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NCBI: db=pubmed; Term=adrenal tumor
Updated: 2 days 24 min ago

Preoperative risk factors of hemodynamic instability during laparoscopic adrenalectomy for pheochromocytoma.

Fri, 08/11/2017 - 04:01
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Preoperative risk factors of hemodynamic instability during laparoscopic adrenalectomy for pheochromocytoma.

Surg Endosc. 2016 Jul;30(7):2984-93

Authors: Gaujoux S, Bonnet S, Lentschener C, Thillois JM, Duboc D, Bertherat J, Samama CM, Dousset B

Abstract
BACKGROUND: Adrenalectomy for pheochromocytoma is considered to be a challenging procedure because of the risk of hemodynamic instability (HI), which is poorly defined and unpredictable. The objective of this retrospective study from a prospectively maintained database was to determine the predictive factors for perioperative HI, which is defined as a morbidity-related variable, in patients undergoing unilateral laparoscopic adrenalectomy (LA) for pheochromocytoma.
METHODS: A total of 149 patients with unilateral pheochromocytoma undergoing LA were included. First, HI was defined using independent hemodynamic variables associated with perioperative morbidity. Next, a multivariable logistic regression analysis was performed to determine the independent preoperative risk factors for HI.
RESULTS: There was no postoperative mortality, and the overall morbidity rate was 10.7 %. The use of a cumulative dose of norepinephrine >5 mg was the only independent hemodynamic predictive factor for postoperative complications; thus, this variable was used to define HI. A multivariate analysis revealed that a symptomatic high preoperative blood pressure (p = 0.003) and a ten-fold increase in urinary metanephrine and/or normetanephrine levels (p < 0.0001) were significant predictors of HI. When no predictive factors were present, the risk of HI and the postoperative morbidity were 1.5 and 4.3 %, respectively. However, when two predictive factors were present, the HI risk and the postoperative morbidity were 53.8 and 30.8 %, respectively.
CONCLUSION: Perioperative HI, defined as the need for a cumulative dose of norepinephrine >5 mg, is significantly associated with postoperative morbidity and can be predicted by symptomatic preoperative high blood pressure and above a ten-fold increase in urinary metanephrine and/or normetanephrine levels.

PMID: 26684206 [PubMed - indexed for MEDLINE]

Applicability of laparoscopic approach to the resection of large adrenal tumours: a retrospective cohort study on 200 patients.

Fri, 08/11/2017 - 04:01
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Applicability of laparoscopic approach to the resection of large adrenal tumours: a retrospective cohort study on 200 patients.

Surg Endosc. 2016 Aug;30(8):3532-40

Authors: Feo CV, Portinari M, Maestroni U, Del Rio P, Severi S, Viani L, Pravisani R, Soliani G, Zatelli MC, Ambrosio MR, Tong J, Terrosu G, Bresadola V

Abstract
BACKGROUND: Controversies exist in the best surgical approach (open vs. laparoscopy) to large adrenal tumours without peri-operative evidence of primary carcinoma, mainly due to possible capsular disruption of an unsuspected malignancy. In addition, intra-operative blood loss, conversion rate, operative time, and hospital stay may be increased with laparoscopy.
THE AIMS OF OUR STUDY WERE: (1) to compare clinical outcomes of laparoscopic adrenalectomy for large versus small adrenal tumours and (2) to identify risk factors associated with increased operative time and hospital stay in laparoscopic adrenalectomy.
METHODS: This is a multicentre retrospective cohort study in a large patient population (N = 200) who underwent laparoscopic adrenalectomy in 2004-2014 at three Italian academic hospitals. Patients were divided into two cohorts according to tumour size: "large" tumours were defined as ≥5 cm (N = 50) and "small" tumours as <5 cm (N = 150). Further analysis adopting a ≥8 cm (N = 15) cut-off size was performed.
RESULTS: The study groups were comparable in age and gender distribution as well as their tumour characteristics. The operative time (p = 0.671), conversion rate (p = 0.488), intra- (p = 0.876) and post-operative (p = 0.639) complications, and hospital stay (p = 0.229) were similar between groups. With a cut-off size ≥5 cm, the early study period (2004-2009), which included operators' learning curve, was associated with increased risk of longer operative time (HR 0.57; 95 % CI 0.40-0.82), while American Society of Anaesthesiology score ≥3 was associated with prolonged hospital stay (HR 0.67; 95 % CI 0.47-0.97). Tumour size ≥8 cm was associated with prolonged operative time (HR 0.47; 95 % CI 0.24-0.94).
CONCLUSIONS: Surgeons skilled in advanced laparoscopy and adrenal surgery can perform laparoscopic adrenalectomy safely in patients with ≥5-cm tumours with no increase in hospital stay, or conversion rate, although operative time may be increased for ≥8-cm tumours. Surgeon' experience, size ≥8 cm, and patient comorbidities have the largest impact on operative time and length of hospital stay in laparoscopic large adrenal tumour resection.

