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1.
J Surg Oncol ; 120(8): 1450-1455, 2019 Dec.
Article in English | MEDLINE | ID: mdl-31733070

ABSTRACT

BACKGROUND: Recurrent adrenocortical carcinoma (ACC) has a poor prognosis with minimal clinical and biochemical factors to guide management. The aim of this study was to evaluate the prognostic significance of systemic inflammatory response in patients with recurrent ACC. METHODS: Patients who underwent resection for recurrent ACC were retrospectively analyzed. Preoperative neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, lymphocyte-to-monocyte ratio (LMR), and mean platelet volume were calculated. RESULTS: Twenty-five patients (age at operation 52.2 ± 9.5 years) were identified. We observed a statistically significant shorter disease-specific survival (DSS) in patients with LMR less than 4 (41 ± 7.4 months vs 71 ± 12.3, P = .023) and male sex (26.6 ± 4.2 months vs 57.6 ± 9.5 months, P = .079), while time-to-recurrence (TTR) less than 12 months (40 ± 7.7 months vs 70.3 ± 13.1 months, P = .059) had a trend on univariate analysis for worse DSS. On multivariable analysis, LMR < 4 (hazard ratio [HR] 4.18; 95% confidence interval [CI]: 1.18-14.76; P = .027) and TTR less than 12 months (HR 2.77 95% CI: 1-7.62; P = .049) were found to be significantly associated with worse DSS. CONCLUSION: Preoperative LMR greater than 4 and TTR greater than 12 months are associated with longer DSS. Patients with LMR greater than 4 and TTR greater than 12 months may benefit from a more aggressive therapeutic approach and may require less frequent surveillance.


Subject(s)
Adrenal Cortex Neoplasms/mortality , Adrenocortical Carcinoma/mortality , Lymphocyte Count , Monocytes/metabolism , Neoplasm Recurrence, Local/mortality , Adrenal Cortex Neoplasms/pathology , Adrenal Cortex Neoplasms/surgery , Adrenocortical Carcinoma/pathology , Adrenocortical Carcinoma/surgery , Adult , Biomarkers/blood , Cell Count , Female , Humans , Male , Middle Aged , Neoplasm Recurrence, Local/surgery , Preoperative Period , Prognosis , Retrospective Studies , Time Factors
2.
Horm Metab Res ; 51(7): 470-482, 2019 Jul.
Article in English | MEDLINE | ID: mdl-31307109

ABSTRACT

Since Felix Fränkel's account of pheochromocytoma in 1886, great discoveries and vast advancements in the diagnosis, genetics, anatomical and functional imaging techniques, and surgical management of pheochromcytoma and paraganglioma (P-PGL) have been made. The improved insight in the pathophysiology of P-PGL and more accurate detection methods enable physicians to tailor the treatment plan to an individual based on the genetic profile and tumor behavior. This review will cover briefly the clinical features, diagnosis, genetic mutations, and imaging modalities that are used to guide current surgical management of these rare and interesting endocrinopathies.


Subject(s)
Adrenal Gland Neoplasms , Pheochromocytoma , Precision Medicine , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/pathology , Adrenal Gland Neoplasms/surgery , Humans , Mutation , Pheochromocytoma/diagnosis , Pheochromocytoma/genetics , Pheochromocytoma/pathology , Pheochromocytoma/surgery
3.
Nucl Med Mol Imaging ; 53(2): 144-147, 2019 Apr.
Article in English | MEDLINE | ID: mdl-31057686

