Ethanol ablation of papillary thyroid carcinoma nodal metastases: Long-term outcomes.

BACKGROUND: Percutaneous ethanol ablation has emerged as a treatment for recurrent papillary thyroid carcinoma in the lateral neck after compartment-oriented therapeutic lymphadenectomy. However, the safety and utility of percutaneous ethanol ablation as a primary treatment modality for lateral neck metastases remains undefined. We aimed to investigate long-term outcomes of percutaneous ethanol ablation of lateral neck papillary thyroid carcinoma recurrence both with and without prior lymphadenectomy. METHODS: We conducted a retrospective study of patients with lateral neck papillary thyroid carcinoma treated with percutaneous ethanol ablation from 2013 to 2018. Patient characteristics, disease volume, morbidity, and recurrence (development of new lymphadenopathy within a percutaneous ethanol ablation-treated nodal compartment) were assessed. RESULTS: We identified 117 patients who underwent percutaneous ethanol ablation for papillary thyroid carcinoma lateral neck metastases-67 (57%) had a prior lateral neck dissection. Median follow-up after percutaneous ethanol ablation was 5.5 years (interquartile range 3.1-7.5). On average, 1.4 lymph nodes (range: 1-6) were treated. Three patients (3%) developed transient nerve-related complications after percutaneous ethanol ablation. Of 15 patients who underwent lateral neck dissection after percutaneous ethanol ablation (including patients undergoing repeat lateral neck dissection), dissection was "difficult" in 8 (53%) (7 of whom had previously undergone lateral neck dissection), and 4 (27%) developed complications (transient nerve dysfunction = 3, lymphatic leak = 1). Thirty-three patients (28%) developed recurrent papillary thyroid carcinoma. No difference in recurrence was seen between patients who did or did not undergo pre-percutaneous ethanol ablation lateral neck dissection (no pre-percutaneous ethanol ablation lateral neck dissection: 24%, pre-percutaneous ethanol ablation lateral neck dissection, 31%; hazard ratio = 1.27, 95% confidence interval 0.62-2.58; P = .514). CONCLUSION: Percutaneous ethanol ablation may be a safe primary treatment modality for papillary thyroid carcinoma lateral neck nodal recurrence in selected patients with low-volume nodal disease.

Incidence of venous thromboembolism following adrenalectomy: A CESQIP analysis.

BACKGROUND: We aim to evaluate the incidence of venous thromboembolism (VTE) following adrenalectomy. METHODS: A retrospective analysis of the Collaborative Endocrine Surgery Quality Improvement Program was performed to assess incidence for VTE, including pulmonary embolism or deep vein thrombosis, in adults undergoing adrenalectomy (2014-2022). RESULTS: 2567 patients undergoing adrenalectomy were included. Surgical approach was 10% open and 90% minimally invasive. Pathology was 13% malignant and 87% benign; 19% had hypercortisolism. VTE developed in 0.27% at a median of 8 days from surgery. The incidence was higher in primary adrenal malignancy compared to benign or metastases to the adrenals, p â€‹< â€‹0.01. VTE was associated with longer hospital stay, longer operative time, readmission, and mortality. VTE rates were similar for hypercortisolism vs no hypercortisolism and between patients with clinical vs subclinical hypercortisolism. CONCLUSION: Although VTE following adrenalectomy is rare, it is more common in cases of primary adrenal malignancy, those with longer operations, or those requiring prolonged hospitalization.

Primary hyperparathyroidism in patients with multiple endocrine neoplasia type 1: Impact of genotype and surgical approach on long-term postoperative outcomes.

BACKGROUND: Protein-truncating germline pathogenic variants in the N- and C-terminal exons (2, 9, and 10) of the MEN1 gene may be associated with aggressive pancreatic neuroendocrine tumors. However, the impact of these variants on parathyroid disease is poorly understood. We sought to investigate the effects of genotype and surgical approach on clinical phenotype and postoperative outcomes in patients with multiple endocrine neoplasia type 1 (MEN1)-related primary hyperparathyroidism. METHODS: We identified patients with MEN1 evaluated at our institution from 1985 to 2020 and stratified them by genotype, (truncating variants in exons 2, 9, or 10, or other variants), and index surgical approach, (less-than-subtotal parathyroidectomy [

The effect of hormonal secretion on survival in adrenocortical carcinoma: A multi-center study.