PMID: 26541739 [PubMed - indexed for MEDLINE]

Lactate dehydrogenase and creatine kinase as poor prognostic factors in lung cancer: A retrospective observational study.

Fri, 08/04/2017 - 01:15
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Lactate dehydrogenase and creatine kinase as poor prognostic factors in lung cancer: A retrospective observational study.

PLoS One. 2017;12(8):e0182168

Authors: Liu L, He Y, Ge G, Li L, Zhou P, Zhu Y, Tang H, Huang Y, Li W, Zhang L

Abstract
PURPOSE: Circulating molecules play important roles in lung cancer diagnosis. In addition, plasma lactate dehydrogenase (LDH) and creatine kinase (CK) have been shown to be closely related to tumor progression in breast cancer, prostate cancer, and colonel cancer. However, the relationships between LDH and CK levels with metastasis occurrence and the survival status of lung cancer patients remain unclear.
EXPERIMENTAL DESIGN: A total of 1142 lung cancer patients were enrolled in this study and were separated into negative or positive groups, according to the plasma levels of CK or LDH. Patients in both groups were assessed for clinical characteristics, metastasis occurrence, and survival status. The Cox regression model was then introduced to confirm whether CK and LDH could act as independent factors for predicting a poor prognosis.
RESULTS: The results indicated that CK had a close relationship with bone (p < 0.05) and lymph node (p < 0.05) metastases. In addition, LDH was strongly related with bone (p < 0.05), adrenal gland (p < 0.05), and lymph node (p < 0.05) metastases. CK and LDH were also correlated with the survival status of the lung cancer patients (all p < 0.001). According to specific histological classification analysis, it was found that CK was closely related to the survival status of adenocarcinoma (ADC) and squamous cell carcinoma (SCC) patients, while LDH was only correlated with that of ADC patients. Cox regression analysis confirmed that CK and LDH could act as independent factors for predicting a poor prognosis in ADC but not SCC patients.
CONCLUSIONS: For the first time, our study confirmed the role of CK in metastasis occurrence and the survival status of lung cancer patients. In addition, it also demonstrated that CK and LDH could be used as independent factors to predict a poor prognosis in ADC patients. The identification of CK and LDH will play important roles in lung cancer diagnosis and poor outcome prediction in the future.

PMID: 28767733 [PubMed - in process]

Retroperitoneal bronchogenic cyst resembling an adrenal tumor with high levels of serum carbohydrate antigen 19-9: A case report.

Fri, 08/04/2017 - 01:15
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Retroperitoneal bronchogenic cyst resembling an adrenal tumor with high levels of serum carbohydrate antigen 19-9: A case report.

Medicine (Baltimore). 2017 Aug;96(31):e7678

Authors: Wang M, He X, Qiu X, Tian C, Li J, Lv M

Abstract
RATIONALE: Retroperitoneal bronchogenic cysts without specific clinical manifestations are extremely rare and difficult to diagnose preoperatively and are easily misdiagnosed as left adrenal or pancreatic tumors.
PATIENT CONCERNS: A 48-year-old woman with the chief complaint of obscure epigastric pain for 1 month and with no other gastrointestinal symptoms and no significant medical history. The patient had signed informed consent for publication of this case report.
DIAGNOSIS: The serum level of carbohydrate antigen 19-9 (CA 19-9) in the patient was >1200 U/mL, which far exceeded the normal level of <37 U/mL. Computed tomography (CT) initially suggested the presence of an adrenal tumor. However, endoscopic ultrasound (EUS) showed that the adrenal gland had an intact capsule and that the mass originated in the retroperitoneal space and did not involve the paranephros.
INTERVENTIONS: Surgical resection was performed on the patient.
OUTCOMES: Histopathological examination demonstrated that the mass was a retroperitoneal bronchogenic cyst. At the 2-month postoperative follow-up, the level of CA 19-9 had returned to normal.
LESSONS: EUS appears to be superior to CT because it clearly delineated the mass from the surrounding structures of the retroperitoneal region. EUS-fine needle aspiration can be used for diagnosis or determining whether the mass is malignant or benign. To the best of our knowledge, retroperitoneal bronchogenic cysts with significantly elevated serum CA 19-9 have not been reported. Measurement of serum CA 19-9 may be helpful in the diagnosis of retroperitoneal bronchogenic cysts. However, this was a rare case, and the mechanism behind CA 19-9 elevation is not clear and needs further investigation.

