Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement.

Adult and paediatric patients with pathogenic variants in the gene encoding succinate dehydrogenase (SDH) subunit B (SDHB) often have locally aggressive, recurrent or metastatic phaeochromocytomas and paragangliomas (PPGLs). Furthermore, SDHB PPGLs have the highest rates of disease-specific morbidity and mortality compared with other hereditary PPGLs. PPGLs with SDHB pathogenic variants are often less differentiated and do not produce substantial amounts of catecholamines (in some patients, they produce only dopamine) compared with other hereditary subtypes, which enables these tumours to grow subclinically for a long time. In addition, SDHB pathogenic variants support tumour growth through high levels of the oncometabolite succinate and other mechanisms related to cancer initiation and progression. As a result, pseudohypoxia and upregulation of genes related to the hypoxia signalling pathway occur, promoting the growth, migration, invasiveness and metastasis of cancer cells. These factors, along with a high rate of metastasis, support early surgical intervention and total resection of PPGLs, regardless of the tumour size. The treatment of metastases is challenging and relies on either local or systemic therapies, or sometimes both. This Consensus statement should help guide clinicians in the diagnosis and management of patients with SDHB PPGLs.

Accuracy of merging scans of definitive fixed prosthodontic impressions to obtain single, accurate digitized master casts.

PURPOSE: Many elastomeric impressions sent to commercial laboratory dental technicians may include marginal defects. To fabricate accurate restorations, digital technology may be used to merge digital files of defective impressions into a single standard tessellation language (STL) file free of errors. This would save clinicians and patients time and may improve clinical care. The purpose of this study was to compare the accuracy of digital master casts reconstructed from merged STL files of defective impressions with the file of the original defect-free preparations. MATERIAL AND METHODS: Ivorine teeth on a dentoform were prepared to receive a posterior fixed dental prosthesis (FDP) with complete coverage preparations. An impression was made in a stock tray using polyvinyl siloxane (PVS) impression material and an extraoral scanner (E3, 3Shape, Denmark) was used to digitize the impression; this was the reference cast. Wax was used to create defects on the buccal and lingual margins of the preparations. Fifteen PVS impressions were made of the FDP preparations with defects in the mesial and distal margins; another set of 15 PVS impressions was made of FDP preparations with defects in the buccal and palatal margins for a total of 30 impressions. All impressions were digitized using the same extraoral scanner (E3, 3Shape, Denmark). Corresponding STL files were paired and merged, and a master cast was created by eliminating the defects using the scanned data. This master cast was compared to the reference cast using reverse engineering software (Geomagic, Morrisville, NC, USA). The results were expressed as average errors and standard deviations in the master casts relative to the reference cast. To account for the presence of positive and negative values in the data set, in terms of errors, the root mean square (RMS) value was calculated for each sample. RESULTS: The mean average error in the sample was -0.4 µm. The average upper limit of 95% confidence interval was +36.5 µm, while the average lower limit of 95% confidence interval was -37.3 µm. The mean RMS of the errors found was 18.9 µm. CONCLUSIONS: The results of this study indicated that merging digitized definitive impressions to correct marginal defects resulted in master casts with a high level of accuracy relative to the reference cast.

Shear bond strength characteristics on surface treatment modalities of CAD-CAM resin polymers.

