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Primary Hyperparathyroidism (PHPT) is rare in pediatric patients. Data regarding surgical outcomes are scarce.Single-center retrospective review (1994-2020) of patients ≤21 years undergoing surgery for PHPT.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.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.

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.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.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 (pgt; 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 (plt; 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%; plt; 0.01).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.

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.

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.

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.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).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.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.

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. 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. 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). 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.

Tertiary hyperparathyroidism following kidney transplantation is most commonly characterized by 4-gland hyperplasia, but single and double adenomatous disease has been demonstrated in this population as well. It is unknown whether preoperative imaging can assist in identifying patients who may qualify for focused surgery for adenomatous disease. We performed a retrospective review of our patient database from 1998–2018 for patients with tertiary hyperparathyroidism following renal transplant. Patient charts were reviewed for patient demographics, laboratory values, preoperative imaging, operative findings, pathology, and complications. We identified 113 patients with tertiary hyperparathyroidism following renal transplant who underwent parathyroidectomy. There were 51 females and 62 males with a mean age of 53.4 ± 13.4 years. Median preoperative calcium and PTH were 10.9 mg/dl (IQR 10.3–11.2) and 228 pg/ml (IQR 118–305). Preoperative ultrasound was performed in 60 patients. Of these, 11 (18%) were negative, 38 (63%) showed 1–2 adenomas, and 11 (18%) showed ≥ 3 adenomas. 99mTc-sestamibi parathyroid scintigraphy was performed in 101/113 patients. Of these, 11 (11%) were negative, 62 (61%) showed 1–2 areas of discordant sestamibi uptake, and 28 (28%) showed ≥ 3 areas of discordant uptake. Ultimately, 19 (17%) patients had a single adenoma removed, 16 (14%) had 2 adenomas removed, and (69%) had multi-gland disease. There were 26 ectopic glands found in 21 patients, 42.3% of which were identified on preoperative imaging. 94.1% of patients were eucalcemic at last follow-up, mean (± SD) 5.8 ± 3.6 years. Adenomas that were visualized on ultrasound were larger on pathology than those non-visualized (997 ± 120 mg (mean ± SE) vs. 388 ± 109 mg, p = 0.0003). This was also true for parathyroid scintigraphy (647 ± 41 mg vs. 355 ± 51 mg, p = 0.0001). In patients with tertiary hyperparathyroidism, preoperative imaging can aid in predicting which patients will have 1–2 gland disease. In patients with 1–2 gland disease on congruent ultrasound and nuclear medicine imaging studies, the accuracy increases to 59%. Preoperative imaging can help identify ectopic glands. Larger adenomas are more likely to be identified on both imaging modalities.

Background Endogenous Cushing syndrome (CS) can be caused by ectopic corticotropin-producing tumors of known (EK) and unknown origin (EU). Bilateral adrenalectomy (BA) can be used as definite treatment of hypercortisolism in such cases. This study compared patients undergoing BA for CS secondary to EK vs EU. Methods Retrospective review (1995–2017) of patients undergoing BA due to EK or EU. We analyzed demographic characteristics, laboratory values, intraoperative variables, surgical outcomes, and survival. Results 48 patients (26 EU, 22 EK) were identified. Serum cortisol and ACTH concentrations were similar. 92% of BA for EU were performed minimally invasively vs 77% for EK, P = 0.22. Complications occurred in 19% of EU and 4.5% EK, P = 0.2. Mean survival was 4.3 years for EU and 4.0 years for EK without difference in all-cause mortality P = 0.63. Conclusion BA cure rate was 100% for CS in EU and EK. Morbidity, long term and all-cause mortality differences were not statistically significant between EK and EU.

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.

Understanding the method of thyroid cancer detection has potential implications on interpreting incidence rates, the diagnosis and management of thyroid cancer. We conducted a systematic review of studies reporting methods of thyroid cancer detection to estimate the frequency of incidentally found cancers and classify triggers of incidental thyroid cancer diagnosis. We searched multiple bibliographic databases from inception to June 2020. A pair of reviewers, working independently and in duplicate selected studies for inclusion, extracted data, and evaluated each trial’s risk of bias. Studies enrolling patients older than 18 years with thyroid cancer confirmed histologically were included. In total, 17 cohorts and 1 cross-sectional study, conducted between 1991 and 2018, enrolling 4668 patients with thyroid cancer were included: 88% had papillary thyroid cancer and 23% had papillary thyroid microcarcinoma. The proportion of patients with non-incidental and incidental thyroid cancer was similar: 49% [95% confidence interval (CI): 40–58%]. Subgroup analysis showed that most patients with incidental thyroid cancers had tumor size 45 (61%; 95% CI: 56–67%), and were detected through imaging (35%; 95% CI: 26–45%), of which ultrasound was the most common modality (27%; 95% CI: 14–43%). The heterogeneity for all the effect sizes was large and significant. About half of thyroid cancers were found incidentally through the use of imaging studies, in particular neck ultrasound. These incidentally found cancers were mostly small papillary thyroid cancer. These results highlight opportunities for interventions aimed at reducing drivers of overdiagnosis.

