Analysis of false-positive and false-negative results in 99mTc-MIBI SPECT/CT parathyroid imaging.

Background: Exact preoperative localization is desirable to perform minimally invasive parathyroidectomy for hyperparathyroidism (HPT). This study aimed to evaluate the diagnostic values of 99mTc-methoxyisobutylisonitrile (99mTc-MIBI) single photon emission computed tomography/computed tomography (SPECT/CT) of parathyroid glands by analyzing the relationship between lesion weight and false-negative (FN) results, as well as to explain the possible reason. Methods: The data from 314 patients with suspected HPT who underwent 99mTc-MIBI SPECT/CT parathyroid imaging between 2011 and 2022 were retrospectively evaluated. The sensitivity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of parathyroid 99mTc-MIBI SPECT/CT were calculated, and the false-positive (FP) and FN findings were analyzed. Results: Accurate localization by 99mTc-MIBI SPECT/CT was significantly associated with the parathyroid hormone (PTH) level. The 99mTc-MIBI SPECT/CT for diagnosis/lesion location reached a sensitivity of 84.6%/56.8%, a PPV of 97.3%/98.4%, an NPV of only 23.7%/4.18%, and an accuracy of 83.4%/57.1%, respectively. The largest diameter, shortest diameter, and lesion volume were lower in the FN group than in the TP group. A total of 7 FP cases were found, including 2 cases of thyroid nodules, 4 cases of thyroid tissue, and 1 case of hibernoma. A total of 45 FN patients, including 321 FN lesions, were confirmed, of which parathyroid hyperplasia accounted for 97.8%. Lesion weights greater than 20 µg were able to be detected, but lightweight lesions less than 100 mg were the principal source of FN results, accounting for approximately 39.3%. With lesion weights 0-100, 101-300, 301-1,000, and >1,000 mg, the FN rate was 70.8% (126/178), 51.8% (103/199), 34.6% (81/234), and 8.33% (11/132), respectively. Conclusions: 99mTc-MIBI SPECT/CT parathyroid imaging provides good sensitivity and high specificity in HPT location. Correct localization by 99mTc-MIBI SPECT/CT correlates positively with lesion weight and PTH levels. The smaller the lesion, the higher the FN rate in 99mTc-MIBI SPECT/CT parathyroid imaging, and lesions weighing less than 100 mg are the main source of FN results in 99mTc-MIBI SPECT/CT parathyroid imaging.

Vertical sleeve gastrectomy normalizes circulating glucocorticoid levels and lowers glucocorticoid action tissue-selectively in mice.

Objectives: Glucocorticoids produced by the adrenal cortex are essential for the maintenance of metabolic homeostasis. Glucocorticoid activation is catalysed by 11ß-hydroxysteroid dehydrogenase 1 (11ß-HSD1). Excess glucocorticoids are associated with insulin resistance and hyperglycaemia. A small number of studies have demonstrated effects on glucocorticoid metabolism of bariatric surgery, a group of gastrointestinal procedures known to improve insulin sensitivity and secretion, which were assumed to result from weight loss. In this study, we hypothesize that a reduction in glucocorticoid action following bariatric surgery contributes to the widely observed euglycemic effects of the treatment. Methods: Glucose and insulin tolerance tests were performed at ten weeks post operatively and circulating corticosterone was measured. Liver and adipose tissues were harvested from fed mice and 11ß-HSD1 levels were measured by quantitative RT-PCR or Western (immuno-) blotting, respectively. 11ß-HSD1 null mice (Hsd11b1 -/-) were generated using CRISPR/Cas9 genome editing. Wild type and littermate Hsd11b1 -/- mice underwent Vertical Sleeve Gastrectomy (VSG) or sham surgery. Results: Under the conditions used, no differences in weight loss were observed between VSG treated and sham operated mice. However, both lean and obese WT VSG mice displayed significantly improved glucose clearance and insulin sensitivity. Remarkably, VSG restored physiological corticosterone production in HFD mice and reduced 11ß-HSD1 expression in liver and adipose tissue post-surgery. Elimination of the 11ß-HSD1/Hsd11b1 gene by CRISPR/Cas9 mimicked the effects of VSG on body weight and tolerance to 1g/kg glucose challenge. However, at higher glucose loads, the euglycemic effect of VSG was superior to Hsd11b1 elimination. Conclusions: Bariatric surgery improves insulin sensitivity and reduces glucocorticoid activation at the tissular level, under physiological and pathophysiological (obesity) conditions, irrespective of weight loss. These findings point towards a physiologically relevant gut-glucocorticoid axis, and suggest that lowered glucocorticoid exposure may represent an additional contribution to the health benefits of bariatric surgery.