PMID: 28767594 [PubMed - in process]

[Bone marrow involvement in primary mediastinal B-cell lymphoma].

Fri, 08/04/2017 - 01:15
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[Bone marrow involvement in primary mediastinal B-cell lymphoma].

Ter Arkh. 2017;89(7):65-68

Authors: Magomedova AU, Fastova EA, Kovrigina AM, Obukhova TN, Skidan NI, Mangasarova YK, Vorobyev AI, Kravchenko SK

Abstract
Primary mediastinal large B-cell lymphoma (PMBCL) is a distinct type of large B-cell lymphoma. In this type of the disease, the neoplastic process is located in the anterior and superior mediastinum, frequently with compression of the superior vena cava and with tumor invasion into the adjacent organs and tissues: the pericardium, lung, pleura, etc. Despite the fact that in PMBCL progression, there may be involvement of extranodal organs, such as the kidney, adrenal glands, liver, and central nervous system, bone marrow (BM) injury is generally absent. Since BM injury in patients with diffuse large B-cell lymphoma is an independent poor prognostic indicator, there is reason to believe that BM involvement in PMBCL affects the prognosis. These cases may need intensified induction therapy followed by autologous hematopoietic stem cell transplantation; and BM injury should be monitored during the therapy. The paper gives reports of clinical cases of bone marrow involvement in 2 PMBCL patients treated at the National Research Center for Hematology, Ministry of Health of the Russian Federation.

PMID: 28766543 [PubMed - in process]

Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review.

Fri, 08/04/2017 - 01:15
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Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review.

Diagn Pathol. 2017 Aug 02;12(1):57

Authors: Nonaka K, Matsuda Y, Okaniwa A, Kasajima A, Sasano H, Arai T

Abstract
BACKGROUND: Gangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pancreas is extremely rare. To date, only three cases have been reported and its clinical characteristics are largely unknown.
CASE PRESENTATION: A nodule located in the pancreatic head was incidentally detected in an asymptomatic 68-year-old woman. Computed tomography revealed 18-, 8-, and 12-mm masses in the pancreatic head, the pancreatic tail, and the left adrenal gland, respectively. Subsequent genetic examination revealed an absence of mutations in the MEN1 and VHL genes. Macroscopically, the tumor located in the pancreatic head was 22 mm in size and displayed an ill-circumscribed margin along with yellowish-white color. Microscopically, it was composed of three cell components: epithelioid cells, ganglion-like cells, and spindle cells, which led to the diagnosis of GP. The tumor was accompanied by a peripancreatic lymph node metastasis. The tumor in the pancreatic tail was histologically classified as a neuroendocrine tumor (NET) G1 (grade 1, WHO 2010), whereas the tumor in the left adrenal gland was identified as an adrenocortical adenoma. The patient was disease-free at the 12-month follow-up examination.
CONCLUSIONS: Pancreatic GP is associated with a higher incidence of metastasis and larger tumor size than duodenal GPs, suggesting that the primary organ of GP is an important prognostic factor.

PMID: 28764742 [PubMed - in process]

Effects of metformin on endometrial cancer: Systematic review and meta-analysis.

Fri, 08/04/2017 - 01:15
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Effects of metformin on endometrial cancer: Systematic review and meta-analysis.

Gynecol Oncol. 2017 Jul 28;:

Authors: Meireles CG, Pereira SA, Valadares LP, Rêgo DF, Simeoni LA, Guerra ENS, Lofrano-Porto A

Abstract
BACKGROUND: Endometrial cancer is one of the most common gynecological cancers, which is frequently preceded by atypical endometrial hyperplasia, a premalignant lesion. Metformin, an antidiabetic drug, has emerged as a new adjunctive strategy for different cancer types, including endometrial cancer. This systematic review and meta-analysis aimed to evaluate the effects of metformin in atypical endometrial hyperplasia and endometrial cancer patients.
METHODS: The search was conducted on January 2017 and the articles were collected in Cochrane, LILACS, PubMed, Scopus and Web of Science. A grey literature search was undertaken using Google SCHOLAR, ProQuest and Open Grey. Nineteen studies were included, which contained information about the following outcomes: reversal of atypical endometrial hyperplasia, cellular proliferation biomarkers expression and overall survival in metformin-users compared to non-users.
RESULTS: Metformin was associated with reversion of atypical endometrial hyperplasia to a normal endometrial, and with decreased cell proliferation biomarkers staining, from 51.94% (CI=36.23% to 67.46%) to 34.47% (CI=18.55% to 52.43%). However, there is a high heterogeneity among studies. Metformin-users endometrial cancer patients had a higher overall survival compared to non-metformin users and non-diabetic patients (HR=0.82; CI: 0.70-0.95; p=0.09, I(2)=40%).
CONCLUSION: Regardless the high heterogeneity of the analyzed studies, the present review suggests that adjunct metformin treatment may assist in the reversal of atypical endometrial hyperplasia to normal endometrial histology, in the reduction of cell proliferation biomarkers implicated in tumor progression, and in the improvement of overall survival in endometrial cancer. Further work on prospective controlled trials designed to address the effects of adjunct metformin on clinical outcomes is necessary for definite conclusions.