BACKGROUND: Current evidence suggests that cortisol secreting adrenocortical carcinoma has worse prognosis compared to non-secreting adrenocortical carcinoma. However, the effect of other secretory subtypes is unknown. METHODS: This multicenter study within the American-Australian-Asian Adrenal Alliance included adults with adrenocortical carcinoma (1997-2020). We compared overall survival and disease-free survival among cortisol secreting, mixed cortisol/androgen secreting, androgen secreting, and non-secreting adrenocortical carcinoma. RESULTS: Of the 807 patients (mean age 50), 719 included in the secretory subtype analysis: 24.5% were cortisol secreting, 13% androgen secreting, 28% mixed cortisol/androgen, 32.5% non-secreting, and 2% were mineralocorticoid secreting. Median overall survival and disease-free survival for the entire cohort were 60 and 9 months, respectively. Median overall survival was 36 months for cortisol, 30 for mixed, 60 for androgen secreting, and 115 for non-secreting adrenocortical carcinoma, P < .01. Median disease-free survival was 7 months for cortisol, 8 for mixed, 10 for androgen, and 12 for non-secreting adrenocortical carcinoma, P = .06. On multivariable analysis of age, sex, Ki67%, secretory subtype, stage, resection, and adjuvant therapy, predictors of worse overall survival were older age, higher Ki67%, stage IV, mixed secreting, R1, and no adjuvant therapy, P < .05. On subgroup analysis of R0 resection, predictors of worse overall survival included older age and higher Ki67%. Ki67% ≥40, stage III and cortisol secretion were associated with worse disease-free survival. CONCLUSION: Mixed cortisol/androgen secreting adrenocortical carcinoma was associated with worse overall survival, while cortisol or androgen secreting alone were not. Notably, among patients after R0 resection, secretory subtype did not affect overall survival. Cortisol secreting adrenocortical carcinoma demonstrated worse disease-free survival. Ki67% remained a strong predictor of worse overall survival and disease-free survival independent of stage.

Disparities in Parathyroidectomy: Who Receives Appropriate Treatment for Primary Hyperparathyroidism?

INTRODUCTION: Parathyroidectomy is underperformed despite clear benefits in primary hyperparathyroidism (PHPT). We evaluated disparities in receipt of parathyroidectomy following PHPT diagnosis to explore barriers to care. METHODS: Adults diagnosed with PHPT 2013-2018 at a health system were identified. Recommended indications for parathyroidectomy include age ≤50 y, calcium >11 mg/dL, or the presence of nephrolithiasis, hypercalciuria, nephrocalcinosis, decreased glomerular filtration rate, osteopenia, osteoporosis, or pathological fracture 1 y prior to diagnosis. Kaplan-Meier analysis assessed rates of parathyroidectomy within 12 mo following diagnosis as well as median time to parathyroidectomy, and multivariable Cox proportional hazards analyses assessed factors associated with undergoing parathyroidectomy. RESULTS: Of 2409 patients, 75% were females, 12% aged ≤50 y, and 92% non-Hispanic White, while 52% had Medicaid/Medicare, 36% were commercial/self-pay or uninsured, and 12% unknown. Parathyroidectomy was performed within 1 y in 50% of patients. Within the 68% that met recommendations, parathyroidectomy was performed within 1 y in 54%; median time from diagnosis to surgery was shorter for males, patients aged ≤50 y, commercial/self-pay/no insurance patients (versus Medicaid/Medicare), and those with fewer comorbidities, P < 0.05. Multivariable analysis demonstrated non-Hispanic White patients and those with commercial/self-pay/uninsured were more likely to undergo parathyroidectomy after adjusting for comorbidity, age, and facility site. Among those strongly indicated, patients not on Medicare/Medicaid and aged ≤50 y were more likely to undergo parathyroidectomy after adjusting for race, comorbidity, and facility site. CONCLUSIONS: Disparities in parathyroidectomy for PHPT were observed. Insurance type was associated with undergoing parathyroidectomy; patients on governmental insurance were less likely to undergo surgery and waited longer for surgery despite strong indications. Barriers to referral and access to surgery should be investigated and addressed to optimize all patients' access to care.

Intraoperative Parathyroid Hormone Monitoring Is of Limited Usefulness in Guiding Autotransplantation in Reoperative or Subtotal Parathyroidectomy for Primary Hyperparathyroidism.