PURPOSE: The purpose of this in vitro study was to analyze the shear bond strength of composite resin to a commercially available high-performance polymer material for fixed, screw-retained full arch restorations. MATERIAL AND METHODS: A total of 135 computer-aided design and computer-aided manufacturing, high-performance polymer (HPP) blocks were cut and obtained from discs (Trilor 95, Harvest Dental, Brea, CA). The samples were 10 mm × 10 mm × 10 mm. The specimen surfaces were grouped as untreated (Group A), 50 µm Al2O3 (Group B), 110 µm Al2O3 (Group C), Rocatec (3 M, St. Paul, MN) activated with silica-modified alumina oxide treatment (Group D); and trimmed coarsely with a carbide bur (Group E). Group A samples were used as controls. After surface treatments, the specimens were gently cleansed with oil-free steam and alcohol wipes. Surface conditioning was performed on all physically treated samples.  The manufacturer's recommendations were followed for bonding composite resin to the samples with light-cured Visio.link (Bredent, Chesterfield, UK). Cylinders were veneered with composite resins (diameter 5 mm, height 4 mm) and polymerized on the specimen surfaces through plastic tubes. Twenty-seven specimens were used for each testing group and aging tests were performed. The experimental samples were thermocycled.  Shear bond strength and scanning electron microscopic tests were performed. Means and standard deviations were calculated.  Statistical analysis was performed with post-hoc Tukey tests. RESULTS: Statistical analysis revealed that there was a significant difference between the groups (p<0.001). The highest shear bond strengths were achieved for the specimens bonded with Visio.link without physical surface treatments (270.47 MPa). The lowest bond strengths were found for specimen surfaces abraded with 110 µm Al2O3 (117.03 Mpa) CONCLUSIONS: The results of this laboratory study indicated that the specimens used with Visio.link as provided by the manufacturer had the highest shear bond strengths between the composite resin and high-performance polymer test specimens. Modifications of the high-performance polymer surfaces with carbide burs did not change bonding strengths with the composite materials.

Long-term Effectiveness of Ethanol Ablation in Controlling Neck Nodal Metastases in Childhood Papillary Thyroid Cancer.

Context: Childhood papillary thyroid carcinoma (CPTC), despite bilateral thyroidectomy, nodal dissection and radioiodine remnant ablation (RRA), recurs within neck nodal metastases (NNM) in 33% within 20 postoperative years. These NNM are usually treated with reoperation or further radioiodine. Ethanol ablation (EA) may be considered when numbers of NNM are limited. Objective: We studied the long-term results of EA in 14 patients presenting with CPTC during 1978 to 2013 and having EA for NNM during 2000 to 2018. Methods: Cytologic diagnoses of 20 NNM (median diameter 9 mm; median volume 203 mm3) were biopsy proven. EA was performed during 2 outpatient sessions under local anesthesia; total volume injected ranged from 0.1 to 2.8 cc (median 0.7). All were followed regularly by sonography and underwent volume recalculation and intranodal Doppler flow measurements. Successful ablation required reduction both in NNM volume and vascularity. Results: Post EA, patients were followed for 5 to 20 years (median 16). There were no complications, including postprocedure hoarseness. All 20 NNM shrank (mean by 87%) and Doppler flow eliminated in 19 of 20. After EA, 11 NNM (55%) disappeared on sonography; 8 of 11 before 20 months. Nine ablated foci were still identifiable after a median of 147 months; only one identifiable 5-mm NNM retained flow. Median serum Tg post EA was 0.6 ng/mL. Only one patient had an increase in Tg attributed to lung metastases. Conclusion: EA of NNM in CPTC is effective and safe. Our results suggest that for CPTC patients who do not wish further surgery and are uncomfortable with active surveillance of NNM, EA represents a minimally invasive outpatient management option.

Persistence and Recurrence of Hypercalcemia After Parathyroidectomy Over 5 Decades (1965-2010) in a Community-based Cohort.

BACKGROUND: There is limited long-term follow-up of patients undergoing parathyroidectomy. Recurrence is described as 4% to 10%. This study evaluated persistence and recurrence of hypercalcemia in primary hyperparathyroidism after parathyroidectomy. METHODS: Single-institution retrospective (1965-2010) population-based cohort from Olmsted County (MN) of patients undergoing surgery for primary hyperparathyroidism. Patients' demographic data, preoperative and postoperative laboratory values, clinical characteristics, surgical treatment, and follow-up were noted. RESULTS: A total of 345 patients were identified, 75.7% female, and median age 58.4 years [interquartile range (IQR): 17.6]. In all, 68% of patients were asymptomatic and the most common symptoms were musculoskeletal complaints (28.4%) and nephrolithiasis (25.6%). Preoperative median serum calcium was 11 mg/dL (IQR: 10.8-11.4 mg/dL), and median parathyroid hormone was 90 pg/mL (IQR: 61-169 pg/dL). Bilateral cervical exploration was performed in 38% and single gland resection in 79% of cases. Median postoperative serum calcium was 9.2 mg/dL (IQR: 5.5-11.3). Nine percent of patients presented persistence of hypercalcemia, and recurrence was found in 14% of patients. Highest postoperative median serum calcium was 10 mg/dL (IQR: 6-12.4), and median number of postoperative calcium measurements was 10 (IQR: 0-102). Postoperative hypercalcemia was identified in 37% of patient. Fifty-three percent were attributed to secondary causes, most commonly medications, 22%. Three percent of patients required treatment for postoperative hypercalcemia. Median time to recurrence and death were 12.2 and 16.7 years, respectively. CONCLUSION: Recurrent hypercalcemia after successful parathyroidectomy is higher than previously reported. Most cases are transient and often associated to other factors with only the minority requiring treatment. Long-term follow-up of serum calcium should be considered in patients after successful parathyroidectomy.