PMID: 28760367 [PubMed - as supplied by publisher]

[Carney triad: Report of one case].

Fri, 08/04/2017 - 01:15
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[Carney triad: Report of one case].

Rev Med Chil. 2017 Apr;145(4):533-537

Authors: Vega J, Navarro Subiabre J, Lovera Riquelme C, Opazo H, Santamarina M

Abstract
Carney described a disorder characterized by the presence of several uncommon tumors which were pulmonary chondromas, gastric sarcomas and extra-adrenal paragangliomas. We report a 14 year-old girl in whom multiple gastric tumors were discovered during a study of an iron deficiency anemia and was subjected to a partial gastrectomy. At 25 years of age, she developed several pulmonary chondromas and at 33 years, a mediastinal tumor with features of an extra-adrenal paraganglioma was found. At 35 years of age, a total gastrectomy was performed to remove a gastrointestinal stromal tumor with excision of peritoneal and lymph node metastasis. One year later, the patient died due to liver failure secondary to liver metastases.

PMID: 28749001 [PubMed - in process]

Fluorodeoxyglucose-positron emission tomography/computed tomography imaging features of colloid adenocarcinoma of the lung: a case report.

Fri, 08/04/2017 - 01:15
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Fluorodeoxyglucose-positron emission tomography/computed tomography imaging features of colloid adenocarcinoma of the lung: a case report.

J Med Case Rep. 2017 Jul 27;11(1):202

Authors: Wang Z, Yu M, Chen Y, Kong Y

Abstract
BACKGROUND: Colloid adenocarcinoma of the lung is a rare subtype of variants of invasive adenocarcinomas. We report the appearance of this unusual entity on (18)F-fluorodeoxyglucose positron emission tomography/computed tomography.
CASE PRESENTATION: A 60-year-old man of Chinese Han nationality coughed with a little white sputum for 1 month. Chest computed tomography showed multiple bilateral subpleural nodules and plaques accompanied by air bronchograms, which were most concentrated in the lower lobe of his right lung. Positron emission tomography indicated increased radioactivity uptake with a maximum standardized uptake value of 3.5. Positron emission tomography/computed tomography showed a soft tissue density lesion in his left adrenal gland with a maximum standardized uptake value of 4.1. The positron emission tomography/computed tomography appearance suggested a primary colloid adenocarcinoma in the lower lobe of his right lung accompanied by intrapulmonary and left adrenal gland metastases. The diagnostic rate of colloid adenocarcinoma can be increased by combining the anatomic and metabolic information of lesions.
CONCLUSIONS: The advantage of positron emission tomography/computed tomography in the diagnosis of colloid adenocarcinoma, as with other cancers, is the ability to locate extrapulmonary disease, facilitating clinical staging.

PMID: 28747211 [PubMed - in process]

Can Imaging Predict Subclinical Cortisol Secretion in Patients With Adrenal Adenomas? A CT Predictive Score.

Fri, 08/04/2017 - 01:15
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Can Imaging Predict Subclinical Cortisol Secretion in Patients With Adrenal Adenomas? A CT Predictive Score.

AJR Am J Roentgenol. 2017 Jul;209(1):122-129

Authors: Mosconi C, Vicennati V, Papadopoulos D, Dalmazi GD, Morselli-Labate AM, Golfieri R, Pasquali R