INTRODUCTION: Patients with primary hyperparathyroidism (1HPT) undergoing reoperative or subtotal parathyroidectomy (PTX) may undergo autotransplantation (ATX) when the viability of remaining tissue is unknown. This study aims to identify whether intraoperative parathyroid hormone levels (IOPTH) can determine ATX candidacy. METHODS: Patients with 1HPT who underwent PTX with ATX at our institution were identified. IOPTH and PTH values within 24 h, 2-4 weeks, and >1 month postoperative were analyzed. Patients were classified as either a candidate for ATX (low PTH after 2-4 weeks) or not a candidate based on postoperative PTH (normal PTH after 2-4 weeks). Associations of ATX candidate status with demographic and clinical attributes were studied. RESULTS: 268 had a reoperative (49%) or subtotal PTX with ATX. 151 had data for PTH analysis, and 21 (14%) were identified as candidates for ATX. The mean % decline in IOPTH from baseline to 20 min post-excision was 51% in noncandidates vs 73% in candidates (P = .002). The mean change in IOPTH from baseline to final was 52% in noncandidates and 83% in candidates (P = .009). A decrease in IOPTH from baseline to 20 min post-excision of 23.4% or greater or a final PTH of 52 pg/mL or less would be an indication for ATX. Of the 21 who needed an ATX, it failed in 10. CONCLUSION: Parathyroid ATX is frequently unnecessary, and the viability is less than expected. While candidates for ATX have a greater IOPTH % decline at all points during surgery and a lower final IOPTH, the clinical practicality of using IOPTH to determine ATX candidacy is limited.

Pediatric primary hyperparathyroidism: Surgical pathology and long-term outcomes in sporadic and familial cases.

BACKGROUND: Primary Hyperparathyroidism (PHPT) is rare in pediatric patients. Data regarding surgical outcomes are scarce. METHODS: Single-center retrospective review (1994-2020) of patients ≤21 years undergoing surgery for PHPT. RESULTS: 66 patients were identified (61% female, 17 ± 3 years). 71% of patients were symptomatic at diagnosis. 32% of patients had known familial syndromes, most commonly MEN-1. 23% of patients without a known mutation had genetic testing, 22% positive. 56% of the total and 19% of the familial cohort underwent focused exploration. Single gland disease was found in 19% of familial vs 85% of sporadic cases, p < 0.00001. Persistence was 9%, all in the sporadic group, p = 0.11. Recurrence was 15%: 38% in the familial vs 2% in the sporadic groups, p=0.0004. Time to recurrence was 59 months (Q1-38, Q3-95), familial 61 vs 124 months sporadic, p=0.001. CONCLUSION: Pediatric PHPT is frequently sporadic, although 5% of apparent sporadic cases are secondary to syndromes. Familial cases have higher rates of recurrence, requiring closer follow-up.

Unilateral Adrenalectomy for Primary Aldosteronism Due to Bilateral Adrenal Disease Can Result in Resolution of Hypokalemia and Amelioration of Hypertension.

BACKGROUND: Bilateral idiopathic hyperaldosteronism (IHA) is responsible for 60% of primary aldosteronism (PA) cases. Medical management is standard of care for IHA. Unilateral adrenalectomy (UA) with the intent of debulking total aldosterone production as a palliative measure remains controversial. METHODS: Single-center retrospective review (2010-2020) of patients undergoing UA with a diagnosis of PA due to IHA (lateralization index [LI] on adrenal venous sampling [AVS] < 4). Demographic, pre-operative, intra-operative, and post-operative variables were assessed. Hypertensive regimens were converted to the WHO Defined Daily Dose (DDD). RESULTS: Twenty-four patients were identified, 14, 58% male and mean age 52 ± 10 years. Preoperative hypokalemia was present in 22, 92% of patients. Median number of antihypertensives taken was 3 (interquartile range [IQR], 2-4) and median DDD was 4 (IQR, 3-5.3). Median lateralization index on AVS was 3.52 (range, 1.19-3.88). All operations were performed in minimally invasive fashion. There were no conversions to open procedure, ICU admissions, or post-operative complications. Median follow-up was 10.5 months (range, 1-145 months). Hypokalemia resolved in 17, 76% of patients at last follow-up. Post-operative median number of antihypertensives taken was 1 (IQR, 1-3) and median DDD was 2 (IQR, 0.5-2.75) from 4, P = 0.003. Three (%) patients required continuation of mineralocorticoid receptor antagonists post-operatively. Blood pressure control improved in 65% of patients. CONCLUSION: Unilateral adrenalectomy in the setting of bilateral hyperaldosteronism can improve blood pressure control and stabilize potassium levels in selected patients. Further prospective studies in larger cohorts will be necessary to further define the role of unilateral adrenalectomy in the setting of PA due to bilateral adrenal disease.