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. 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. 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

Background Hypercalciuria is an important manifestation of primary hyperparathyroidism and may contribute to the risk of nephrolithiasis. This study examined the impact of parathyroidectomy on 24-hour urinary calcium (24-hour UCa) levels and rates of resolution of hypercalciuria after surgery. Methods A retrospective cohort study was performed of patients who underwent curative parathyroidectomy for primary hyperparathyroidism from 2007 to 2017. Baseline and postoperative urine and serum biochemistry levels were analyzed. The relationship between preoperative 24-hour UCa levels and the absolute decrease in postoperative UCa excretion was assessed using Spearman’s rank correlation coefficient. Results Of 110 patients, 84 (76.4%) experienced a ≥20% decrease in 24-hour UCa level postoperatively. These patients had a higher baseline median 24-hour UCa level (293.5 vs 220.5 mg/24-hour; P = .001), higher baseline mean serum parathyroid hormone (106.5 vs 83; P = .05) and were more likely to have single gland disease (85.7% vs 65.4%, P = .04) compared with patients in whom 24-hour UCa excretion did not improve. Of the 28 patients (25%) who were hypercalciuric (24-hour UCa >400 mg/day) at baseline, 22 (79%) became normocalciuric postoperatively. A linear correlation was observed between preoperative 24-hour UCa levels and the decline in 24-hour UCa excretion after surgery (R2 = 0.59, P Conclusion Parathyroidectomy reduces 24-hour UCa excretion in the majority of patients with PHPT and restores normocalciuria in 79% of patients with hypercalciuria at baseline.

Bilateral adrenalectomy (BA) is a curative treatment for hypercortisolism in patients with corticotropin (ACTH)-dependent Cushing syndrome. Indications include refractory Cushing’s disease (CD) following failed pituitary surgery, and occult or unresectable ectopic ACTH-producing neoplasms (EA). This was a single-center, retrospective review (1995–2017) of patients undergoing BA for CD or EA. Demographics, laboratory findings, and intraoperative and postoperative variables were analyzed. Of 137 patients, 83 (61%) had CD and 54 (39%) had EA; 87% of CD patients were female versus 46% of EA patients (p

OBJECTIVE: Systematic approach to myelolipoma remains poorly defined. We aimed to describe clinical course of myelolipoma and to identify predictors of tumor growth and need for surgery. METHODS: We conducted a retrospective study of consecutive patients with myelolipoma. RESULTS: A total of 321 myelolipomas (median size, 2.3 cm) were diagnosed in 305 patients at median age of 63 years (range, 25-87). Median follow-up was 54 months. Most myelolipomas were incidentally detected (86%), whereas 9% were discovered during cancer staging, and 5% during workup of mass effect symptoms. Thirty seven (12%) patients underwent adrenalectomy. Compared to myelolipomas

BACKGROUND: Excluding a pheochromocytoma is important when a patient presents with an incidentally discovered adrenal mass. However, biochemical testing for pheochromocytoma can be cumbersome, time consuming, or falsely positive. Our objective was to determine if unenhanced computed tomography (CT) imaging alone can be used to rule out pheochromocytoma. METHODS: We performed a retrospective study of all patients with a pathologically confirmed pheochromocytoma and unenhanced CT imaging who were treated at the Mayo Clinic between 1998 and 2016. Additionally, we performed a systematic review and meta-analysis of original studies published after 2005 with patients who had adrenal masses, more than 10 patients with pheochromocytomas, and reported attenuation on unenhanced CT imaging in Hounsfield units (HU). RESULTS: In the Mayo cohort, we identified 186 patients and 199 pheochromocytomas with unenhanced CT imaging. The mean unenhanced CT attenuation was 35±9 HU (range, 15–62), and only 15 tumors had attenuation ≤20 HU. The systematic review identified 26 studies (1,217 tumors), and 23 studies provided a mean unenhanced CT attenuation. The overall mean unenhanced CT attenuation across the studies was 35.6 HU (95% CI, 22.0–49.1 HU). A cutoff of >10 HU had a 100% sensitivity (95% CI, 1.00–1.00) for pheochromocytoma with low heterogeneity between the 21 qualified studies (I(2)=0%). Sensitivity for pheochromocytoma was 100% and 99% for an unenhanced CT attenuation cutoff of >15 and >20 HU. CONCLUSIONS: Biochemical testing may not be required to exclude pheochromocytoma if an incidental adrenal mass has low attenuation (

