation to depression, differences were observed between the results of the preoperative period and 3 months after surgery, with a significant decrease in depression score (16.80 ± 9.98 vs 13.08 ± 10.76; P = .001). This improvement was maintained after 1 year (P < .001). Regarding the quality of sleep, there were no differences 3 months after the intervention, but there were differences at 12 months (9.48 ± 4.76 vs 8.27 ± 4.38; P = .032). The dimensions of the Pittsburgh Sleep Quality Index were analyzed, observing significant differences only in daytime dysfunction after 3 months and 12 months after surgery (1.02 ± 0.99 vs 0.69 ± 0.82; P = .01 and 1.04 ± 0.98 vs 0.60 ± 0.76; P = .004). CONCLUSION: Mood changes and sleep quality improve after primary hyperparathyroidism surgery, although at different postoperative times, with this improvement being more pronounced in mood. This assessment should be taken into account in the preoperative consultation of patients with primary hyperparathyroidism.

Tumor microenvironment in thyroid cancer: Immune cells, patterns, and novel treatments.

The tumor immune microenvironment of thyroid cancer is the heterogeneous histological space in which tumor cells coexist with host cells. Published data from this review were identified by search and selection database of Pubmed, Elsevier, and Science Direct. Searching was made in two steps using different keywords. In thyroid pathology, the inflammatory response is very important, and might have a key role finding new diagnostic and therapeutic methods, particularly in thyroid cancer. Different immune cells may be more or less present in different types of thyroid cancer and may even have different functions, hence the importance of knowing their presence in different thyroid tumor pathologies. Cancer-related inflammation could be a useful target for new diagnostic and therapeutic strategies by analyzing peritumoral and intratumoral immune cells in different types of thyroid tumors. Moreover, novel strategies for thyroid cancer treatments, such as monoclonal antibodies targeting checkpoint inhibitors, are emerging as promising alternatives.

The Influence of Hyperparathyroidism Patient Profile on Quality of Life After Parathyroidectomy.

BACKGROUND: Improvements in quality of life (QoL) after parathyroidectomy in patients with primary hyperparathyroidism (PHPT) is discussed. It has not been analyzed whether these improvements can be influenced by a specific socio-personal or clinical patient profile. OBJECTIVES: to analyze QoL differences after parathyroidectomy and to determine a socio-personal and clinical profile that influences improvement after parathyroidectomy. METHODS: A longitudinal prospective cohort study in patients with PHPT. SF-36 and PHPQOL questionnaires were completed by the patients. A comparative preoperatory analysis was carried out, at three and twelve months after surgery. Student's t test was used for the correlations. The size of the effect was assessed using G*Power software. A multivariate analysis was performed to evaluate the socio-personal and clinical variables affecting the improvement in QoL after surgery. RESULTS: Forty-eight patients were analyzed. Three months after surgery an improvement was found in physical function, general health, vitality, social function, emotional role, mental health and in the patient's declared health assessment. One year after the intervention a general improvement was observed, with a greater effect on mental health and declared health evolution. Patients with bone pain presented with a higher probability of improvement after surgery. Patients with prior psychological disease had a lower associated probability of an improvement and high levels of PTH related to a greater probability of improvement after surgery. CONCLUSIONS: There is an improvement in the QoL of PHPT patients after parathyroidectomy. Patients with bone pain and high PTH levels prior to the parathyroidectomy present with a greater probability of having a greater improvement in QoL after surgery.

[Evaluation of the telematic consultation in endocrine surgery during confinement due to COVID-19]. / Evaluación de la consulta telemática en cirugía endocrina durante el confinamiento por COVID-19.

BACKGROUND: The coronavirus disease pandemic of 2019 (COVID-19) made necessary to remodel the hospital's clinical consultations. OBJECTIVE: To evaluate the telematic consultation in endocrine surgery, as well as to select the group of patients susceptible to perform a telematic consultation in the future according to this assessment. METHODS: The study population were patients who had a clinical consultation by telephone. The evaluation was made with the Net Promoter Score (NPS). STATISTICAL ANALYSIS: SPSS v.28, X2 test and a multivariate analysis. RESULTS: 55 patients were analyzed. The NPS was +16, 24 promoters (43.6%) and 15 detractors (27.2%). There were differences between those whose main reason for consultation was malignant pathology (OR 4.5; p = 0.033). The NPS between malignant vs. non-malignant pathology was -13 vs. +38 (p > 0.001). The evaluation of the telematic consultations for the future was: telephone (83% very well), video call (58%), videoconference (19%). CONCLUSIONS: The evaluation of the telephone consultation has been good, finding a better assessment among patients with non-malignant pathology. Its future implementation could be necessary, being the telephone the preferred way.

Malignant prediction in paragangliomas: analysis for clinical risk factors.