Gastric perforation secondary to Rapunzel syndrome.

Rapunzel syndrome is rare and describes a trichobezoar that extends through the pylorus into the jejunum, ileum or even the colon. Due to the large intraluminal size and weight they can attain, acute presentations of obstruction or perforation may occur. We report a case of a 17-year-old girl who presented to the emergency department following a syncopal episode. On examination, a left upper quadrant mass was appreciated with no signs of peritonism. Contrast-enhanced CT demonstrated a giant trichobezoar with resulting gastric perforation and intra-abdominal free fluid. Laparotomy and gastrotomy were performed and the patient had an uneventful recovery with psychiatric review prior to discharge. Though uncommon, bezoars should be included in our differential diagnosis as they can present in various ways owing to their size and weight. This case illustrates the risk of gastric perforation with large gastric bezoars.

Understanding the clinical course of genotype-negative MEN1 patients can inform management strategies.

BACKGROUND: It is unclear whether genotype-negative clinical multiple endocrine neoplasia type 1 patients derive equal benefit from prospective surveillance as genotype-positive patients. METHODS: In this retrospective cohort study, we compared genotype-negative patients with clinical multiple endocrine neoplasia type 1 with genotype-positive index cases. Primary outcome was age-related penetrance of manifestations; secondary outcomes were disease-specific survival and clinical course of endocrine tumors. RESULTS: We included 39 genotype-negative patients with clinical multiple endocrine neoplasia type 1 (Male: 33%) and 63 genotype-positive multiple endocrine neoplasia type 1 index cases (Male: 59%). Genotype-negative patients with clinical multiple endocrine neoplasia type 1 were 65 years old at last follow-up; genotype-positive multiple endocrine neoplasia type 1 index cases were 50 (P < .001). Genotype-negative patients with clinical multiple endocrine neoplasia type 1 were significantly older at their first and second primary manifestation. Only 1 developed a third primary manifestation. No genotype-negative patients with clinical multiple endocrine neoplasia type 1 with primary hyperparathyroidism and a pituitary adenoma developed a duodenopancreatic neuroendocrine tumor. Disease-specific survival was significantly better in genotype-negative patients with clinical multiple endocrine neoplasia type 1. In genotype-negative patients with clinical multiple endocrine neoplasia type 1, primary hyperparathyroidism was single-gland disease in 47% of parathyroidectomies versus 0% in genotype-positive multiple endocrine neoplasia type 1 index cases. In genotype-negative patients with clinical multiple endocrine neoplasia type 1, 17% of duodenopancreatic neuroendocrine tumors were multifocal versus 68% in genotype-positive multiple endocrine neoplasia type 1 index cases. Genotype-negative patients with clinical multiple endocrine neoplasia type 1 had more pituitary macroadenomas, fewer prolactinomas, and more somatotroph adenomas. CONCLUSION: Genotype-negative patients with clinical multiple endocrine neoplasia type 1 have a different clinical course than genotype-positive multiple endocrine neoplasia type 1 index cases. This may support a separate classification and a tailored surveillance regimen. Of the genotype-negative patients with clinical multiple endocrine neoplasia type 1 who had parathyroidectomy, almost half had no evidence of multigland disease and may be potential candidates for a more targeted single-gland approach.