We performed a retrospective study of our large patient cohort aiming to examine the ability to predict postthyroidectomy hypocalcemia. A retrospective review of patients who underwent total or near-total thyroidectomy at our institution between 2008 and 2018 was performed. Postoperative hypocalcemia was defined as Ca level

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.

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 &gt, 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 &lt, 0.0001) and ACTH (8.3 vs. 16 pg/mL, p &lt, 0.0001) compared to patients with non-functioning adrenal tumors (NFAT). Serum DHEA-S concentration &lt, 40 mcg/dL diagnosed ACS with 84% specificity and 81% PPV, while serum ACTH concentration &lt, 10 pg/mL diagnosed ACS with 75% specificity and 78% PPV. The combination of serum concentrations of DHEA-S &lt, 40 mcg/dL and ACTH &lt, 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.

Patients undergoing unilateral adrenalectomy for primary aldosteronism (PA) may have a solitary adenoma, unilateral hyperplasia, or multiple adenomas on final pathology. This study investigated whether the underlying pathological diagnosis was associated with differences in clinical presentation and postoperative outcomes. A retrospective cohort study of patients undergoing unilateral adrenalectomy for PA from 2004 to 2015 at our institution was performed. Baseline clinical and laboratory parameters, as well as postoperative biochemical and hypertension cure rates, were compared across the three aforementioned pathological groups. Of 206 patients who met criteria for inclusion, 152 (73.8%) had a single adenoma, 33 (16%) had unilateral hyperplasia, and 21 (10.2%) had multiple unilateral adenomas. Patients with unilateral hyperplasia were more likely to be male (81.2% vs 57.9%, P = .03), undergo left-sided adrenalectomy (78.8% vs 47.4%, P

BACKGROUND There is a paucity of data regarding optimal treatment options and outcomes for recurrent disease after lateral neck dissection in patients with papillary thyroid carcinoma. METHODS Retrospective review of patients who underwent either percutaneous ethanol injection or surgery for first-time ipsilateral recurrences after ipsilateral lateral neck dissection for papillary thyroid carcinoma was performed. RESULTS Follow-up data were available for 54 patients with recurrences in 57 lateral necks treated by either percutaneous ethanol injection (n = 32) or surgery (n = 25). Tumor burden at the time of lateral neck recurrence differed between the groups including the largest lymph node diameter (mean: 13 mm vs 18 mm, P < .01) and the mean number of metastatic lymph nodes identified on ultrasound (1.3 vs 1.9, P = .04). Each modality alone achieved similar estimated rates of disease control at 36 months (75% for percutaneous ethanol injection and 74% for surgery, P = .8) with similar number of reinterventions (1.8 for percutaneous ethanol injection, 1.6 for surgery, P = .6). CONCLUSIONS Both ethanol ablation and surgery can achieve disease control in the majority of patients with recurrences after ipsilateral lateral neck dissection for papillary thyroid carcinoma. Ethanol ablation, when used for treatment of a single small lymph node, can result in outcomes that are similar to reoperative surgery for larger and multiple lymph nodes.

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 (70% had nonfunctioning tumors, >35% had insulinomas, 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. Conclusions: 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.

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.

We present the robotic harvest of a pedicled omentum flap for reconstruction of a near-total anterior chest wall defect. The patient was a 68-year-old woman with recurrent secondary chest wall angiosarcoma after previous mastectomy and radiation therapy. She underwent neoadjuvant chemotherapy and radiation, followed by wide radical chest wall resection with a final defect size of 15×35 cm. A one-stage reconstruction was performed with an omentum flap harvested by robotic technique and split-thickness skin grafts from thigh donor sites. The patient healed with minimal complications. Our case supports more widespread application of robotics in plastic and reconstructive surgery.

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.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.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).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.

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.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.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.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.