Comparison Between Sporadic and Multiple Endocrine Neoplasia Type 1-Associated Insulinoma.

BACKGROUND: The differences between sporadic and multiple endocrine neoplasia type 1 (MEN-1)-associated insulinoma are not well described. Herein, we compared demographics, neoplasm characteristics, presentation, and survival in patients with sporadic vs MEN-1 insulinomas including benign and malignant disease. STUDY DESIGN: A retrospective study identified insulinoma patients. MEN-1 was defined based on genetic testing or clinically in patients with 2 or more primary MEN-1 tumor types. RESULTS: A total of 311 patients were identified: 84% benign and 16% malignant. The incidence of malignancy was similar (18% vs 16%, MEN-1 vs sporadic, p = 0.76). Within malignant patients, the median (interquartile range) age was 33 (25 ,44) years in MEN-1 vs 54 (41, 70) years in sporadic insulinoma (p = 0.04). There was no difference in sex or tumor size between MEN-1 and sporadic malignant insulinoma (p > 0.05). Of the 260 patients with benign insulinoma, 7% had MEN-1 syndrome. MEN-1 patients presented with insulinoma at a younger age: median (interquartile range) age was 38 (24, 49) years vs 52 (43, 65) years (p < 0.01). Resection of benign insulinoma was performed in 78% of the MEN-1 and 94% of the sporadic group (p = 0.03). Resected benign tumors were larger in the MEN-1 group: 2.0 (1.65, 2.45) cm vs 1.5 (1.2, 2.0) cm, respectively (p = 0.03). Concurrent insulinomas were more common in MEN-1 (17% vs 2%; p < 0.01). CONCLUSIONS: MEN-1 patients present with insulinoma at younger age and have larger benign pancreatic lesions at the time of resection compared with sporadic neoplasms. Younger patients and those with multifocal pancreatic neuroendocrine tumor in the setting of endogenous hyperinsulinism should be evaluated for MEN-1.

Radioiodine remnant ablation in stage I adult papillary thyroid carcinoma: does it improve postoperative outcome?

Objective: To determine whether radioiodine remnant ablation (RRA) reduces cause-specific mortality (CSM) or tumor recurrence rates (TRR) after potentially curative bilateral thyroidectomy (BT) in low-risk adult papillary thyroid carcinoma (APTC) patients, we compared postoperative outcomes in 1836 pTNM stage I APTC patients having BT alone with 832 having BT+RRA during two consecutive 25-year periods. Methods: The THEN cohort (consecutively managed during 1966-1990) comprised 809 patients (36% having BT+RRA) and the NOW cohort (1991-2015) comprised 1859 patients (29% BT+RRA). Analyses of differences in occurrence rates between BT alone and BT+RRA patients were performed with SAS software. Results: During 1966-1990, when RRA rates rose ten-fold, 20-year CSM after BT alone was 0.6% and after BT+RRA was 1.2% (P = 0.66); during 1991-2015, when RRA rates progressively fell, no PTC deaths occurred in 1859 patients. In the THEN cohort, RRA did not significantly improve TRR at local, regional, or distant sites (P > 0.1), when compared to BT alone. RRA in NOW cohort was administered to 49% of node-positive (pN1) patients and 17% of node-negative (pN0/NX) patients (P < 0.0001); TRR therefore, were examined separately for pN0/NX and pN1 patients. In 1157 pN0/NX cases, 20-year locoregional TRR were 3.1% after BT and were higher (P = 0.049) at 8.6% after BT+RRA. In four pN1 groups, stratified by metastatic nodal burden, RRA did not significantly reduce the locoregional TRR observed after BT with curative intent (P > 0.5). Conclusions: In a 5-decade experience, RRA administered postoperatively to stage I APTC patients did not reduce either CSM or TRR and should probably not be indicated when such patients undergo potentially curative BT.