Abstract
OBJECTIVE: The aim of this study is to determine whether any correlation between CT findings and functional parameters exists to predict subclinical glucocorticoid secretion.
MATERIALS AND METHODS: This is a retrospective database study of 55 patients with incidentally discovered adenomas, investigated through CT with an adrenal protocol, assessing diameters and attenuation values on the unenhanced and contrast-enhanced phases. Patients underwent blood cortisol and corticotropin evaluation and overnight dexamethasone suppression test (DST), in accordance with clinical recommendations. Cortisol levels higher than 50 nmol/L after DST identified subclinical cortisol secretion. We identified 28 subjects with lipid-rich nonsecreting adenomas, nine with lipid-rich secreting adenomas, 11 with lipid-poor nonsecreting adenomas, and seven with lipid-poor secreting adenoma.
RESULTS: Cortisol levels after DST were significantly and positively related to mass diameters. At univariate analysis, maximum and minimum diameters and attenuation in the delayed phase were significantly related to the presence of secreting or nonsecreting adenoma; at multivariate analysis, only the minimum diameter and the attenuation in the venous phase entered the stepwise logistic regression. Similarly, minimum diameter and attenuation in the venous phase emerged also at the multivariate stepwise regression between radiologic parameters and cortisol levels after DST. The formula of the radiologic score computed by using the coefficients of the multivariate regression was as follows: (0.1914 × minimum diameter) + (0.0308 × enhanced attenuation). The diagnostic accuracy of this discriminatory score in differentiating secreting from nonsecreting adenomas was 84.9%, the sensitivity was 81.3%, and the specificity was 87.2%. Adenomas with scores greater than 7.59 were considered as secreting adenomas, and adenomas with scores less than 7.36 were considered as nonsecreting adenomas.
CONCLUSION: This study shows that imaging parameters can predict subclinical cortisol hypersecretion in patients with adrenal adenomas.

PMID: 28402131 [PubMed - indexed for MEDLINE]

Work up of incidental adrenal mass: state of the art.

Fri, 08/04/2017 - 01:15
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Work up of incidental adrenal mass: state of the art.

Urologia. 2016 Nov 18;83(4):179-185

Authors: Bada M, Castellan P, Tamburro FR, Berardinelli F, Neri F, Cindolo L, Schips L

Abstract
Due to the increasing use of radiological investigations, the detection of incidental adrenal masses has become even more frequent. Therefore, it is crucial to identify the nature of the adrenal mass in order to decide the type of treatment that should be undertaken. Toward this goal, biochemical tests are useful in order to assess catecholamines levels for the presence of a pheochromocytoma or cortisol excess in case of Cushing's syndrome. Furthermore, the dexamethasone suppression test and late-night salivary cortisol may be useful in measuring plasma cortisol, respectively, in the blood and urine. Hyperaldosteronism could be suspected in the presence of arterial hypertension. With regard to imaging modalities, the contrast washout and Hounsfield units estimation might play a role as indicators on computed tomography. In terms of treatment, a surgical approach is most suitable for a hyperfunctioning adrenal mass irrespective of size, and for nonfunctioning masses >4 cm. For indeterminate smaller lesions, with washout >50%, <10 Hounsfield Unit, nonfunctioning, benign-appearing, undergoing a follow-up in regular intervals is more appropriate in order to estimate mass growth. This paper summarizes recent findings on the management of incidental adrenal masses, with a special focus on the use of imaging, surgical management and follow-up modalities in improving patient outcomes.

PMID: 27338981 [PubMed - indexed for MEDLINE]

Frequency of Cushing's syndrome due to ACTH-secreting adrenal medullary lesions: a retrospective study over 10 years from a single center.

Fri, 08/04/2017 - 01:15
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Frequency of Cushing's syndrome due to ACTH-secreting adrenal medullary lesions: a retrospective study over 10 years from a single center.

Endocrine. 2017 Jan;55(1):296-302

Authors: Falhammar H, Calissendorff J, Höybye C

Abstract
Cushing's syndrome due to ectopic adrenocorticotropic hormone production from adrenal medullary lesions has occasionally been described. We retrospectively reviewed all 164 cases of Cushing's syndrome and 77 cases of pheochromocytomas during 10 years. Of all cases with Cushing's syndrome, only two cases (1.2 %) were due to ectopic adrenocorticotropic hormone production from adrenal medullary lesions (one case of pheochromocytoma and one case of adrenal medullary hyperplasia). Of all pheochromocytomas only the above-mentioned case (1.3 %) also gave rise to an ectopic adrenocorticotropic hormone syndrome. The clinical presentation of adrenocorticotropic hormone-secreting pheochromocytoma and adrenal medullary hyperplasia can be anything from mild to dramatic. These are rare conditions important to bear in mind in the workup of a patient with Cushing's syndrome or with pheochromocytoma. The identification of ectopic adrenocorticotropic hormone secretion from adrenal medullary lesions can be life-saving.

PMID: 27699708 [PubMed - indexed for MEDLINE]

Techniques to perform robotic left adrenalectomy in the obese patient.

Fri, 08/04/2017 - 01:15
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Techniques to perform robotic left adrenalectomy in the obese patient.