How malignant insulinomas present relative to benign insulinomas is unknown.A single-institution retrospective study identified patients with insulinoma. Malignancy was defined by distant metastases, positive lymph node(s), T stage of 4, direct invasion into surrounding peripancreatic tissue, or presence of lymphovascular invasion. Wilcoxon Rank Sum tests and Kaplan-Meier analysis were used.A total of 311 patients were identified: 51 malignant and 260 benign. Patients with malignant insulinoma presented with higher levels of insulin, proinsulin, and c-peptide. Malignant lesions were larger: 4.2 ± 3.2 vs 1.8 ± 0.8 cm in benign lesions, p 0.01. Overall survival at 5 years was 66.8% vs 95.4% for malignant and benign insulinoma respectively, p 0.01.Larger size of insulinoma and increased serum β-cell polypeptide concentrations were associated with malignancy. Malignant insulinoma has poorer survival. Further work-up to rule out malignancy may be indicated for larger pancreatic lesions and for patients with higher pre-operative insulin and pro-insulin.

In 2018, our institution implemented opioid prescribing guidelines for endocrine surgery.We evaluated prescribing trends before and after the guidelines (60 MME following adrenal procedures and 37.5 MME for thyroid/parathyroid procedures) using chi-squared and Wilcoxon Rank-Sum tests.We identified 357 patients in the pre-guideline and 397 in the post-guideline period. The proportion discharged with any opioid prescription decreased from 96.1% to 77.3%, p 0.01, and the median (IQR) prescribed amount decreased from 150.0 (100.0, 200.0) to 50.0 (25.0, 75.0), p 0.01 overall and within each category. The proportion receiving prescription above the upper guidelines limit also decreased, while opioid refills within 30-day of discharge remained stable (2.8% before and 4.5% after the guidelines, p = 0.21).Opioid prescribing guidelines for endocrine surgical procedures decreased both the proportion of patients receiving opioids and the amount when prescribed, therefore further supporting the utility of opioid prescribing guidelines in decreasing over-prescription.

We performed a retrospective study of our large patient cohort aiming to examine the ability to predict postthyroidectomy hypocalcemia. A retrospective review of patients who underwent total or near-total thyroidectomy at our institution between 2008 and 2018 was performed. Postoperative hypocalcemia was defined as Ca level8.0 mg/dL (reference range: 8.9-10.1 mg/dL) within 30 days of operation. Logistic regression was used to develop models for prediction of the occurrence of postoperative hypocalcemia. Inclusion criteria were met by 1463 patients. Hypocalcemia was documented in 223 patients (15%). Models based on parathyroid hormone (PTH) levels alone had an associated receiver operator characteristic with an areas under the curve (AUC) of 0.79. There was an inverse relationship between time of measurement and PTH levels within the first two hours after thyroidectomy (

Background The diagnostic threshold for mild autonomous cortisol secretion using low dose, overnight, dexamethasone suppression testing is recognized widely as a serum cortisol ≥1.8 mcg/dL. The degree to which these patients require postoperative glucocorticoid replacement is unknown. Methods We reviewed adult patients with corticotropin (ACTH)-independent hypercortisolism who underwent unilateral laparoscopic adrenalectomy for benign disease with a dexamethasone suppression testing ≥1.8 mcg/dL at our institution from 1996 to 2018. Patients with a dexamethasone suppression testing of 1.8 to 5 mcg/dL were compared with those with a dexamethasone suppression testing >5 mcg/dL. Results We compared 68 patients with a preoperative dexamethasone suppression testing of 1.8 to 5 mcg/dL to 53 patients with a preoperative dexamethasone suppression testing >5 mcg/dL. Preoperative serum ACTH (mean 10.0 vs 9.2 pg/mL), adenoma size (mean 3.4 vs 3.5 cm), and side of adrenalectomy (37 and 47% right) were similar between groups (P > .05 each). Patients with a dexamethasone suppression testing 1.8 to 5 mcg/dL were older (mean values 58 ± 11 vs 52 ± 16 years ; P = .01), less likely to be female (63 vs 81%; P = .03), had greater body mass indexes (33.1 ± 8.4 vs 29.1 ± 5.6; P = .01), and had lesser 24 hour preoperative urine cortisol excretions (32.6 ± 26.7 vs 76.1 ± 129.4 mcg; P = .03). Postoperative serum cortisol levels were compared in 22 patients with a dexamethasone suppression testing of 1.8 to 5 mcg/dL to 14 patients with a dexamethasone suppression testing >5 mcg/dL. Those with dexamethasone suppression testing 1.8 to 5 mcg/dL had greater postoperative serum cortisol levels (8.0 ± 5.7 vs 5.0 ± 2.6 mcg/dL; P = .03), were less likely to be discharged on glucocorticoid replacement (59% vs 89%; P = .003), and had a decreased duration of treatment (4.4 ± 3.8 vs 10.7 ± 18.0 months; P = .04). Conclusion Assessment of early postoperative adrenal function with mild autonomous cortisol secretion is necessary to minimize unnecessary glucocorticoid replacement.