Quantitative matching of forensic evidence fragments utilizing 3D microscopy analysis of fracture surface replicas.

Silicone casts are widely used by practitioners in the comparative analysis of forensic items. Fractured surfaces carry unique details that can provide accurate quantitative comparisons of forensic fragments. In this study, a statistical analysis comparison protocol was applied to a set of 3D topological images of fractured surface pairs and their replicas to provide confidence in the quantitative statistical comparison between fractured items and their silicone cast replicas. A set of 10 fractured stainless steel samples were fractured from the same metal rod under controlled conditions and were replicated using a standard forensic casting technique. Six 3D topological maps with 50% overlap were acquired for each fractured pair. Spectral analyses were utilized to identify the correlation between topological surface features at different length scales of the surface topology. We selected two frequency bands over the critical wavelength (greater than two-grain diameters) for statistical comparison. Our statistical model utilized a matrix-variate t-distribution that accounts for overlap between images to model match and non-match population densities. A decision rule identified the probability of matched and unmatched pairs of surfaces. The proposed methodology correctly classified the fractured steel surfaces and their replicas with a posterior probability of match exceeding 99.96%. Moreover, the replication technique shows potential in accurately replicating fracture surface topological details with a wavelength greater than 20 µm, which far exceeds the feature comparison range on most metallic alloy surfaces. Our framework establishes the basis and limits for forensic comparison of fractured articles and their replicas while providing a reliable fracture mechanics-based quantitative statistical forensic comparison.

Edge chipping of translucent zirconia.

STATEMENT OF PROBLEM: More translucent dental zirconias have been developed by incorporating the cubic phase and reducing the tetragonal phase content that undergoes transformation toughening, leading to reduced mechanical properties. Whether the clinically relevant mechanical property of the edge chipping toughness of the material is also reduced is unclear. PURPOSE: The purpose of this in vitro study was to evaluate the edge chipping toughness and translucency of translucent zirconia, 3mol% yttria-stabilized tetragonal zirconia polycrystal (3Y-TZP), and lithium disilicate. MATERIAL AND METHODS: Two translucent zirconia products, Katana and Lava Esthetic; one 3Y-TZP, Lava Plus; and one lithium disilicate, IPS e.max Press were prepared and tested for phase composition via X-ray diffraction (XRD) (n=3), translucency via a spectrophotometer (n=20), and edge chipping via a universal testing machine with a custom-machined specimen holder and diamond indenter (n=20). The 3Y-TZP and lithium disilicate served as the optimal control materials for edge chipping and translucency, respectively. Translucency was compared with 1-way ANOVA and edge toughness with ANCOVA (α=.05). RESULTS: The XRD showed the 3Y-TZP to be almost completely tetragonal phase compared with the 2 translucent zirconia products that were predominantly cubic. Katana UTML and IPS e.max Press had a statistically similar (P>.05) translucency that was significantly (P<.05) greater than that of Lava Esthetic and Lava Plus. The edge toughness of Katana UTML was 304 N/mm, IPS e.max Press was 354 N/mm, Lava Esthetic was 394 N/mm, and Lava Plus was 717 N/mm, with significance rankings of Katana UTM

Routine Use of Preoperative Neck Ultrasound in Primary Hyperparathyroidism Identifies Coexisting Thyroid Disease and Improves Parathyroid Localization.