ABSTRACT

A 36-year-old male patient initially presented with hypertension, tinnitus, bilateral carotid masses, a right jugular foramen, and a periaortic arch mass with an elevated plasma dopamine level but an otherwise normal biochemical profile. On surveillance MRI 4 years after initial presentation, he was found to have a 2.2-cm T2 hyperintense lesion with arterial enhancement adjacent to the gallbladder, which demonstrated avidity on 68Ga-DOTATATE PET/CT and retrospectively on 18F-FDOPA PET/CT but was non-avid on 18F-FDG PET/CT. Biochemical work-up including plasma catecholamines, metanephrines, and chromogranin A levels were found to be within normal limits. This lesion was surgically resected and was confirmed to be a paraganglioma (PGL) originating from the gallbladder wall on histopathology. Pheochromocytoma (PHEO) and PGL are rare tumors of the autonomic nervous system. Succinate dehydrogenase subunit D (SDHD) pathogenic variants of the succinate dehydrogenase complex are usually involved in parasympathetic, extra-adrenal, multifocal head, and neck PGLs. We report an unusual location of PGL in the gallbladder associated with SDHD mutation which could present as a potential pitfall on 18F-FDOPA PET/CT as its normal excretion occurs through biliary system and gallbladder. This case highlights the superiority of 68Ga-DOTATATE in comparison to 18F-FDOPA and 18F-FDG in the detection of SDHD-related parasympathetic PGL. ClinicalTrials.gov Identifier: NCT00004847.

5.
Ann Surg ; 269(4): 741-747, 2019 04.
Article in English | MEDLINE | ID: mdl-29334561

ABSTRACT

BACKGROUND: Fluorodeoxyglucose (F-FDG) positron emission tomography/computed tomography (PET/CT) imaging is recommended in patients with metastatic pheochromocytoma (PC) and paraganglioma (PGL). There are no data on whether routine preoperative F-FDG PET/CT in all patients with PC/PGL impacts surgical management. OBJECTIVE: The aim of this study was to determine whether routine preoperative F-FDG PET/CT imaging affects the surgical management of patients with PC/PGLs. METHODS: We analyzed clinical, biochemical, genetic, and anatomic imaging data in 93 consecutive patients with PC/PGL who collectively underwent a total of 100 operations and who had preoperative F-FDG PET/CT imaging. RESULTS: Of 100 operations, preoperative F-FDG PET/CT showed additional lesions compared to anatomic imaging in 15 cases. These patients were more likely to undergo an open surgical approach (P < 0.05). Presence of genetic mutation, redo operations, sex, age, or tumor size had no significant association with finding additional lesions on F-FDG PET/CT. CONCLUSIONS: Additional lesions detected on preoperative F-FDG-PET/CT imaging have an impact on the surgical approach in patients with PC/PGLs. Therefore, surgeons should routinely obtain F-FDG-PET/CT imaging in patients with PC/PGL to allow for a more precise surgical intervention.


Subject(s)
Adrenal Gland Neoplasms/diagnostic imaging , Adrenal Gland Neoplasms/surgery , Adrenalectomy/methods , Fluorodeoxyglucose F18/therapeutic use , Paraganglioma/diagnostic imaging , Paraganglioma/surgery , Pheochromocytoma/diagnostic imaging , Pheochromocytoma/surgery , Positron Emission Tomography Computed Tomography , Radiopharmaceuticals/therapeutic use , Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Paraganglioma/secondary , Pheochromocytoma/secondary , Precision Medicine , Preoperative Care , Prospective Studies , Young Adult
6.
JAMA Surg ; 154(1): 40-45, 2019 01 01.
Article in English | MEDLINE | ID: mdl-30267071