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

PURPOSE: Disruption of calcium homeostasis is the most common complication after total thyroidectomy in adults. We explored the incidence and risk factors of hypocalcaemia and hypoparathyroidism after total thyroidectomy in children (≤18 years of age). METHODS: One hundred six children underwent total thyroidectomy. Patient, operative and outcome data were collected and analyzed. RESULTS: The indication for surgery was Graves' disease in 52 children (49.1%), Multiple Endocrine Neoplasia type-2 in 36 (33.9%), multinodular goiter in 3 (2.8%) and follicular/papillary thyroid carcinoma in 15 (14.2%). Neck dissection was performed in 23 children (18.9%). In 14 children (13.2%), autotransplantation was performed; in 31 (29.2%), ≥1 glands were found in the specimen. Hypocalcaemia within 24 h of thyroidectomy was observed in 63 children (59.4%) and 52 (49.3%) were discharged on supplements. Hypoparathyroidism at 6 months persisted in 23 children (21.7%). The ratios of all forms of calcium-related-morbidity were larger among children with less than four parathyroid glands remaining in situ: hypocalcaemia within 24 h of thyroidectomy (54.0% versus 47.5%; p = 0.01), hypoparathyroidism on discharge (64.4% versus 37.7%; p = 0.004) and long-term hypoparathyroidism (31.1% versus 14.8%; p = 0.04). CONCLUSION: The incidence of postoperative hypocalcaemia and hypoparathyroidism among children undergoing total thyroidectomy is considerable. The inability to preserve the parathyroid glands in situ during surgery seems an important factor. For optimal outcomes, the parathyroid glands should be preserved in situ. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: Level IV.

Using a Novel Diagnostic Nomogram to Differentiate Malignant from Benign Parathyroid Neoplasms.

We sought to develop an immunohistochemical (IHC) tool to support the diagnosis of parathyroid carcinoma (PC) and help differentiate it from atypical parathyroid neoplasms (atypical) and benign adenomas. Distinguishing PC from benign parathyroid neoplasms can be challenging. Many cases of PC are histopathologically borderline for definitive malignancy. Recently, individual IHC biomarkers have been evaluated to aid in discrimination between parathyroid neoplasms. PC, atypical parathyroid neoplasms, and parathyroid adenomas treated at our institution from 1997 to 2014 were studied retrospectively. IHC analysis was performed to evaluate parafibromin, retinoblastoma (RB), protein gene product 9.5 (PGP9.5), Ki67, galectin-3, and E-cadherin expression. Receiver operating characteristic (ROC) analysis and multivariable logistic regression model for combinations of biomarkers were evaluated to classify patients as PC or atypical/adenoma. A diagnostic nomogram using 5 biomarkers was created for PC. Sixty-three patients were evaluated. The percent staining of parafibromin (p < 0.0001), RB (p = 0.04), Ki67 (p = 0.02), PGP9.5 (p = 0.04), and Galectin-3 (p = 0.01) differed significantly in the three diagnostic groups. ROC analysis demonstrated that parafibromin had the best performance in discriminating PC from atypical/adenoma; area under the curve (AUC) was 81% (cutoff, 92.5%; sensitivity rate, 64%; specificity rate, 87%). We created a diagnostic nomogram using a combination of biomarkers; AUC was 84.9% (95% confidence interval, 73.4-96.4%). The optimism-adjusted AUC for this model was 80.5% (mean absolute error, 0.043). A diagnostic nomogram utilizing an immunoexpression, a combination of immunohistochemical biomarkers, can be used to help differentiate PC from other parathyroid neoplasms, thus potentially improving diagnostic classification.

Long-term quality of voice is usually acceptable after initial hoarseness caused by a thyroidectomy or a parathyroidectomy.