BACKGROUND: In Jan 2018, we began routinely obtaining neck ultrasound (US) with 123I/99Tc-sestamibi (MIBI) for parathyroid gland localization and to identify thyroid pathology in the setting of primary hyperparathyroidism (1HPT). The aim of this study is to assess if routine neck US is a useful adjunct to 123I/99Tc-MIBI in 1HPT. METHODS: Patients undergoing surgery for 1HPT with both 123I/99Tc-MIBI and US at our institution after implementation of routine US were reviewed. Biopsy and surgical management of thyroid pathology was evaluated. 123I/99Tc-MIBI and US results were compared to intraoperative findings to determine sensitivity and positive predictive value (PPV) for parathyroid localization. RESULTS: From January 2018 to September 2019, there were 423 patients (mean, 61 years) that met inclusion criteria (80% women). Thyroid nodules were found on US in 57%, mean size 1.3 + 0.8 cm. Fine needle aspiration (FNA) was performed in 87 patients with nodules (36%). 35 patients (8.5%) required total or partial thyroidectomy for diagnoses/treatment. Papillary thyroid cancer (PTC) was found in 3.5% of the cohort with micro-PTC 53% and PTC 1-2 cm 40%. A successful parathyroid operation for 1HPT was achieved in 98.6% of patients. Positive predictive value for localization of abnormal parathyroid glands was 97% when US and 123I/99Tc-MIBI had concordant findings. DISCUSSION: Routine use of US in 1HPT commonly identifies nodules that are benign or low-risk PTC. Ultrasound is less sensitive for parathyroid localization but when used with 123I/99Tc-MIBI, concordant imaging has a high PPV.

Comparison of two arbitrary cast transfer systems with a kinematic facebow for mounting a maxillary cast on a semiadjustable articulator.

STATEMENT OF PROBLEM: The Kois Dento-Facial Analyzer (KDFA) is used by clinicians to mount maxillary casts and to evaluate and treat patients. Limited information is available for understanding whether the KDFA should be considered as an alternative to an arbitrary facebow. PURPOSE: The purpose of this clinical study was to evaluate and compare maxillary casts mounted by using the KDFA with casts mounted by using the Panadent Pana-Mount Facebow (PMF) and a kinematic axis (KA) facebow. MATERIAL AND METHODS: Fifteen participants were enrolled in the study. Three maxillary impressions were made of each study participant. One cast from each study participant was mounted on an articulator by means of the KDFA, PMF, and KA. A standardized photograph of each mounting was made, and the condylar center-incisor distance and the occlusal and incisal plane angles were measured. A randomized complete block design analysis of variance (RCBD) (α=.05) and post hoc tests (Tukey-Kramer HSD) were used to evaluate the occlusal and incisal plane angles and the condylar center-incisor distance. RESULTS: Compared with the occlusal plane angle (OPA), the KDFA mounted the maxillary cast at an angle that was statistically lower than those of PMF and KA (P<.001). The KDFA and the PMF condylar center-incisor distances were both significantly greater than that of KA (P=.01). No differences were found between the incisal plane angle (IPA) on maxillary casts mounted with the KDFA, KA, or PMF (P=.16). CONCLUSIONS: The KDFA and PMF mounted the maxillary casts in a position that was farther from the axis when compared with the KA mounted casts. The KDFA resulted in a lower articulator OPA compared with both PMF and KA. No difference was found between the IPAs of the KDFA, PMF, and KA.

Medullary thyroid cancer: What is the optimal management of the lateral neck in a node negative patient at index operation?

BACKGROUND: Medullary thyroid cancer is a neuroendocrine malignancy that can occur sporadically or as the result of genomic rearranged during transfection mutations. Medullary thyroid cancer has a higher rate of metastasis than well-differentiated thyroid cancer. Lateral neck dissection is often performed, and its prophylactic use is controversial. METHODS: Single-center, retrospective review (2000-2017) of patients undergoing primary surgical treatment for medullary thyroid cancer who had negative lateral neck imaging preoperatively. Demographics, genetic associations, clinical, and imaging findings were analyzed. Locoregional recurrence, overall recurrence, and overall survival were examined. RESULTS: A total of 110 patients were identified, of which 18 underwent prophylactic lateral neck dissection and 92 did not. Age, sex distribution, preoperative calcitonin levels, and follow-up were similar among groups. Overall recurrence was 20% for no prophylactic lateral neck dissection and 39% for prophylactic lateral neck dissection (P = .46). Most recurrences were locoregional recurrence, 7.6% for no prophylactic lateral neck dissection versus 22% for prophylactic lateral neck dissection (P = .08), half of it being to the lateral neck in both groups. A total of 7 patients from the no prophylactic lateral neck dissection group required treatment for recurrences versus 4 patients in prophylactic lateral neck dissection group (P = .57). Overall survival at 5 years was similar, 43% the no prophylactic lateral neck dissection group and 31% for prophylactic lateral neck dissection group (P = .52). CONCLUSION: Lateral neck dissection has no effect in decreasing locoregional or overall recurrences in medullary thyroid cancer and has no effect in overall survival when performed prophylactically at index surgical intervention.