Surg Endosc. 2017 Feb;31(2):950

Authors: Maker AV, Maker VK

Abstract
INTRODUCTION: Minimally invasive adrenalectomy may be associated with reduction in postoperative pain, morbidity, and length of stay and, as a result, has become a preferred approach for many adrenal tumors. Left-sided adrenal tumors, however, are particularly challenging to address in the morbidly obese patient due to difficulties in maintaining exposure and dissection. The robotic platform offers instruments with greater degrees of freedom that aid in retraction and dissection, especially of the adrenal vein, but fixed patient positioning and the large distance needed between patient ports to avoid arm collisions can be restrictive in patients with a large amount of retroperitoneal fat and small working space.
METHODS/RESULTS: We demonstrate robotic left adrenalectomy (RLA) in a consecutive series of patients with a mean weight of 99 kg and mean BMI of 36. Techniques to safely and efficiently perform RLA in obese patients are stepwise demonstrated, including (1) Patient positioning, (2) Management of the pannus, (3) Customized port placement, (4) Medial retraction of the pancreas, (5) Finding the left adrenal vein, and (6) Management of bleeding. Intraoperative videos from multiple patients also show surgical pitfalls, examples of poor port placement, arm collisions, alternative approaches to the vein, and techniques to control unexpected bleeding. All patients in the series underwent successful RLA with negative margins, no major intra- or postoperative complications, and discharge on POD 1-2.
CONCLUSION: Though poor exposure due to patient body habitus is a relative contraindication, even large left-sided adrenal tumors can be safely approached robotically while adhering to oncologic principles, as is demonstrated in this video.

PMID: 27387175 [PubMed - indexed for MEDLINE]

Utility of FDG-PET imaging in screening for succinate dehydrogenase B and D mutation-related lesions.

Fri, 08/04/2017 - 01:15
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Utility of FDG-PET imaging in screening for succinate dehydrogenase B and D mutation-related lesions.

Clin Endocrinol (Oxf). 2016 Aug;85(2):172-9

Authors: Kornaczewski ER, Pointon OP, Burgess JR

Abstract
OBJECTIVE: Mutations of the genes encoding succinate dehydrogenase B and D (SDHB, SDHD) are associated with highly penetrant phenotypes, including paragangliomas and phaeochromocytomas. Patients with these mutations require lifelong surveillance; however, there is currently ambiguity regarding the optimal screening regimen. We sought to determine the utility of fluorodeoxyglucose (18F) positron emission tomography (18F-FDG PET) imaging, compared to other modalities for detecting SDHB and SDHD mutation-related lesions.
DESIGN: A retrospective audit of patients with SDHB or SDHD mutation.
PATIENTS: All adult patients with confirmed SDHB and SDHD mutations who underwent 18F-FDG PET/CT at our institution between 1 July 2011 and 30 May 2015.
MEASUREMENTS: 18F-FDG PET/computed tomography (CT) performed during surveillance of patients with SDHB and SDHD mutations. Lesion numbers and locations detected by 18F-FDG PET were compared to those identified on the CT component, as well as other imaging modalities and histology when available.
RESULTS: Thirty-one 18F-FDG PET/CT studies were completed on 22 patients. For SDHB (20 patients), there were five positive and 21 negative studies. There were no false-negative 18F-FDG PET studies. Positive 18F-FDG PET findings correlated with magnetic resonance imaging (MRI), CT and [68 Ga]-DOTA(0)-Tyr(3)-octreotate (68 Ga DOTATATE PET/CT) imaging with no missed lesions; the only potential false-positive result relating to nonspecific postoperative changes (sensitivity 100·0%, specificity 95·5%). For SDHD (two patients), lesions were detected on 18F-FDG PET and correlated with other imaging in three of five studies. Metastatic lesions were incompletely visualized on 18F-FDG PET but were detected on the noncontrast fusion CT.
CONCLUSIONS: 18F-FDG PET/CT is suitable for detecting SDHB and SDHD mutation-related lesions and may be considered effective for periodic surveillance of patients with these mutations.

PMID: 26776272 [PubMed - indexed for MEDLINE]

Laparoscopic radical 'no-touch' left pancreatosplenectomy for pancreatic ductal adenocarcinoma: technique and results.

Fri, 08/04/2017 - 01:15
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Laparoscopic radical 'no-touch' left pancreatosplenectomy for pancreatic ductal adenocarcinoma: technique and results.