BACKGROUND: Vocal cord (VC) palsy following a thyroidectomy or parathyroidectomy can result in significant morbidity for the patient. We aimed to investigate the incidence of VC palsy in a tertiary referral Institution, track the management of these cases and record the long-term outcomes and VC recovery rates. METHODS: Retrospective review of all thyroidectomy/parathyroidectomy operations performed over 11 years. Patients with an unequivocal hoarse voice postoperatively were included. We analysed the patient's clinical characteristics and voice outcomes, operative, pathology and laryngoscopy reports during their follow-up. RESULTS: Ten patients fitted the inclusion criteria and were analysed. Median age at date of operation was 47.5 years (range, 16-81 years) and the M:F ratio was 1:2.3 (M:3, F:7). The median FU was 62.5 months (range, 12-144 months). The median hospital stay was 1.5 days (range, 1-87 days). There were 7 recurrent laryngeal nerve (RLN) injuries by manipulation, 1 case of RLN resection, 1 inadvertent division (with primary nerve repair) and 1 RLN was shaved off the thyroid. Long-term voice outcomes for the 7 patients with an RLN manipulation injury were: 3/7 patients had normal voice, 3/7 had moderate hoarseness and 1/7 had long-term hoarseness. The long-term voice outcome of the patient with RLN shaving off the thyroid gland was excellent while the 2 remaining patients (RLN resection and inadvertent division) needed 12 and 18 months respectively to achieve a normal quality of voice. Four out of the 10 patients had permanent VC palsy in the long-term and their voice outcomes varied: 1 patient had a normal voice, 2 patients had moderate hoarseness and 1 patient had persistent hoarseness. Only 1/10 patients did not show any voice improvement after 12 months. CONCLUSIONS: In the vast majority of cases post-operative hoarseness due to RLN palsy improves in the long-term, albeit voice may not return completely to normal.

Seventeen Cases of Primary Hyperparathyroidism in Pregnancy: A Call for Management Guidelines.

CONTEXT: The risks of primary hyperparathyroidism (pHPT) to pregnant women and their fetuses appear to increase commensurate with serum calcium levels. The management strategy for pHPT must be adapted in pregnancy and should reflect the severity of hypercalcemia. However, no guidelines exist to assist clinicians. METHODS: The experience of a high-volume multidisciplinary endocrine surgical service in treating a consecutive series of pregnant women with pHPT referred for parathyroidectomy is presented and data are compared with a nonpregnant cohort with pHPT. A review of pHPT and pregnancy outcomes in the literature is provided. RESULTS: Seventeen pregnant women and 247 age range-matched nonpregnant women with pHPT were referred for surgery over 11 years. Mean serum calcium level was higher in the pregnant cohort (2.89 vs 2.78 mmol/L; P = 0.03). Preoperative localization with ultrasound succeeded in eight pregnant women (47%) and sestamibi scanning did in two of six (33% imaged preconception), compared with 84 (34%) and 102 (42%) control subjects, respectively (not significant). Parathyroidectomy was performed under general anesthesia between 12 and 28 weeks' gestation with no adverse pregnancy outcomes resulting. Cure rate was 100% vs 96% in controls. CONCLUSION: pHPT in pregnancy is a threat to mother and child. Medical management may be appropriate in mild disease, but in moderate to severe disease, parathyroidectomy under general anesthesia in the second trimester is safe. Localization using ionizing radiation/MRI is unnecessary, because surgical intervention in a high-volume multidisciplinary setting has excellent outcomes. Guidelines on the topic would assist clinicians.

Comprehensive Genomic Characterization of Parathyroid Cancer Identifies Novel Candidate Driver Mutations and Core Pathways.

CONTEXT: Elucidating the genomic landscape of sporadic parathyroid carcinoma (PC) has been limited by low tumor incidence. OBJECTIVE: Identify driver mutations of sporadic PC and potential actionable pathways. METHODS: Patients undergoing surgical resection for sporadic PC between 1980 and 2016 at MD Anderson Cancer Center were identified. Patients with sporadic PC according to World Health Organization diagnostic criteria and with available formalin-fixed, paraffin-embedded (FFPE) PC tumor tissue were included and their clinical data analyzed to assess extent of disease. Patients with parathyroid tumors of uncertain malignancy or atypical parathyroid neoplasms were excluded. Thirty-one patients meeting diagnostic criteria had available tissue for analysis. FFPE PC tumors were subjected to DNA extraction and next-generation whole-exome sequencing. All variant calls are single-algorithm only. Twenty-nine samples passed quality assurance after DNA extraction. MAIN OUTCOME MEASURES: Somatic or private germline mutations present in sporadic PC and identification of pathways involved in tumorigenesis. RESULTS: We identified 35 genes with considerable mutational load; only eight genes were previously identified in other PC cohorts. These genes mediate critical processes, including chromosome organization, DNA repair, and cell cycle regulations. Gene mutations involved in MAPK signaling and immune response are also heavily implicated. These findings are limited by inherent molecular artifacts in FFPE tissue analysis and the absence of matched germline DNA. Additionally, variant calls are only single algorithm and may include false-positive/negative calls. CONCLUSION: We identified 33 candidate driver genes of sporadic PC, in addition to previously known driver genes CDC73 and MEN1.