Consensus Statement by the American Association of Clinical Endocrinology (AACE) and the American Head and Neck Society Endocrine Surgery Section (AHNS) on Pediatric Benign and Malignant Thyroid Surgery.

OBJECTIVES: To provide a clinical disease state review of recent relevant literature and to generate expert consensus statements regarding the breadth of pediatric thyroid cancer diagnosis and care, with an emphasis on thyroid surgery. To generate expert statements to educate pediatric practitioners on the state-of-the-art practices and the value of surgical experience in the management of this unusual and challenging disease in children. METHODS: A literature search was conducted and statements were constructed and subjected to a modified Delphi process to measure the consensus of the expert author panel. The wording of statements, voting tabulation, and statistical analysis were overseen by a Delphi expert (J.J.S.). RESULTS: Twenty-five consensus statements were created and subjected to a modified Delphi analysis to measure the strength of consensus of the expert author panel. All statements reached a level of consensus, and the majority of statements reached the highest level of consensus. CONCLUSION: Pediatric thyroid cancer has many unique nuances, such as bulky cervical adenopathy on presentation, an increased incidence of diffuse sclerosing variant, and a longer potential lifespan to endure potential complications from treatment. Complications can be a burden to parents and patients alike. We suggest that optimal outcomes and decreased morbidity will come from the use of advanced imaging, diagnostic testing, and neural monitoring of patients treated at high-volume centers by high-volume surgeons.

Inability of Radioiodine Remnant Ablation to Improve Postoperative Outcome in Adult Patients with Low-Risk Papillary Thyroid Carcinoma.

OBJECTIVE: To determine whether radioiodine remnant ablation (RRA) reduces cause-specific mortality (CSM) or tumor recurrence (TR) rate after bilateral lobar resection (BLR). PATIENTS AND METHODS: There were 2952 low-risk adult papillary thyroid cancer (LRAPTC) patients (with MACIS scores <6) who underwent potentially curative BLR during 1955-2014. During 1955-1974, 1975-1994, and 1995-2014, RRA was administered in 3%, 49%, and 28%. Statistical analyses were performed using SAS software. RESULTS: During 1955-1974, the 20-year CSM and TR rates after BLR alone were 1.0% and 6.8%; rates after BLR+RRA were 0% (P=.63) and 5.9% (P=.82). During 1975-1994, post-BLR 20-year rates for CSM and TR were 0.3% and 7.5%; after BLR+RRA, rates were higher at 0.9% (P=.31) and 12.8% (P=.01). When TR rates were examined separately for 448 node-negative and 317 node-positive patients, differences were nonsignificant. In 1995-2014, post-BLR 20-year CSM and TR rates were 0% and 9.2%; rates after BLR+RRA were higher at 1.4% (P=.19) and 21.0% (P<.001). In 890 pN0 cases, 15-year locoregional recurrence rates were 3.4% after BLR and 3.7% after BLR+RRA (P=.99). In 740 pN1 patients, 15-year locoregional recurrence rates were 10% higher after BLR+RRA compared with BLR alone (P=.01). However, this difference became nonsignificant when stratified by numbers of metastatic nodes. CONCLUSION: RRA administered to LRAPTC patients during 1955-2014 did not reduce either the CSM or TR rate. We would therefore not recommend RRA in LRAPTC patients undergoing BLR with curative intent.

Presentation and outcomes of adrenal ganglioneuromas: A cohort study and a systematic review of literature.

