The development of patient-specific 3D anatomical models in minimally invasive parathyroidectomy.

Background: Surgery is the preferred treatment for primary hyperparathyroidism. Minimally invasive parathyroidectomy is only feasible with accurate preoperative localisation. Virtual 3D anatomical models can be constructed from patient-specific CT scans using segmentation software.

Ultrafast intraoperative parathyroid hormone monitoring system: prospective, multicentre, clinical validity study.

Background: Intraoperative parathyroid hormone (PTH) monitoring is a proven and reliable adjunct to parathyroid surgery, able to improve the outcomes and efficiency of the diagnostic and therapeutic pathway for patients with primary hyperparathyroidism. This study evaluated the innovative, compact, fully automated NBCL CONNECT Analyzer, which can measure whole-blood PTH in 5 min.

Methods: A prospective multicentre study was conducted in stages: results reviews, recommendations, and implementation of improvements to the mechanical design, components of cartridges, calibration, and sampling protocols.

Paediatric differentiated thyroid carcinoma: a UK National Clinical Practice Consensus Guideline.

This guideline is written as a reference document for clinicians presented with the challenge of managing paediatric patients with differentiated thyroid carcinoma up to the age of 19 years. Care of paediatric patients with differentiated thyroid carcinoma differs in key aspects from that of adults, and there have been several recent developments in the care pathways for this condition; this guideline has sought to identify and attend to these areas. It addresses the presentation, clinical assessment, diagnosis, management (both surgical and medical), genetic counselling, follow-up and prognosis of affected patients.

Treatment outcome with a selective RET tyrosine kinase inhibitor selpercatinib in children with multiple endocrine neoplasia type 2 and advanced medullary thyroid carcinoma.

Background: Medullary thyroid carcinoma (MTC) in the context of multiple endocrine neoplasia type 2 (MEN2) is caused by mutations in the RET proto-oncogene. Therefore, in children with MEN2 and advanced MTC, the RET tyrosine kinase (TK) pathway is a target for treatment with selpercatinib, a selective RET TK inhibitor.

Patients And Methods: A retrospective review of the clinical, genetic, biochemical (calcitonin and carcinoembryonic antigen [CEA]) and imaging data of six medically untreated children with MEN2 and recurrent and or progressive MTC.

Development of [F]AldoView as the First Highly Selective Aldosterone Synthase PET Tracer for Imaging of Primary Hyperaldosteronism.

The purpose of this study was to synthesize a fluorine-18 labeled, highly selective aldosterone synthase (CYP11B2) inhibitor, [F]AldoView, and to assess its potential for the detection of aldosterone-producing adenomas (APAs) with positron emission tomography in patients with primary hyperaldosteronism (PHA). Using dibenzothiophene sulfonium salt chemistry, [F]AldoView was obtained in high radiochemical yield in one step from [F]fluoride. In mice, the tracer showed a favorable pharmacokinetic profile, including rapid distribution and clearance.

Misleading GALLIUM-dotatate PET scan in a patient with a history of a phaeochromocytoma: Unsuspected uptake in papillary thyroid carcinoma metastases.

Scanning for somatostatin receptors using Ga-dotatate positron emission tomography with co-registration with computed tomography or magnetic resonance imaging is now in widespread use for the identification of neuroendocrine tumours, phaeochromocytomas, and paragangliomas and their metastases. We present a case where a patient with a phaeochromocytoma showed uptake in her neck considered diagnostic of a head-and-neck paraganglioma, which was subsequently confirmed to be a metastatic papillary thyroid carcinoma. We alert clinicians to such falsely-identified tumours using this extensively used imaging technique.

Late Recovery of Parathyroid Function after Total Thyroidectomy in Children and Adults: Is There a Difference?

Background: Parathyroid failure after total thyroidectomy is the commonest adverse event amongst both children and adults. The phenomenon of late recovery of parathyroid function, especially in young patients with persistent hypoparathyroidism, is not well understood. This study investigated differences in rates of parathyroid recovery in children and adults and factors influencing this.

Treating papillary and follicular thyroid cancer in children and young people: Single UK-center experience between 2003 and 2018.

Aim: Differentiated thyroid cancer (DTC) in children and adolescents is rare and data about its presentation and management are not well known. The aim of this study was to provide evidence of the current practice in the United Kingdom before the launch of the Rare National Paediatric Endocrine Tumours Guidelines (to be published in 2020).

Methods: Seventy-two children and adolescents with DTC (<18 years) who were treated at our institution between 2003 and 2018 were identified and their presentation, treatment and outcomes were reviewed.

The use of computed tomography as a first-line imaging modality in patients with primary hyperparathyroidism.

Background: The success of minimally invasive parathyroidectomy (MIP) relies on accurate localization of the abnormal parathyroid glands. Concordant findings on ultrasound (US) and Tc-scintigraphy (sestamibi) are currently considered the 'gold standard'. Computed tomography (CT) has also recently been used in preoperative planning.

Papillary thyroid microcarcinoma presenting as a metastasis to the brain.