CONTEXT Modern pheochromocytomas (PHEOs) are often discovered by incidental finding on cross-sectional imaging or mutation-based genetic case detection testing. Little is known about how these PHEOs behave. OBJECTIVE To describe the characteristics and behavior of PHEOs discovered incidentally on imaging or through mutation-based genetic case detection testing. DESIGN Retrospective study. SETTING Referral center. PATIENTS Consecutive patients with pathology-confirmed PHEOs, treated from 2005 to 2016. MAIN OUTCOME MEASURE(S) Tumor size, plasma/urine fractionated metanephrines and catecholamines, and preoperative management. RESULTS Two hundred seventy-one patients (52% women, median age 52.0 years) presented with 296 PHEOs. Discovery method was most often incidental finding on cross-section imaging (61%) rather than PHEO-related symptoms (27%) or mutation-based case detection testing (12%). Patients with incidentally discovered PHEOs were older than symptomatic and mutation-based case detection testing patients (median age 56.6 vs 43 vs 35 years, P < 0.0001). Mutation-based case detection PHEOs were smaller than those discovered due to symptoms (median size 29.0 vs 50.5 mm, P = 0.0027). Patients with PHEOs discovered due to symptoms had the highest median concentration of 24-hour urinary metanephrines and total plasma metanephrines (P < 0.0001). These patients required a higher cumulative phenoxybenzamine dose than patients with incidental or case detection PHEO (median 450 vs 375 vs 270 mg, P = 0.029). CONCLUSIONS PHEOs are primarily discovered due to incidental finding on cross-sectional imaging rather than PHEO-related symptoms. PHEOs discovered through mutation-based genetic case detection testing were smaller and required less α-adrenergic blockade preoperatively compared with PHEOs found due to symptoms, which supports routine case detection testing for patients genetically predisposed for PHEOs.

To Identify predictors of recurrent disease following lateral neck dissection (LND) for papillary thyroid carcinoma (PTC). A retrospective review of patients who underwent first-time LND for PTC at our institution (2000–2015) was performed. Medical records were examined for biopsy or pathologically proven lateral neck recurrence. Differences between the groups with and without recurrence were compared. All LNDs were then classified in to two groups: “comprehensive” (CND), involving levels IIa-Vb at minimum, or “selective”, labelling less extensive dissection (SND). Four hundred nine patients underwent 467 LNDs. Surveillance data were available for 317 patients who underwent 362 LNDs (mean age 45 ± 16; range 18–88). The median follow-up was 64 ± 48 months (range 3–197). Recurrence was detected in 71 lateral necks (20%). The total number of lymph nodes was greater in the group without recurrence compared to those with recurrence (23 vs. 19, p = 0.02). Among patient demographics, radioactive iodine treatment, primary tumor characteristics and characteristics of nodal metastases, only an older patient age (mean 50 vs. 43 years) was associated with lateral neck recurrence (p

Introduction: Cowden syndrome patients are at an increased risk of developing differentiated thyroid carcinoma (DTC). There are limited studies of thyroid cancer biology within this population. We aimed to describe the frequency and progression of DTC in this population. Method: We reviewed all patients with clinically or genetically confirmed Cowden syndrome treated at our institution between 1979 and 2017. Presentation, operative details, pathology, and adjuvant treatment for all thyroid diseases were analyzed. Results: Forty-seven patients were identified. Thirty-seven received a clinical diagnosis while 10 tested positive on genetic testing for the PTEN gene. Average follow-up time was 10 years. Twenty-three patients underwent a total thyroidectomy. Nine patients were diagnosed with thyroid cancer, with a mean age of 28.5 years, 4 cases of the follicular variant of papillary thyroid cancer (PTC), 4 of PTC, and 1 of cystic PTC. One patient required an interval lateral neck dissection but no patients developed distant metastasis. Fourteen patients underwent thyroidectomy, 12 due to follicular adenoma, Hürthle cell adenoma, or multinodular goiter, 1 due to Graves’ disease, and 1 who elected to undergo a prophylactic thyroidectomy. No mortality was associated with thyroid cancer in these patients. Conclusion: Patients with Cowden syndrome are at a significantly increased risk of developing DTC and have a younger age of onset. In this study, 20% of the patients with Cowden syndrome were diagnosed with DTC, but distant metastasis and overall mortality were absent. Clinical features were similar to those of sporadic DTC, proving that, apart from screening, Cowden syndrome-related DTC can be treated in similar fashion.