OBJECTIVE: To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs). DESIGN: Single-centre retrospective cohort study (1 January 1995 to 31 December 2019) and systematic review of literature (1 January 1980 to 19 November 2019). PATIENTS: Diagnosed with histologically confirmed AGN. MEASUREMENTS: Baseline clinical, imaging and biochemical characteristics, recurrence rates and mortality. Subgroup analysis was performed on tumours with histologic elements of ganglioneuroma and pheochromocytoma (ie composite tumours). RESULTS: The cohort study included 45 patients with AGN, 20 (44%) of which had composite tumours. Compared to pure AGN, patients with composite tumour were older (median age, 62.5 vs. 35 years, p < .001), had smaller tumours (median size, 3.9 vs. 5.7 cm, p = .016) and were discovered incidentally less frequently (65% vs. 84%, p = .009). No recurrences or ganglioneuroma-specific mortality occurred during follow-up (range, 0-266 months). The systematic review included 14 additional studies and 421 patients. The mean age of diagnosis was 39 years, and 47% were women. AGNs were discovered incidentally in 72% of patients, were predominantly unilateral (99%) and had a mean diameter of 5.8 cm and an unenhanced computed tomography (CT) attenuation of -118 to 49 Hounsfield units (HU). On imaging, 69% of AGNs were homogenous, 41% demonstrated calcifications, and 40% were lobulated. CONCLUSIONS: AGNs are rare benign tumours that present with variable imaging features including large size, unenhanced CT attenuation >20 HU, calcifications and lobulated shape. Imaging characteristics can assist in establishing a diagnosis and avoiding an unnecessary adrenalectomy. The association of pheochromocytomas with AGNs is frequent. Diagnosis should include biochemical testing.

Consensus statement by the American Association of Clinical Endocrinology (AACE) and the American Head and Neck Society Endocrine Surgery Section (AHNS-ES) on Pediatric Benign and Malignant Thyroid Surgery.

OBJECTIVES: To provide a clinical disease state review of recent relevant literature and to generate expert consensus statements regarding the breadth of pediatric thyroid cancer diagnosis and care, with an emphasis on thyroid surgery. To generate expert statements to educate pediatric practitioners on the state-of-the-art practices and the value of surgical experience in the management of this unusual and challenging disease in children. METHODS: A literature search was conducted and statements were constructed and subjected to a modified Delphi process to measure the consensus of the expert author panel. The wording of statements, voting tabulation, and statistical analysis were overseen by a Delphi expert (J.J.S.). RESULTS: Twenty-five consensus statements were created and subjected to a modified Delphi analysis to measure the strength of consensus of the expert author panel. All statements reached a level of consensus, and the majority of statements reached the highest level of consensus. CONCLUSION: Pediatric thyroid cancer has many unique nuances, such as bulky cervical adenopathy on presentation, an increased incidence of diffuse sclerosing variant, and a longer potential lifespan to endure potential complications from treatment. Complications can be a burden to parents and patients alike. We suggest that optimal outcomes and decreased morbidity will come from the use of advanced imaging, diagnostic testing, and neural monitoring of patients treated at high-volume centers by high-volume surgeons.

Positive Lymph Nodes in Adrenocortical Carcinoma: What Does It Mean?

BACKGROUND: The role of lymphadenectomy in adrenocortical carcinoma resection is controversial. Therefore, we conducted a population-based study to assess the association between positive lymph nodes (LN) and survival. METHODS: The Surveillance, Epidemiology, and End Results set of cancer registries were utilized. The associations between positive lymph nodes and tumor size, grade and laterality were assessed. Cancer specific survival (CSS) trends and factors affecting survival were analyzed. RESULTS: A total of 2170 adult patients were identified; 60% underwent resection. Among those resected, LN were examined in 23% and were positive in 25% of patients with LN examined. Patients with positive LN tended to have smaller tumors compared to those with negative LN (12 ± 5 vs 15 ± 11 cm, p = 0.02). The rate of positive LN was higher in right ACC, p = 0.03. Median overall CSS was 21 months, with significant differences between resection (42 months) and no resection (4 months), p < 0.01. Median CSS did not change over time when comparing ACC patients who underwent surgery before 2000, 2000-2009, and 2010-2016. On multivariable analysis including resection group, advanced age, grades III and IV, regional and distant stage, in addition to positive LN were associated with worse survival, p < 0.05. CONCLUSION: Lymphadenectomy is infrequently performed during ACC resection, and when performed, regional LN involvement tends to be associated with worse survival. Neoplasm size and grade were not associated with LN involvement and therefore, do not inform lymphadenectomy need. Further studies are needed to assess the indications for, and value of lymphadenectomy in ACC.