Use of fluorescence imaging and indocyanine green during thyroid and parathyroid surgery: Results of an intercontinental, multidisciplinary Delphi survey.

BACKGROUND: In recent years, fluorescence imaging-relying both on parathyroid gland autofluorescence under near-infrared light and angiography using the fluorescent dye indocyanine green-has been used to reduce risk of iatrogenic parathyroid injury during thyroid and parathyroid resections, but no published guidelines exist regarding its use. In this study, orchestrated by the International Society for Fluorescence Guided Surgery, areas of consensus and nonconsensus were examined among international experts to facilitate future drafting of such guidelines. METHODS: A 2-round, online Delphi survey was conducted of 10 international experts in fluorescence imaging use during endocrine surgery, asking them to vote on 75 statements divided into 5 modules: 1 = patient preparation and contraindications to fluorescence imaging (n = 11 statements); 2 = technical logistics (n = 16); 3 = indications (n = 21); 4 = potential advantages and disadvantages of fluorescence imaging (n = 20); and 5 = training and research (n = 7). Several methodological steps were taken to minimize voter bias. RESULTS: Overall, parathyroid autofluorescence was considered better than indocyanine green angiography for localizing parathyroid glands, whereas indocyanine green angiography was deemed superior assessing parathyroid perfusion. Additional surgical scenarios where indocyanine green angiography was thought to facilitate surgery are (1) when >1 parathyroid gland requires resection; (2) during redo surgeries, (3) facilitating parathyroid autoimplantation; and (4) for the predissection visualization of abnormal glands. Both parathyroid autofluorescence and indocyanine green angiography can be used during the same procedure and employing the same imaging equipment. However, further research is needed to optimize the dose and timing of indocyanine green administration. CONCLUSION: Though further research remains necessary, using fluorescence imaging appears to have uses during thyroid and parathyroid surgery.

Histopathology and Genetic Causes of Primary Aldosteronism in Young Adults.

CONTEXT: Due to its rare incidence, molecular features of primary aldosteronism (PA) in young adults are largely unknown. Recently developed targeted mutational analysis identified aldosterone-driver somatic mutations in aldosterone-producing lesions, including aldosterone-producing adenomas (APAs), aldosterone-producing nodules (APNs), and aldosterone-producing micronodules, formerly known as aldosterone-producing cell clusters. OBJECTIVE: To investigate histologic and genetic characteristics of lateralized PA in young adults. METHODS: Formalin-fixed, paraffin-embedded adrenal tissue sections from 74 young patients with lateralized PA (<35 years old) were used for this study. Immunohistochemistry (IHC) for aldosterone synthase (CYP11B2) was performed to define the histopathologic diagnosis. Somatic mutations in aldosterone-producing lesions were further determined by CYP11B2 IHC-guided DNA sequencing. RESULTS: Based on the CYP11B2 IHC results, histopathologic classification was made as follows: 48 APAs, 20 APNs, 2 multiple aldosterone-producing nodules (MAPN), 1 double APN, 1 APA with MAPN, and 2 nonfunctioning adenomas (NFAs). Of 45 APAs with successful sequencing, 43 (96%) had somatic mutations, with KCNJ5 mutations being the most common genetic cause of young-onset APA (35/45, 78%). Of 18 APNs with successful sequencing, all of them harbored somatic mutations, with CACNA1D mutations being the most frequent genetic alteration in young-onset APN (8/18, 44%). Multiple CYP11B2-expressing lesions in patients with MAPN showed several aldosterone-driver mutations. No somatic mutations were identified in NFAs. CONCLUSION: APA is the most common histologic feature of lateralized PA in young adults. Somatic KCNJ5 mutations are common in APAs, whereas CACNA1D mutations are often seen in APNs in this young PA population.

Clinical course and imaging characteristics of benign adrenal cysts: a single-center study of 92 patients.