It has been suggested that accurate clinical decisions may be made in patients with primary aldosteronism (PA) in the setting of failed cannulation of an adrenal vein, thereby utilizing only data from either right or left adrenal venous sampling (AVS) alone.Retrospective analysis was performed for all patients with PA who underwent successful bilateral AVS. Adrenal vein/inferior vena cava index (AV/IVC index) was calculated by dividing aldosterone/cortisol ratio of the adrenal vein by aldosterone/cortisol ratio in the inferior vena cava, as described in a previously published study. We examined the rates of inappropriate adrenalectomy and failure to recognize unilateral disease when previously published cutoffs are used.Inclusion criteria were met in 150 patients; 61 with bilateral and 89 with unilateral disease. AV/IVC index cutoff of ≤0.5 to predict contralateral disease would have not led to any inappropriate adrenalectomies and would have missed 19% of patients with unilateral disease; AV/IVC index cutoff of ≥5.5 to predict ipsilateral unilateral disease would have resulted in inappropriate adrenalectomy in 18% of patients (95% CI 8-34%, P .01) and would have not recognized 55% of patients with unilateral disease (P .01).The cortisol-corrected adrenal vein/inferior vena cava aldosterone index with a cutoff value of ≤0.5 performed well in identifying patients with contralateral unilateral disease. AV/IVC index of ≥5.5 cannot be used to reliably diagnose ipsilateral unilateral disease because 18% of patients undergoing adrenalectomy based on this cutoff would have bilateral disease.

The Delphian nodes (DNs) are frequently involved in cancers of the head and neck, including laryngeal and thyroid malignancies. Positivity in the DN has been considered a predictor of recurrence as well as an overall aggressive tumor biology. However, little has been written regarding the consequences of recurrence at the site of the DN. We present two case reports regarding recurrence in the DN and the unique challenges associated with DN metastases. In addition, we discuss our surgical approach to disease at the prelaryngeal space, including workup, imaging, and resection., Delphian lymph nodes are a rare site of thyroid cancer recurrence but can have significant associated morbidity. We review recurrence and a multidisciplinary approach to treatment.

Autonomous functioning thyroid nodules (AFTNs) are generally considered to be benign entities, with malignancy found in about 1 per cent considered to be rare in the general population. Because of this low rate, fine needle aspiration is generally not recommended, and these lesions are most often treated with radioactive iodine, medication, or surgery. However, AFTNs are exceedingly rare in the pediatric population and the optimal treatment is not defined. We present a 14-year-old female patient with an AFTN treated with surgical resection and found to contain a follicular carcinoma with capsular invasion.

Bilateral adrenalectomy (BA) is a curative treatment for hypercortisolism in patients with corticotropin (ACTH)-dependent Cushing syndrome. Indications include refractory Cushing's disease (CD) following failed pituitary surgery, and occult or unresectable ectopic ACTH-producing neoplasms (EA).This was a single-center, retrospective review (1995-2017) of patients undergoing BA for CD or EA. Demographics, laboratory findings, and intraoperative and postoperative variables were analyzed.Of 137 patients, 83 (61%) had CD and 54 (39%) had EA; 87% of CD patients were female versus 46% of EA patients (p 0.0001). Mean age at diagnosis was 40 ± 15 years for CD and 49 ± 18 years for EA (p = 0.004). Preoperative serum cortisol concentrations were higher in the EA cohort (63 ± 40 μg/dL) versus the CD cohort (33 ± 19 μg/dL) [p 0.001], with no significant differences in serum ACTH. Time from diagnosis until adrenalectomy was 54 ± 69 months for CD versus 4 ± 13 for EA (p = 0.002). Most patients underwent minimally invasive surgery (MIS; 86% CD vs. 75% EA; p = 0.19). There was no difference between the rates of conversion to an open approach, intraoperative blood loss, or operative time between groups, and no difference between complications in CD versus EA (p = 1.0). Five-year survival was significantly shorter among the EA cohort (30% for EA vs. 80% for CD; p 0.001).Patients with EA presented with higher serum cortisol levels compared with patients with CD. EA patients were more likely to require intraoperative transfusion and postoperative intensive care. BA in patients with CD and EA can be performed safely in an MIS fashion, with similar morbidity; however, survival at 5 years was significantly less in the EA cohort.