Operation duration and adrenal gland size, but not BMI, are correlated with complication rate for posterior retroperitoneoscopic adrenalectomy for benign diseases.

BACKGROUND: We sought to determine whether obesity is correlated with complications after posterior retroperitoneoscopic adrenalectomy for benign diseases and to develop surrogate markers of abdominal fat in preoperative computed tomography. METHODS: We conducted a retrospective chart review of all patients who had undergone posterior retroperitoneoscopic adrenalectomy and preoperative computed tomography between January 1, 2008 and December 31, 2015. The cross-sectional components of fat assessed by computed tomography included total fat area, subcutaneous fat area, retroperitoneal fat area, and peritoneal fat area. The patients were grouped into 2 categories according to the absence or presence of a postoperative complication (the no-complications group and the complications group, respectively). RESULTS: Of 116 study patients, 20 patients (17%) had a postoperative complication. Operations of greater duration and smaller adrenal gland size were significantly correlated with complications both in univariate and multivariate analyses. Body mass index, total fat area, subcutaneous fat area, retroperitoneal fat area, peritoneal fat area, and distance of skin to muscle (in axial and sagittal images) correlated with complications in univariate analysis but not in multivariate analysis. In the no-complications group, the removed adrenal gland was larger than that removed in the complications group (6 vs 4 cm, respectively, P = .001), whereas the complications group had a greater operative duration (139 vs 104 min, respectively, P = .001) and a greater duration of hospital stay (3 days vs 1 day, respectively, P = .001). CONCLUSION: In this study, operations of greater duration and smaller adrenal gland size were better predictors of complications after posterior retroperitoneoscopic adrenalectomy for benign disease than measures of obesity, including body mass index.

Establishing a successful rat model of duodenal- jejunal bypass: A detailed guide.

Gastric bypass surgery, an operation that restricts the stomach and bypasses the duodenum and part of the jejunum, results in major improvement or remission of type 2 diabetes. Duodenual-jejunal bypass was developed by one of the authors (FR) as an experimental, stomach-sparing variant of gastric bypass surgery to investigate weight-independent mechanisms of surgical control of diabetes. Duodenual-jejunal bypass has been shown to improve various aspects of glucose homeostasis in rodents and in humans, thus providing an experimental model for investigating mechanisms of action of surgery and elusive aspects of gastrointestinal physiology. Performing duodenual-jejunal bypass in rodents, however, is associated with a steep learning curve. Here we report our experience with duodenual-jejunal bypass and provide practical tips for successful surgery in rats. Duodenual-jejunal bypass was performed on 50 lean rats as part of a study aimed at investigating the effect of the procedure on the physiologic mechanisms of glucose homeostasis. During the study, we have progressively refined details of anatomic exposure, technical aspects of duodeno-jejunostomy and peri-operative care. We analysed the role of such refinements in improving operative time and post-operative mortality. We found that refinement of exposure methods of the gastro-duodenal junction aimed at minimizing tension on small visceral vasculature, technical aspects of duodeno-jejunal anastomosis and peri-operative management played a major role in improving the survival rate and operative time. Overall, an experimental model of duodenual-jejunal bypass was successfully reproduced. Based on this experience, we describe here what we believe are the most important technical tips to reduce the learning curve for the procedure.

Metastasis to the thyroid gland from a tonsil squamous cell carcinoma.