Objective: Benign adrenal cysts are rare lesions of the adrenal glands. Limited data are available to guide management. We aimed to describe the presentation and outcomes of patients with benign adrenal cysts. Design: Retrospective longitudinal cohort study. Methods: Consecutive patients with histologically or radiologically confirmed adrenal cysts between 1995 and 2021 were identified. Pheochromocytomas and malignancy were excluded. Results: Benign adrenal cysts were diagnosed in 92 patients (53, 57% women) at a median age of 45 years. Mode of discovery was incidental on imaging in 81 (88%), symptoms of mass effect in 9 (9.8%), and others in 2 (2.2%). Majority (89, 97%) of patients had unilateral cysts (45 right, 44 left) with a median size of 48 mm (range 4-200) at diagnosis. On imaging, most cysts were round/oval (85.4%), homogenous (83.2%) lesions with calcifications (64.0%) and no vascular enhancement (97.7%). During a median follow-up of 65 months (range 7-288), adrenal cysts demonstrated minimal enlargement (median size change 6 mm, median growth rate 2 mm/year). On hormonal evaluation, 10% (5/50 tested) had an abnormal overnight dexamethasone suppression test, and 9.5% (4/42 tested) had an abnormal case detection testing for primary aldosteronism. Patients treated with adrenalectomy (46, 50%) were younger (36.9 years vs 50.8 years, P = 0.0009) and had more rapidly enlarging cysts (median growth rate 5.5 mm/year vs 0.4 mm/year, P = 0.0002). Conclusion: Benign adrenal cysts are usually incidentally discovered, non-functional, homogenous lesions without vascular enhancement that demonstrate minimal growth. Adrenalectomy should be reserved for patients with heterogeneous lesions, abnormal hormonal evaluation, or those with mass effect symptoms.

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.

Informing therapeutic lymphadenectomy: Location of regional metastatic lymph nodes in adrenocortical carcinoma.

BACKGROUND: The anatomic boundaries of lymphadenectomy for adrenocortical carcinoma (ACC) are not defined. METHODS: Adults undergoing resection of ACC were included. Locations were categorized based on positive LN locations on final pathology. RESULTS: Of 231 resected ACC, 6% had positive LN during initial resection. Positive LN in left ACC (n = 7) were: 2 para-aortic, 2 left renal-hilar, 1 para-aortic and left renal-hilar and 1 unknown, while for right ACC (n = 7): 2 para-caval, 1 para-caval and right renal-hilar, 1 inter-aortocaval, 1 celiac, 1 para-aortic, and 1 unknown. Of 55 resections for recurrent ACC, positive LN in left ACC (n = 2) were: 1 para-aortic, and 1 para-aortic with left renal-hilar, while LN for right ACC (n = 7): 2 inter-aortocaval, 2 right renal-hilar, 2 para-caval and one retrocrural. CONCLUSION: The most common LN metastases are para-caval for right, and para-aortic and left renal-hilar for left ACC. Further studies are necessary to determine the boundaries of lymphadenectomy in ACC resection.

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.

Perspectives on virtual interviews-A follow-up study of the Comprehensive Endocrine Surgery Fellowship interview process.

BACKGROUND: The American Association of Endocrine Surgeons Comprehensive Endocrine Surgery Fellowship interview stakeholders previously favored in-person interviews, despite time and expense. This study assessed perception changes given mandated virtual interviews because of coronavirus disease 2019. METHODS: Immediately after the 2020 Match, anonymous surveys were distributed to applicants (n = 37) and program directors (n = 22). Mixed-methods analyses were used to evaluate responses. Results were compared to data from a prior study of the 2013 to 2018 in-person interview process. RESULTS: Response rates were 82% (program directors) and 60% (applicants). Compared with prior applicants, 2020 applicants attended similar numbers of interviews (1-10, 32% vs 37%; P = .61), used fewer vacation days (23% vs 56%; P = .01), and most reported 0 expenses. Burdens included lack of protected time for interviews. The virtual format did not compromise applicant ability to meet faculty (mean rank = 6.8/10) or make favorable impressions (mean rank = 6.8/10). Program directors reported equivalent or improved assessments of applicants. Program directors (72%) and applicants (77%) indicated that future interviews should be partially or completely virtual. CONCLUSION: In contrast to prior survey data, applicants and program directors now express interest in virtual or hybrid interview processes. Virtual interviews were less costly, less time-consuming, and met goals effectively. Integrating virtual interview components will require innovative strategies to reduce redundancies and promote equitable access.

Multiple endocrine neoplasia type 1 in children and adolescents: Clinical features and treatment outcomes.