The brain is a particularly rare site of metastasis from papillary microcarcinoma, with only few cases described in the literature. We present a case of 59-year-old man who presented with seizures and dysphasia due to left frontal lobe cystic mass, which was excised and turned out on histopathology to be of thyroid origin. Total thyroidectomy was performed and histology showed multifocal papillary microcarcinoma with the largest focus of 3mm with no other adverse features.

Influence of common clinical variables on intraoperative parathyroid hormone monitoring during surgery for primary hyperparathyroidism.

Background: Intraoperative monitoring of parathyroid hormone (IOPTH) is a reliable method of predicting the cure of primary hyperparathyroidism (PHPT). The aim of this study is to assess whether common clinical variables (CCV) frequently encountered in patients with PHPT may affect the magnitude of PTH drop or the likelihood of patients meeting the intraoperative cure criterion.

Design: Patients who were surgically cured from PHPT caused by single gland disease (SGD) and had full IOPTH protocol (4 measurements) were stratified according to age, gland weight, renal function, vitamin D status and severity of hypercalcemia.

The value of 18F-FDG PET-CT and 131I-norcholesterol scintigraphy in the characterization of high-risk adrenal masses.

Purpose: To assess the value of F-FDG PET-computed tomography (CT) and I-norcholesterol scintigraphy in noninvasive characterization of high-risk adrenal lesions using surgical pathology as the gold standard.

Methods: We retrospectively reviewed clinical cases referred to the adrenal multidisciplinary team in a tertiary centre over the last 6 years. Inclusion criteria were the presence of indeterminate adrenal lesions and performance of combined imaging with 2-deoxy-2-[fluorine-18] fluoro- D-glucose Positron emission tomography/ computed tomography and I-norcholesterol scans.

Children are at a high risk of hypocalcaemia and hypoparathyroidism after total thyroidectomy.

Purpose: Disruption of calcium homeostasis is the most common complication after total thyroidectomy in adults. We explored the incidence and risk factors of hypocalcaemia and hypoparathyroidism after total thyroidectomy in children (≤18 years of age).

Methods: One hundred six children underwent total thyroidectomy.

Natural history, treatment, and long-term follow up of patients with multiple endocrine neoplasia type 2B: an international, multicentre, retrospective study.

Background: Multiple endocrine neoplasia type 2B is a rare syndrome caused mainly by Met918Thr germline RET mutation, and characterised by medullary thyroid carcinoma, phaeochromocytoma, and extra-endocrine features. Data are scarce on the natural history of multiple endocrine neoplasia type 2B. We aimed to advance understanding of the phenotype and natural history of multiple endocrine neoplasia type 2B, to increase awareness and improve detection.

Impact of intraoperative parathyroid hormone monitoring on the management of patients with primary hyperparathyroidism.

Background/objective: Intraoperative parathyroid hormone (IOPTH) monitoring during surgery for primary hyperparathyroidism (PHPT) could improve cure rate and simplify current care pathways. This study assesses the performance of US, MIBI and IOPTH monitoring and their impact on outcomes and perioperative strategy.

Design: This is a retrospective study of a prospectively maintained database of patients who underwent parathyroidectomy guided by preoperative US, MIBI and IOPTH monitoring.

Prophylactic thyroidectomy in children with multiple endocrine neoplasia type 2.

Background: In patients with multiple endocrine neoplasia type 2 (MEN2) syndrome, genetic testing offers early diagnosis, stratifies the risk of developing medullary thyroid cancer (MTC) and informs the timing of thyroidectomy. The efficacy of treatment, which depends on timely and safe surgery, is not well established.

Methods: This was a retrospective review of diagnostic and clinicopathological outcomes of prophylactic thyroidectomy in children with MEN2 between 1995 and 2013 in the UK.

Dictating genomic destiny: Epigenetic regulation of pancreatic neuroendocrine tumours.

Pancreatic neuroendocrine tumours are a diverse group of neoplasms with an increasingly well-defined genomic basis. Despite this, much of what drives this disease is still unknown and epigenetic influences represent the next tier of gene, and hence disease modifiers that are of unquestionable importance. Moreover, they are of arguably more significance than the genes themselves given their malleable nature and potential to be exploited for not only diagnosis and prognosis, but also therapy.

Phaeochromocytomas and paragangliomas: A difference in disease behaviour and clinical outcomes.

Background: Phaeochromocytomas and paragangliomas arise from the same chromaffin cell, but evidence suggests they do not represent a single clinical entity. The aim of this study was to compare clinical presentations, outcomes of surgical and oncological treatments and survival in patients with phaeochromocytomas and paragangliomas.

Methods: A retrospective review was undertaken of all patients treated for these conditions at our centre between 1983 and 2012.

Aetiology, Diagnosis and Surgical Treatment of Primary Hyperparathyroidism in Children: New Trends.

Primary hyperparathyroidism (PHPT) in children is a rare disorder with sharp contrasts in its presentation and aetiology compared with the disease process in adults. This review outlines the current literature, which is limited to about 200 cases, with reference to the aetiology, clinical features, outcomes of investigations, and surgery in children affected by PHPT. Familial conditions account for almost half of all cases of PHPT in children, suggesting that routine genetic testing would be appropriate.