We present a case of rare metastasis of tonsil squamous cell carcinoma to the thyroid gland, after 2 years of treatment without recurrence at the primary site. The patient had incidental finding of a nodule in the left lobe of the thyroid gland during a screening scan for his previous tonsil squamous cell carcinoma that was treated surgically 2 years ago. The patient was completely asymptomatic with normal thyroid function tests. After further investigations and discussion in the MDT meeting, the patient underwent left thyroid lobectomy as a definitive treatment for his thyroid metastasis.

Undiagnosed Primary Hyperparathyroidism and Recurrent Miscarriage: The First Prospective Pilot Study.

BACKGROUND: Primary hyperparathyroidism (pHPT) in pregnancy is reported to be associated with significant maternal and foetal complications and an up to threefold increase in the risk of miscarriage. However, the true incidence of pHPT in pregnancy, complete and miscarried, is unknown and there are no data on the prevalence of undiagnosed pHPT in recurrent miscarriage (RM) (≥3 consecutive miscarriages under 24-week gestation). This is the first prospective study aiming to establish the prevalence of undiagnosed pHPT in RM. METHODS: Following UK National ethics committee approval, women who had experienced 3 or more consecutive miscarriages were recruited from a nationwide RM clinic. Serum corrected calcium, phosphate, PTH and vitamin D were evaluated. Patients with raised serum calcium and/or PTH were recalled for confirmatory tests. Power calculations suggested that a minimum of 272 patients were required to demonstrate a clinically significant incidence of pHPT. RESULTS: Three hundred women were recruited, median age 35 years (range 19-42). Eleven patients had incomplete data, leaving 289 patients suitable for analysis; 50/289 patients (17%) with abnormal tests were recalled. The prevalence of vitamin D deficiency (<25 nmol/l) and insufficiency (25-75 nmol/l) was 8.7 and 67.8%, respectively. One patient was diagnosed with pHPT (0.34%) and underwent successful parathyroidectomy. CONCLUSIONS: The prevalence of undiagnosed pHPT (0.34%) in RM in this study appears to be many times greater than the 0.05% expected in this age group. The findings of this pilot study merit follow-up with a larger-scale study. Routine serum calcium estimation is not currently undertaken in RM and should be considered.

Gender differences in bone mineral density in patients with sporadic primary hyperparathyroidism.

CONTEXT: Primary hyperparathyroidism reduces bone mineral density, which increases the risk of fracture. OBJECTIVE: To investigate differences in bone mineral density and clinical characteristics after parathyroidectomy between men and women (premenopausal and postmenopausal) with sporadic primary hyperparathyroidism. DESIGN: This is a retrospective study of adult patients who underwent parathyroidectomy in a tertiary referral center from 1990 to 2013. PATIENTS: A total of 1529 patients underwent parathyroidectomy during the study period; 80 patients met the inclusion criteria. Of these, 24 were men and 56 were women (10 premenopausal and 46 postmenopausal). MEASUREMENTS: Demographics, preoperative and postoperative biochemical analysis, preoperative and postoperative T-scores, preoperative Z-scores, preoperative and postoperative absolute bone mineral density values, and percentage change in bone mineral density from baseline to 12 ± 6 months after parathyroidectomy in the lumbar spine, femoral neck, total hip and distal one-third of the nondominant radius. RESULTS: Preoperative 24-hour urinary calcium levels were significantly higher in men than in women overall (P = 0.02) and postmenopausal women (P = 0.01). Men had significantly lower preoperative Z-scores than women overall, premenopausal women and postmenopausal women. Men had greater percentage change of increase in bone mineral density in the femoral neck than did women overall (2.77%; P = 0.04) and postmenopausal women (2.98%; P = 0.03) 1 year after parathyroidectomy. CONCLUSIONS: From this study, men demonstrated a greater improvement of bone mineral density in the femoral neck from baseline after parathyroidectomy compared with women.

Genotype-phenotype pancreatic neuroendocrine tumor relationship in multiple endocrine neoplasia type 1 patients: A 23-year experience at a single institution.