INTRODUCTION: Paragangliomas are infrequent neuroendocrine tumours whose only criterion for malignancy is presence of metastases; thus, all paragangliomas show malignant potential. Actually, different risk factors have been analyzed to predict metastases but they remain unclear. PURPOSE: To analyze clinical, histological, and genetic factors to predict the occurrence of metastasis. PATIENTS AND METHOD: A multicentre retrospective observational analysis was performed between January 1990 and July 2019. Patients diagnosed with paraganglioma were selected. Clinical, histological, and genetic features were analyzed for the prediction of malignancy. RESULTS: A total of 83 patients diagnosed with paraganglioma were included, of which nine (10.8%) had malignant paraganglioma. Tumour size was greater in malignant tumours than in benign (6 cm vs. 4 cm, respectively; p = 0.027). The most frequent location of malignancy was the thorax-abdomen-pelvis area observed in six cases (p = 0.024). No differences were observed in histological differentiation, age, symptoms, and catecholaminergic production. The most frequent genetic mutation was SDHD followed by SDHB but no differences were observed between benign and malignant tumours. In the univariate analysis for predictive factors for malignancy, location, tumour size, and histological differentiation showed statistical significance (p = 0.025, p = 0.014, and p = 0.046, respectively); however, they were not confirmed as predictive factors for malignancy in the multivariate analysis. CONCLUSION: In this study, no risk factors for malignancy have been established; therefore, we recommend follow-up of all patients diagnosed with paraganglioma.

Prediction of hypocalcemia after total thyroidectomy using indocyanine green angiography of parathyroid glands: A simple quantitative scoring system.

BACKGROUND: Hypocalcemia is one of the most common complications after total thyroidectomy. Recently, indocyanine green (ICG) angiography of the parathyroid glands (PGs) has been suggested as a reliable tool for predicting postoperative hypocalcemia. The aim of our study was to evaluate the performance of a simple quantitative score based on ICG angiography of the PGs (4-ICG score) for predicting postoperative hypocalcemia. METHODS: Thirty nine consecutive patients who underwent total thyroidectomy for multinodular goiter were included. For each patient, the 4-ICG score was calculated, adding the individual viability value of the four PGs. Discrimination and correlation analyses were performed. RESULTS: In 32/39 patients, the four PGs were identified. Patients with postoperative hypocalcemia (n = 6, 19%) had a lower 4-ICG score (2.5 [1.8-3.3] vs. 4.0 [3.0-6.0]; p = 0.003). The 4-ICG score showed good discrimination in terms of predicting postoperative hypocalcemia (AUC = 0.875 (0.710-0.965); p = 0.001) and a good correlation with postoperative parathyroid function. CONCLUSIONS: The 4-ICG score predicts postoperative hypocalcemia and correlates well with postoperative parathyroid function in patients undergoing total thyroidectomy for multinodular goiter.

Prophylactic thyroidectomy in multiple endocrine neoplasia 2 (MEN2) patients with the C634Y mutation: A long-term follow-up in a large single-center cohort.

BACKGROUND: Medullary thyroid carcinoma (MTC) is the main cause of death in multiple endocrine neoplasia 2A (MEN2A) patients. It is therefore important to treat this disease at an early stage. The mutation in codon 634 is considered to be associated with an aggressive clinical course, whereas the C634Y mutation may result in a more indolent course. Prophylactic thyroidectomy is performed before thyroid disease occurs. However, controversy surrounds this disease regarding levels of calcitonin (Ct) and age. In this context, few studies have investigated this mutation over a long period. OBJECTIVE: To analyze a large cohort of patients with the C634Y mutation who received prophylactic thyroidectomy. MATERIALS AND METHODS: In a group of 110 MEN2 patients, we analyzed those with the C634Y mutation who had received prophylactic thyroidectomy (absence of clinical and radiological thyroid disease) treated in a tertiary referral hospital between 1983 and 2016. MTC is related to age and Ct. Statistical analysis was performed using the χ2 test, partial correlations, and logistic regression. RESULTS: Fifty patients with a mean age of 12 ±â€¯9 years were analyzed; 56% of these had MTC (100% stage I). There was no case of hypoparathyroidism or permanent recurrent damage. MTC was associated mainly with age (OR 1.38). One 5-year-old patient presented with MTC. Mean follow-up time was 16 ±â€¯6 years, and no cases of recurrence were observed. CONCLUSIONS: Performing prophylactic thyroidectomy in patients with the C634Y mutation allows us to cure the disease without causing long-term complications. Our results support the notion that age <5 years should be a criterion for carrying out prophylactic thyroidectomy in these patients.

Primary hyperparathyroidism associated with MEN 1: Experience in 71 cases. / Hiperparatiroidismo primario asociado a neoplasia endocrina múltiple tipo 1 (MEN 1). Experiencia en 71 casos.

INTRODUCTION: Primary hyperparathyroidism (pHPT) in MEN 1 is characterized by multiglandular disease and early involvement of parathyroid glands at different times. Persistence and recurrence range from 25%-35%. The purpose was: a) to describe the experience and the treatment of patients with pHPT in MEN 1; b) to analyze the variables related with clinical presentation and recurrence. METHOD: A total of 97 patients with MEN 1 were diagnosed in a tertiary hospital. A retrospective analysis was made in patients with pHPT (n=71). STUDY VARIABLES: age at diagnosis, mutation, clinical presentation, laboratory tests, surgical technique, and recurrence of HPT. RESULTS: Mean age was 38 years, and 50 patients were asymptomatic. The surgical technique was: subtotal parathyroidectomy (n=55), resection of three glands (n=7), and resection of less glands (n=9). Transcervical thymectomy was performed in 53 patients. Mean follow-up was 102.9 months. There were 21 recurrences, There were correlations between age at diagnosis and serum calcium levels with the presence of symptoms (P<.0001). There were also correlations between recurrence and surgical technique (P<.03), non-association with thymectomy (P<.0001), and follow-up time (P<.03). CONCLUSION: Performing genetic and clinical screening allows us to make a diagnosis in the asymptomatic period and to provide early treatment for HPT in MEN 1. The recurrence rate is high, and follow-up time and the surgical technique used are risk factors for recurrence.