Adrenalectomy for non-neuroblastic pathologies in children is rare with limited data on outcomes. We reviewed our experience of adrenalectomy in this unique population. Retrospective study of children (age ≤ 18) who underwent adrenalectomy with non-neuroblastic pathology from 1988 to 2018. Clinical and operative details of patients were abstracted. Outcomes included length of stay and 30-day postoperative morbidity. Forty children underwent 50 adrenalectomies (12 right-sided, 18 left-sided, 10 bilateral). Six patients (15%) presented with an incidental adrenal mass while 4 (10%) had masses found on screening for genetic mutations or prior malignancy. The remaining 30 (75%) presented with symptoms of hormonal excess. Nineteen patients (48%) underwent genetic evaluation and 15 (38%) had genetic predispositions. Diagnoses included 9 patients (23%) with pheochromocytoma, 8 (20%) with adrenocortical adenoma, 8 (20%) with adrenocortical carcinoma, 7 (18%) with adrenal hyperplasia, 2 (5%) with metastasis, and 6 (14%) with additional benign pathologies. Of 50 adrenalectomies, twenty-five (50%) were laparoscopic. Median hospital length of stay was 3 days (range 0–11). Post-operative morbidity rate was 17% with the most severe complication being Clavien–Dindo grade II. Adrenalectomy for non-neuroblastic pathology can be done with low morbidity. Its frequent association with genetic mutations and syndromes requires surgeons to have knowledge of appropriate pre-operative testing and post-operative surveillance.

Porcelain gallbladder is characterized by calcification of the gallbladder wall, possibly associated chronic inflammation from cholelithiasis. It is unknown whether porcelain gallbladder is associated with higher rates of hypercalcemia and/or hyperparathyroidism compared to cholelithiasis without porcelain gallbladder.We searched our patient database for patients with porcelain gallbladder on imaging and patients with cholelithiasis without porcelain gallbladder. We collected data on patient age, gender, calcium levels, parathyroid hormone (PTH) levels, and medications/comorbidities known to cause hypercalcemia.1000 patients within our database had porcelain gallbladder on imaging. Of these, 661 (245 male) had at least one serum calcium value for analysis. These patients were matched by age and gender with 6610 patients with cholelithiasis who had at least one serum calcium value. Rates of recurrent/persistent hypercalcemia were higher among patients with porcelain gallbladder at 16.8% versus 11.1% (p 0.01). Rates of hyperparathyroidism were also higher among porcelain gallbladder patients at 12% versus 7.5% (p 0.01).Patients with porcelain gallbladder show higher rates of hypercalcemia and hyperparathyroidism than patients with cholelithiasis alone.

Background To help control opioid overprescription, we conducted a large institutional, 3-site initiative to provide discharge prescribing guidelines for different procedures. Our aim is to refine institutional guidelines for parathyroidectomy. Methods Patients undergoing parathyroidectomy completed a 28-question survey about opioid consumption. Discharge opioid prescription amounts were converted into morphine milligram equivalents (MMEs) and reported as median and interquartile range (IQR). Consumption was dichotomized into top quartile MME users (Q4) versus standard users (Q1, Q3). Univariate analysis compared opioid consumption. Results A total of 91 patients were included; 90% were opioid-naive. While the median prescribed was 75 (IQR 75, 150) MME, the median consumed was 0 (IQR 0, 20). Top users reported higher pain scores [median (IQR): 2 (2, 4)] compared to standard users [1 (0, 3), P = 0.01]. However, there was no difference in opioid consumption between unilateral neck exploration, bilateral exploration, or thyroidectomy and parathyroidectomy, P = 0.11. There was no difference in opioid consumption by age, sex, or BMI (all P > 0.05). Of those receiving a prescription, 94.6% had left-over opioids at the time of survey, resulting in 82% of prescribed opioids being unused. Conclusions Over half of patients undergoing parathyroidectomy did not consume any opioid, and very few needed more than 2 d of opioid. Moreover, most patients did not dispose the unused opioids, which put these pills at risk of diversion and misuse. Surgical approach did not change consumption, illustrating that these guidelines are applicable to thyroidectomy given the similarity between techniques. We recommend prescribing nonopioid analgesics for patients undergoing parathyroidectomy.