BACKGROUND: The aim of this study was to investigate the genotype-phenotype relationship of pancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1 treated at our institution. METHODS: We conducted a retrospective chart review of all patients with multiple endocrine neoplasia type 1 treated at our center from January 1993 to December 2015. Presence of a pancreatic neuroendocrine tumor was determined based on imaging performed at any time from presentation to conclusion of follow-up. RESULTS: We reviewed 188 patients. The most common site of multiple endocrine neoplasia type 1 mutation was in exon 2 (34/188; 18%). Of 188 patients, 125 had a pancreatic neuroendocrine tumor (61%). Among all patients, 30 of 34 (88%) with an exon 2 mutation had a pancreatic neuroendocrine tumor compared with 95 of 154 (62%) with a mutation in exons 3-10 (P = .002). In the age group of 20 to 40 years, 8 of 9 patients with an exon 2 mutation had a pancreatic neuroendocrine tumor, compared with 24 of 52 patients (46%) with a mutation in exons 3-10 (P = .028). Patients with an exon 2 mutation had a greater frequency of pancreatic neuroendocrine tumor distant metastasis (53% vs 23%, P = .049). CONCLUSION: Young patients with multiple endocrine neoplasia type 1 and an exon 2 mutation appear to have a 2-fold greater risk for developing a pancreatic neuroendocrine tumor, and patients with an exon 2 mutation may be at greater risk for developing distant metastasis. Consideration should be given to more intensive screening and more liberal application of primary operative intervention in this potentially high-risk group.

Laparoscopic Adrenalectomy for Conn's Syndrome is Beneficial to Patients and is Cost Effective in England.

PURPOSE: Adrenalectomy has the potential to cure or improve the control of hypertension in patients with primary hyperaldosteronism due to unilateral adrenal adenoma (Conn's syndrome). This study assesses the patients' perception of, and costs associated with, laparoscopic adrenalectomy for Conn's syndrome. MATERIALS AND METHODS: Clinical, radiological, operative, and pathological data were collected on patients undergoing adrenalectomy for Conn's syndrome over 8-years period in a UK tertiary referral center. RESULTS: Thirty-eight patients (17M:21F, age 34-79 yrs, median 54 yrs) operated between Jan2005-Sept2012 had lateralization based on CT scans (n = 30) and/or MRI scans (n = 18) and confirmed on selective adrenal venous sampling (n = 25). Laparoscopic adrenalectomy was performed in all patients, with two cases requiring conversion to open operation. Median operative time was 105 min (range: 27-315). Costs were estimated as £19k for preoperative investigations, £20k for in-hospital stay, £53k for operating theatre use and £29k for disposable surgical instruments, with average £3499/patient (national tariff for adrenalectomy in 2015/2016 £3624). Follow-up at a mean of 30 months postoperatively using a visual analogue scale and a standardized questionnaire showed significantly improved quality of life (QoL) post-operatively. Majority of patients (85%) reported taking none or fewer anti-hypertensive medications (median reduction of 2 antihypertensive drugs). All patients stated that they would definitely have the operation again in preference to anti-hypertensive medications and they would recommend the operation to friends/relatives. CONCLUSIONS: Laparoscopic adrenalectomy for Conn's syndrome has a positive impact on hypertension control, leads to improved QoL and its costs are covered in the NHS financial model.

The diagnostic accuracy of neck ultrasound, 4D-Computed tomographyand sestamibi imaging in parathyroid carcinoma.

