Purpose Pheochromocytomas/paragangliomas (PHEOs/PGLs) are rare in children with only a few SDHB mutation-related cases. Previous studies on children were conducted in small cohorts. This large set of pediatric patients provides robust data in the evaluation of clinical outcomes. Methods Sixty-four pediatric PHEO/PGL patients with SDHB germline mutations were included in the present study. The clinical presentation, disease course, and survival rate were evaluated. Results Thirty-eight males and 26 females were diagnosed with PHEO/PGL at a median age of 13 years. The majority of patients displayed norepinephrine hypersecretion and 73.44% initially presented with a solitary tumor. Metastases developed in 70% of patients at the median age of 16 years and were mostly diagnosed first 2 years and in years 12-18 post-diagnosis. The presence of metastases at the time of diagnosis had a strong negative impact on survival in males but not in females. The estimated 5-, 10-, and 20-year survival rates were 100%, 97.14%, and 77.71%, respectively. Conclusion The present report has highlighted several important aspects in the management of pediatric patients with SDHB mutations associated-PHEO/PGL. Initial diagnostic evaluation of SDHB mutation carriers should be started at age of 5-6 years with initial work-up focusing on abdominal region. Thorough follow-up is crucial first 2 years post-diagnosis and more frequent follow-ups are needed in years 10-20 post-diagnosis due to the increased risk of metastases. Although this age group developed metastasis as early as 5 years from diagnosis, we have shown that the overall 20-year prognosis and survival are good.
Objective. Micropapillary thyroid carcinoma (micro-PTC) has a good prognosis but a number of cases will present with aggressive behavior. This study aims to determine the clinical outcomes with surgical management and histopathologic characteristics of Filipino patients with micro-PTC at University of Santo Tomas Hospital.Methodology. 139 patients were diagnosed with micro-PTC from the year 2004-2011. Seventy five patients had complete data and were included in this retrospective study. Chi square test with Yates correction, T-test for tumor diameter, statistical means and percentages were used in data analysis.Results. A total of 1,689 thyroid surgeries were done between 2004 and 2011. There were 1,054 patients (62.4%) diagnosed with benign thyroid tumor(s) and 635 patients (37.6%) with well-differentiated thyroid carcinoma. Of these, 139 (22%) patients have micro-PTC. The prevalence rate of micro-PTC was 22%, with a female predominance (86.6%). The patients' ages ranged from 24-80 years old with a mean age of 47 years. Comparison of groups showed that having either incidental or non-incidental micro-PTC is independent of the clinical variables of the patient. Two (2.6%) patients initially presented with cranial and supraclavicular metastasis. This study had a low recurrence rate (5.3%) and a mortality rate of 1.3%.Conclusion. Male gender is the only significant variable for lymph node and distant metastasis. The patient's age, family history of cancer, number of foci, size and histological type of tumor have no prognostic value.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.