Surg Endosc. 2016 Sep;30(9):3830-8

Authors: Abu Hilal M, Richardson JR, de Rooij T, Dimovska E, Al-Saati H, Besselink MG

Abstract
BACKGROUND: Laparoscopic left pancreatectomy has been well described for benign pancreatic lesions, but its role in pancreatic adenocarcinoma remains open to debate. We report our results adopting a laparoscopic technique that obeys established oncologic principles of open distal pancreatosplenectomy.
METHODS: This is a post hoc analysis of a prospectively kept database of 135 consecutive patients undergoing laparoscopic left pancreatectomy, performed across two sites in the UK and the Netherlands (07/2007-07/2015 Southampton and 10/2013-07/2015 Amsterdam). Primary outcomes were resection margin and lymph node retrieval. Secondary endpoints were other perioperative outcomes, including post-operative pancreatic fistula. Definition of radical resection was distance tumour to resection margin >1 mm. All patients underwent 'laparoscopic radical left pancreatosplenectomy' (LRLP) which involves 'hanging' the pancreas including Gerota's fascia, followed by clockwise dissection, including formal lymphadenectomy.
RESULTS: LRLP for pancreatic adenocarcinoma was performed in 25 patients. Seven of the 25 patients (28 %) had extended resections, including the adrenal gland (n = 3), duodenojejunal flexure (n = 2) or transverse mesocolon (n = 3). Mean age was 68 years (54-81). Conversion rate was 0 %, mean operative time 240 min and mean blood loss 340 ml. Median intensive/high care and hospital stay were 1 and 5 days, respectively. Clavien-Dindo score 3+ complication rate was 12 % and ISGPF grade B/C pancreatic fistula rate 28 %; 90-day (or in-hospital) mortality was 0 %. The pancreatic resection margin was clear in all patients, and the posterior margin was involved (<1 mm) in 6 patients, meaning an overall R0 resection rate of 76 %. No resection margin was microscopically involved. Median nodal sample was 15 nodes (3-26). With an average follow-up of 17.2 months, 1-year survival was 88 %.
CONCLUSIONS: A standardised laparoscopic approach to pancreatic adenocarcinoma in the left pancreas can be adopted safely. Our study shows that these results can be reproduced across multiple sites using the same technique.

PMID: 26675941 [PubMed - indexed for MEDLINE]

Sialadenitis after radioiodine therapy. Analysis of factors that influence the response to medical treatment.

Fri, 08/04/2017 - 01:15
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Sialadenitis after radioiodine therapy. Analysis of factors that influence the response to medical treatment.

Endocrinol Nutr. 2015 Dec;62(10):493-8

Authors: Geres AE, Mereshian PS, Fernández S, Rey Caro DG, Castro R, Podio R, Ojeda S

Abstract
OBJECTIVES: To assess the incidence of 131I-induced sialadenitis (SD) in patients with differentiated thyroid cancer (DTC), to analyze clinical and other factors related to metabolic radiotherapy that may predict the lack of response to conventional medical therapy (CMT), and to determine the effectiveness of intraductal steroid instillation in patients failing CMT.
MATERIAL AND METHODS: Fifty-two patients with DTC, 45 females (86.5%) and 7 males (13.5%) with a mean age of 44.21±13.3 years (r=17-74) who received ablation therapy with 131I after total thyroidectomy. Patients with diseases and/or medication causing xerostomia were excluded. Patients underwent salivary gland scintigraphy with 99Tc (10mCi).
RESULTS: Eighteen patients (34.62%) had SD and received antibiotics, antispasmodics, and oral steroids for 15 days. They were divided into two groups: responders to medical therapy (n=12, age 44.3±14.4 years, 2 men [17%], 10 women [83%], cumulative dose 225±167.1 mCi) and non-responders to medical treatment, who underwent steroid instillation into the Stensen's duct (n=6 [33%], 2 men [33%], 4 women [67%], age 50±13.8 years, cumulative dose 138.3±61.7 mCi). Scintigraphy showed damage to the parotid and submaxillary glands.
CONCLUSION: Incidence of 131I-induced sialadenitis was similar to that reported by other authors. Age, mean cumulative dose of 131I, and involvement of parotid and submaxillary glands did not condition response to CMT; however, male sex was a conditioning factor. Symptom persistence for more than 15 days makes instillation into the Stensen's duct advisable. This is an effective and safe method to avoid surgical excision of salivary glands.

PMID: 26459118 [PubMed - indexed for MEDLINE]

Primary adrenal leiomyosarcoma treated by laparoscopic adrenalectomy.

Fri, 08/04/2017 - 01:15
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Primary adrenal leiomyosarcoma treated by laparoscopic adrenalectomy.

Endocrinol Nutr. 2015 Nov;62(9):472-3

Authors: Quildrian S, Califano I, Carrizo F, Daffinoti A, Calónico N

PMID: 26215892 [PubMed - indexed for MEDLINE]

Black adrenal adenoma causing Cushing's syndrome: 40 years ago and today.

Fri, 08/04/2017 - 01:15
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Black adrenal adenoma causing Cushing's syndrome: 40 years ago and today.