Background While roughly half of adrenocortical carcinomas are functional, whether functional status impacts outcomes remains controversial. We compared presentation and survival for functional and nonfunctional neoplasms. Methods Adult patients presented with adrenocortical carcinomas at the Mayo Clinic were included. Tumor characteristics and outcomes were analyzed. Results The 266 identified patients presented with stage I (6%), II (33%), III (26%), and IV disease (32%); stage was unknown in 3%. Fifty-three percent of tumors were functional; patients with functional adrenocortical carcinomas were younger, more likely to be female, and more likely to present with metastatic disease. Surgical resection was undertaken in 84% of patients with 69% having R0 resection. While 30-day morbidity was similar between functional and nonfunctional adrenocortical carcinomas, median overall survival was better for nonfunctional adrenocortical carcinomas (median 66 vs 22 months, P = .01). Functional adrenocortical carcinomas was independently associated with shorter survival after adjusting for age, sex, grade, stage, and resection attempt: hazard ratio = 1.5 (95% confidence interval, 1.04–2.14, P = .03). Conclusion In our cohort, long term survival was worse for all patients with functional tumors. However, when analyzing patients with R0 resection, there was no survival difference between functional and nonfunctional adrenocortical carcinomas, signaling need for better understanding of adrenocortical carcinomas behavior to individualize and optimize treatment strategies.

Studies have demonstrated an association of the BRAF(V600E) mutation and microRNA (miR) expression with aggressive clinicopathologic features in papillary thyroid cancer (PTC). Analysis of BRAF(V600E) mutations with miR expression data may improve perioperative decision making for patients with PTC, specifically in identifying patients harboring central lymph node metastases (CLNM).Between January 2012 and June 2013, 237 consecutive patients underwent total thyroidectomy and prophylactic central lymph node dissection (CLND) at four endocrine surgery centers. All tumors were tested for the presence of the BRAF(V600E) mutation and miR-21, miR-146b-3p, miR-146b-5p, miR-204, miR-221, miR-222, and miR-375 expression. Bivariate and multivariable analyses were performed to examine associations between molecular markers and aggressive clinicopathologic features of PTC.Multivariable logistic regression analysis of all clinicopathologic features found miR-146b-3p and miR-146b-5p to be independent predictors of CLNM, while the presence of BRAF(V600E) almost reached significance. Multivariable logistic regression analysis limited to only predictors available preoperatively (molecular markers, age, sex, and tumor size) found miR-146b-3p, miR-146b-5p, miR-222, and BRAF(V600E) mutation to predict CLNM independently. While BRAF(V600E) was found to be associated with CLNM (48% mutated in node-positive cases vs. 28% mutated in node-negative cases), its positive and negative predictive values (48% and 72%, respectively) limit its clinical utility as a stand-alone marker. In the subgroup analysis focusing on only classical variant of PTC cases (CVPTC), undergoing prophylactic lymph node dissection, multivariable logistic regression analysis found only miR-146b-5p and miR-222 to be independent predictors of CLNM, while BRAF(V600E) was not significantly associated with CLNM.In the patients undergoing prophylactic CLNDs, miR-146b-3p, miR-146b-5p, and miR-222 were found to be predictive of CLNM preoperatively. However, there was significant overlap in expression of these miRs in the two outcome groups. The BRAF(V600E) mutation, while being a marker of CLNM when considering only preoperative variables among all histological subtypes, is likely not a useful stand-alone marker clinically because the difference between node-positive and node-negative cases was small. Furthermore, it lost significance when examining only CVPTC. Overall, our results speak to the concept and interpretation of statistical significance versus actual applicability of molecular markers, raising questions about their clinical usefulness as individual prognostic markers.

Autonomous functioning thyroid nodules (AFTN) are generally considered to be benign entities with malignancy found in about 1%. considered to be rare in the general population (1). Because of this low rate, fine needle aspirate (FNA) is generally not recommended and these lesions are most often treated with radioactive iodine (RAI), medication, or surgery. However, AFTN are exceedingly rare in the pediatric population and the optimal treatment is not defined. We present a 14 year-old female patient with an AFTN treated with surgical resection and found to contain a follicular carcinoma with capsular invasion.