BACKGROUND: Clinical manifestations and treatment outcomes in children and adolescents with multiple endocrine neoplasia type 1 are not well characterized. METHODS: We conducted a retrospective cohort study of 80 patients with multiple endocrine neoplasia type 1 who commenced tumor surveillance at ≤18 years of age. RESULTS: Fifty-six patients (70%) developed an endocrine tumor by age ≤18 years (median age = 14 years, range = 6-18 years). Primary hyperparathyroidism occurred in >80% of patients, with >70% undergoing parathyroidectomy, in which less-than-subtotal (<3-gland) resection resulted in decreased disease-free outcomes versus subtotal (3-3.5-gland) or total (4-gland) parathyroidectomy (median 27 months versus not reached; P = .005). Pancreaticoduodenal neuroendocrine tumors developed in ∼35% of patients, of whom >70% had nonfunctioning tumors, >35% had insulinomas, and <5% had gastrinomas, with ∼15% having metastases and >55% undergoing surgery. Pituitary tumors developed in >30% of patients, and ∼35% were macroprolactinomas. Tumor occurrence in male patients and female patients was not significantly different. Genetic analyses revealed 38 germline MEN1 mutations, of which 3 were novel. CONCLUSION: Seventy percent of children aged ≤18 years with multiple endocrine neoplasia type 1 develop endocrine tumors, which include parathyroid tumors for which less-than-subtotal parathyroidectomy should be avoided; pancreaticoduodenal neuroendocrine tumors that may metastasize; and pituitary macroprolactinomas.

Comparison of short- and long-term postoperative occurrences after robotic single-incision cholecystectomy versus multiport laparoscopic cholecystectomy.

BACKGROUND: Long-term outcomes of SIRC are not well established. Furthermore, SIRC is only now being considered more frequently for patients with independent risk factors for PSH, such as obesity. As such, the paucity of data on longer-term post-surgical outcomes of SIRC is particularly notable as it pertains to obese patients. METHODS: All patients undergoing cholecystectomy performed by two surgeons at our institution from 2008-2018 were reviewed. Our inclusion criteria were patients who underwent SIRC or multiport laparoscopic cholecystectomy (MPLC) and had at least one month of postoperative follow-up. Patients who underwent additional procedures at the time of cholecystectomy were excluded. Our outcomes of interest were the 30-day POC rate and the long-term PSH rate. Analysis was conducted on an intention-to-treat basis, using logistic regression analysis for POC and time-to-event analysis for PSH. RESULTS: We examined 584 patients who underwent either SIRC (51%) or MPLC (49%). Of the 296 patients who underwent SIRC, 15 (5%) developed a POC and 23 (8%) developed a PSH. Of the 288 patients who underwent MPLC, 11 (4%) developed a POC, and 28 (10%) developed a PSH. Procedure group and obesity was not associated with the risk of POC (p = 0.29, p = 0.21, respectively). Procedure group was not associated with an increased risk of PSH (p = 0.29). Obese patients, however, were 1.94 times more likely to develop PSH compared to non-obese patients overall (p = 0.02). CONCLUSIONS: There is no statistically significant difference in POC and PSH rate following SIRC when compared with MPLC in patients in the same BMI group. Male gender and prior abdominal surgery are risk factors for POC, while advancing age and obesity are risk factors for PSH.

Diagnostic Accuracy of Dehydroepiandrosterone Sulfate and Corticotropin in Autonomous Cortisol Secretion.

Autonomous cortisol secretion (ACS) affects up to 50% of patients with adrenal adenomas. Despite the limited evidence, clinical guidelines recommend measurement of serum concentrations of dehydroepiandrosterone-sulfate (DHEA-S) and corticotropin (ACTH) to aid in the diagnosis of ACS. Our objective was to determine the accuracy of serum concentrations of DHEA-S and ACTH in diagnosing ACS. We conducted a retrospective single center study of adults with adrenal adenoma evaluated between 2000-2020. Main outcome measure was diagnostic accuracy of DHEA-S and ACTH. ACS was defined as post-dexamethasone cortisol >1.8 mcg/dL. Of 468 patients, ACS was diagnosed in 256 (55%) patients with a median post-DST cortisol of 3.45 mcg/dL (range, 1.9-32.7). Patients with ACS demonstrated lower serum concentrations of DHEA-S (35 vs. 87.3 mcg/dL, p < 0.0001) and ACTH (8.3 vs. 16 pg/mL, p < 0.0001) compared to patients with non-functioning adrenal tumors (NFAT). Serum DHEA-S concentration <40 mcg/dL diagnosed ACS with 84% specificity and 81% PPV, while serum ACTH concentration <10 pg/mL diagnosed ACS with 75% specificity and 78% PPV. The combination of serum concentrations of DHEA-S <40 mcg/dL and ACTH <10 pg/mL diagnosed ACS with the highest accuracy with 92% specificity and 87% PPV. Serum concentrations of DHEA-S and ACTH provide additional value in diagnosing ACS.