Endocrinol Nutr. 2015 Nov;62(9):466-9

Authors: Yu R, Wei M, Fan X, Ellis RR, Braunstein GD

PMID: 26088906 [PubMed - indexed for MEDLINE]

A Case Report of Cystic Pheochromocytoma.

Fri, 07/28/2017 - 00:54
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A Case Report of Cystic Pheochromocytoma.

Am J Case Rep. 2017 Jul 25;18:826-829

Authors: Junejo SZ, Tuli S, Heimann DM, Sachmechi I, Reich D

Abstract
BACKGROUND Pheochromocytoma is a rare catecholamine-producing tumor with an estimated incidence of less than 0.1% in the global population. We present a case of cystic pheochromocytoma that was diagnosed as an incidental finding. The patient presented with abdominal pain and had a history of hypertension. CASE REPORT A 64-year-old man with hypertension presented with a clinical history of intermittent abdominal pain for one year. He denied sweating, palpitations, headache or back pain. He was found to have an elevated blood pressure of 170/90 and no palpable abdominal mass. Contrast-enhanced computed tomography (CT) imaging of the abdomen and pelvis were performed that showed cystic mass measuring 9 cm in diameter arising from the left adrenal gland with contrast-enhancing mural nodules. Magnetic resonance imaging (MRI) confirmed the cystic nature of the mass. Laboratory analysis showed an elevated plasma normetanephrine (NMN) of 1,087 pg/ml and metanephrine (MN) of 372 pg/ml; 24-hour urine showed elevated levels of NMN and MN, 3,002 mg/24 h and 1,596 mg/24 h, respectively. Given the laboratory and radiologic findings, a diagnosis of cystic pheochromocytoma was made. After controlling blood pressure with the alpha-blocker, doxazosin, the patient was hydrated and scheduled for an elective adrenalectomy. The histopathology of the excised adrenal gland was consistent with a cystic pheochromocytoma. CONCLUSIONS Cystic pheochromocytoma is a very rare tumor that may present without symptoms. The clinical course of cystic pheochromocytoma is similar to that of solid pheochromocytoma. Early surgical intervention is recommended, following blood pressure control with an alpha-blocker, and adequate hydration.

PMID: 28740068 [PubMed - in process]

Late onset asymptomatic pancreatic neuroendocrine tumor - A case report on the phenotypic expansion for MEN1.

Fri, 07/28/2017 - 00:54
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Late onset asymptomatic pancreatic neuroendocrine tumor - A case report on the phenotypic expansion for MEN1.

Hered Cancer Clin Pract. 2017;15:10

Authors: Kaiwar C, Macklin SK, Gass JM, Jackson J, Klee EW, Hines SL, Stauffer JA, Atwal PS

Abstract
BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary cancer syndrome associated with several endocrine as well as non-endocrine tumors and is caused by mutations in the MEN1 gene. Primary hyperparathyroidism affects the majority of MEN1 individuals by age 50 years. Additionally, MEN1 mutations trigger familial isolated hyperparathyroidism. We describe a seemingly unaffected 76-year-old female who presented to our Genetics Clinic with a family history of primary hyperparathyroidism and the identification of a pathogenic MEN1 variant.
CASE PRESENTATION: The patient was a 76 year-old woman who appeared to be unaffected. She had a family history of a known MEN1 pathogenic variant. Molecular testing for the known MEN1 mutation c.1A > G, as well as, biochemical testing, MRI of the brain and abdomen were all performed using standard methods. Molecular testing revealed our patient possessed the MEN1 pathogenic variant previously identified in her two offspring. Physical exam revealed red facial papules with onset in her seventies, involving her cheeks, nose and upper lip. Formerly, she was diagnosed with rosacea by a dermatologist and noted no improvement with treatment. Clinically, these lesions appeared to be facial angiofibromas. Brain MRI was normal. However, an MRI of her abdomen revealed a 1.5 cm lesion at the tail of the pancreas with normal adrenal glands. Glucagon was mildly elevated and pancreatic polypeptide was nearly seven times the upper limit of the normal range. The patient underwent spleen sparing distal pancreatectomy and subsequent pathology was consistent with a well-differentiated pancreatic neuroendocrine tumor (pNET).
CONCLUSIONS: Age-related penetrance and variable expressivity are well documented in families with MEN1. It is thought that nearly all individuals with MEN1 manifest disease by age 40. We present a case of late-onset MEN1 in the absence of the most common feature, primary hyperparathyroidism, but with the presence of a pNET and cutaneous findings. This family expands the phenotype associated with the c.1A > G pathogenic variant and highlights the importance of providing comprehensive assessment of MEN1 mutation carriers in families that at first blush may appear to have isolated hyperparathyroidism.

PMID: 28736585 [PubMed]

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