INTRODUCTION: Our aim was to investigate the accuracy of available imaging modalities for parathyroid carcinoma (PC) in our institution and to identify which imaging modality, or combination thereof, is optimal in preoperative determination of precise tumor location. METHODS: All operated PC patients in our institution between 2000 and 2015 that had at least one of the following in-house preoperative scans: neck ultrasonography (US), neck 4D-Computed Tomography (4DCT) and 99mTc Sestamibi SPECT/CT (MIBI). Sensitivity, specificity and accuracy of PC tumor localization were assessed individually and in combination. RESULTS: 20 patients fulfilled the inclusion criteria and were analysed. There were 18 US, 18 CT and 9 MIBI scans. The sensitivity and accuracy for tumor localisation of US was 80% (CI 56-94%) and 73% respectively, of 4DCT was 79% (CI 58-93%) and 82%, and of MIBI was 81% (CI 54-96%) and 78%. The sensitivity and accuracy of the combination of CT and MIBI was 94% (CI 73-100%) and 95% and for the combination of US, CT and MIBI was 100% (CI 72-100%) and 100% respectively. The wash-out of the PC lesions, expressed as a percentage change in Hounsfield Units from the arterial phase to early delayed phase was -9.29% and to the late delayed phase was -16.88% (n=11). CONCLUSIONS: The sensitivity of solitary preoperative imaging of PC patients, whether by US, CT or MIBI, is approximately 80%. Combinations of CT with MIBI and US increase the sensitivity to 95% or better. Combined preoperative imaging of patients with clinical possibility of PC is therefore recommended.

Clinical features and prognosis of thymic neuroendocrine tumours associated with multiple endocrine neoplasia type 1: A single-centre study, systematic review and meta-analysis.

OBJECTIVE: Thymic neuroendocrine tumour (TH-NET) accounts for almost 20% of multiple endocrine neoplasia type 1 (MEN1)-associated mortality. Identifying risk factors for the development of these rare tumours and prognostic factors for clinical outcomes will be helpful in clinical practice. DESIGN AND PATIENTS: We performed a retrospective analysis of patients treated for TH-NET associated with MEN1 in a single institution and meta-analysis of literature reports. We used a fixed effect model to pool results across studies to evaluate the prevalence, clinical features and prognosis. RESULTS: TH-NET was detected in 9 (7.4%) of 121 patients with MEN1 seen in our institution, and 5 (55.6%) were women. Seven additional studies were identified through a systematic review of the literature. The pool estimate of TH-NET prevalence was 3.7% (n = 99) in MEN1 (n = 2710), sex ratio was 79:20 (male vs female), and the median age at diagnosis was 43.0 years (range, 16.0-72.0 years). Forty-three patients died with a median survival time of 8.4 years. Older age at diagnosis (HR = 1.4, 95% CI = 1.0-1.8, P = .03), maximum tumour diameter (HR = 1.5, 95% CI = 1.0-2.3, P = .04) and presence of metastasis (HR = 1.6, 95% CI = 1.0-2.5, P = .04) were associated with worse outcome. A male predominance (91.9% vs 59.5%, P < .001) and history of smoking (59.0% vs 23.5%, P = .015) were more common in American/European series compared to Asian reports. CONCLUSION: TH-NET is a rare but fatal component of MEN1. Earlier detection of TH-NET in patients with MEN1 may be recommended which should theoretically result in better outcomes. Different genetic backgrounds (race) appear to result in clinical difference.

Postoperative local-regional radiation therapy in the treatment of parathyroid carcinoma: The MD Anderson experience of 35 years.

PURPOSE: We aimed to investigate outcomes of locoregional radiation therapy (XRT) in parathyroid carcinoma (PC) patients at a single institution over 35 years. METHODS AND MATERIALS: Retrospective review of PC patients who received postoperative locoregional XRT (1980-2015). Patients were grouped according to XRT after PC initial operation or after salvage surgery. All patients with a histopathological diagnosis of PC who had postoperative locoregional XRT at our institution were included. All patients with a histopathological diagnosis of uncertain malignancy, suggestive for malignancy, or atypical parathyroid tumors and those who had XRT outside our institution were excluded. RESULTS: Eight patients received XRT, 4 in each group, with a median follow-up of 12.5 years. The 4 patients who had XRT after initial surgery all had an oncologic operation, including ipsilateral thyroid lobectomy and central neck dissection, and all of these patients were disease-free at last follow-up. Of the 4 patients who received XRT after salvage surgery, only 1 remained disease free. There were no major complications/long-term side effects associated with XRT. CONCLUSIONS: XRT is well tolerated as postoperative locoregional treatment of patients with PC. Whether it is needed, and the timing of administering XRT, remains to be established.