Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries.

Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF. Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still's disease patients and Behçet's disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression. Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet's disease patients and 497 Still's disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p=0.10) in FMF compared to Still's disease and 0.60 (95% CI. 0.29-1.28, p=0.18) in FMF compared to Behçet's disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (ß1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (ß1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (ß1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (ß1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (ß1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (ß1 = 2.089, 95% CI. 0.7-3.5, p=0.002). Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.

Optimal Monitoring Technology for Pediatric Thyroidectomy.

This retrospective study aimed to describe, firstly, characteristics and outcomes of the intraoperative neural monitoring technology in the pediatric population, and secondarily the recurrent laryngeal nerve complication rate. Thirty-seven patients (age <18 years) operated on from 2015 to 2021 by conventional open thyroid surgery were included. Twenty-four (64.9%) total thyroidectomies and 13 (35.1%) lobectomies were performed. Seven central and six lateral lymph node dissections completed 13 bilateral procedures. Histology showed malignancy in 45.9% of the cases. The differences between the electromyographic profiles of endotracheal tubes or electrodes for continuous monitoring were not statistically significant. In our series of young patients, both adhesive (even in 4- or 5-year-olds) and embedded endotracheal tubes were used, while in patients 3 years old or younger, the use of a more invasive detection method with transcartilage placement recording electrodes was required. Overall, out of 61 total at-risk nerves, 5 (8.2%) recurrent laryngeal nerves were injured with consequent intraoperative loss of the signal; however, all these lesions were transient, restoring their normal functionality within 4 months from surgical procedure. To our knowledge, this is the first study of intraoperative neural monitoring management in a cohort of Italian pediatric patients.

Considerations for Balance Between Fundamental Treatment and Improvement of Quality of Life of Pediatric Thyroid Cancer Patient: Comparative Analysis With Adult Using Propensity Score Matching.

Background: Thyroid cancer is very rarely observed in children and adolescents, some reports have shown that the long-term outcome of treatment is better than that of adult patients, despite many treatment failures or a high risk of recurrence. This study considers whether it is appropriate to treat pediatric thyroid cancer patients aggressively, as per the ATA guidelines, based on the balance between the fundamental treatment of thyroid cancer and the improvement of the long-term quality of life of pediatric patients. Methods: A total of 1,950 patients were recruited, including 83 pediatric and 1,867 adult patients, who were diagnosed with thyroid cancer and underwent surgical treatment at one of our medical center hospitals from March 2000 to January 2020. Results: Sixty-nine pairs of pediatric and adult patients were matched in a ratio of 1:2 through propensity score matching. When compared through propensity score matching, there was no significant difference in prognosis such as recurrence rate in children and adults at the same stage. Conclusion: This study showed that the prognosis of both pediatric and adult patients who underwent a total thyroidectomy and lobectomy was not significantly different. If more pediatric patients can be considered for the less-aggressive lobectomy than a total thyroidectomy through various preoperative examinations and meticulous pre-diagnosis, it may be possible to properly determine the balance between improving long-term quality of life while providing fundamental cancer treatment.