ABSTRACT

Importance: Neuroendocrine tumors (NETs) express somatostatin receptors, which can be targeted with radiolabeled peptides. In a variety of solid tumors, radioguided surgery (RGS) has been used to guide surgical resection. Gallium 68 (68Ga) dota peptides have been shown to be more accurate than other radioisotopes for detecting NETs. A pilot study previously demonstrated the feasibility and safety of 68Ga-dotatate RGS for patients with NETs. Objective: To evaluate what intraoperative techniques and thresholds define positive lesions that warrant resection during 68Ga-dotatate RGS. Design, Setting, and Participants: This prospective cohort study, conducted between October 23, 2013, and February 14, 2018, included 44 patients with NETs who underwent 68Ga-dotatate RGS. Intervention: Gallium 68-dotatate RGS. Main Outcomes and Measures: The in vivo and ex vivo tumor to background ratio (TBR) was assessed for resected lesions and correlated with the histopathologic findings. Results: Forty-four patients (22 women and 22 men; mean [SD] age, 51.0 [13.7] years) had 133 lesions detected on preoperative imaging scans, with a diagnosis of a pancreatic NET (19 of 44 [43%]), gastrointestinal NET (22 of 44 [50%]), and pheochromocytoma or paraganglioma (3 of 44 [7%]). The TBR was obtained by normalizing to the omentum (106 of 133 [79.7%]) or other solid organs (27 of 133 [20.3%]). The omentum had a significantly lower mean (SD) count than other solid organs for background count activity 3 hours after injection (22.1 [17.0] vs 34.5 [39.0]; P < .001). The lesions containing NETs had a higher TBR than those that did not contain NETs (18.9 vs 4.4; P < .001). On a receiver operating characteristic curve analysis, a TBR of 2.5 had a sensitivity of 90% and a specificity of 25%, and a TBR of 16 had a sensitivity of 54% and a specificity of 81%. Conclusions and Relevance: A TBR of 2.5 or greater is a highly sensitive threshold for indicating a lesion to be consistent with a NET on histologic findings and thus warranting surgical resection. The omentum should be used as the background count activity for 68Ga-dotatate RGS for patients with abdominal NETs.


Subject(s)
Gastrointestinal Neoplasms/surgery , Neuroendocrine Tumors/surgery , Pancreatic Neoplasms/surgery , Female , Humans , Male , Middle Aged , Organometallic Compounds , Paraganglioma/surgery , Pheochromocytoma/surgery , Positron Emission Tomography Computed Tomography/methods , Positron-Emission Tomography/methods , Prospective Studies , Radiopharmaceuticals , Surgery, Computer-Assisted/methods , Treatment Outcome
7.
Surgery ; 165(1): 50-57, 2019 01.
Article in English | MEDLINE | ID: mdl-30327187

ABSTRACT

BACKGROUND: There are conflicting reports on whether familial nonmedullary thyroid cancer is more aggressive than sporadic nonmedullary thyroid cancer. Our aim was to determine if the clinical and pathologic characteristics of familial nonmedullary thyroid cancer are different than nonmedullary thyroid cancer. METHODS: We compared patients with familial nonmedullary thyroid cancer to a cohort of 53,571 nonmedullary thyroid cancer patients from the Surveillance, Epidemiology, and End Results database. RESULTS: A total of 78 patients with familial nonmedullary thyroid cancer from 31 kindreds presented at a younger age (P = .04) and had a greater rate of T1 disease (P = .019), lymph node metastasis (P = .002), and the classic variant of papillary thyroid cancer on histology (P < .001) compared with the Surveillance, Epidemiology, and End Results cohort. Patients with ≥3 affected family members presented at a younger age (P = .04), had a lesser female-to-male ratio (P = .04), and had a greater rate of lymph node metastasis (P = .009). Compared with the Surveillance, Epidemiology, and End Results cohort, we found a higher prevalence of lymph node metastasis in familial nonmedullary thyroid cancer index cases (P = .003) but not in those diagnosed by screening ultrasonography (P = .58). CONCLUSION: Patients with familial nonmedullary thyroid cancer present at a younger age and have a greater rate of lymph node metastasis. The treatment for familial nonmedullary thyroid cancer should be more aggressive in patients who present clinically and in those who have ≥3 first-degree relatives affected.


Subject(s)
Carcinoma, Medullary/congenital , Multiple Endocrine Neoplasia Type 2a/pathology , Thyroid Cancer, Papillary/pathology , Thyroid Neoplasms/pathology , Adult , Age Distribution , Carcinoma, Medullary/epidemiology , Carcinoma, Medullary/pathology , Carcinoma, Medullary/surgery , Cohort Studies , Female , Humans , Lymphatic Metastasis , Male , Middle Aged , Multiple Endocrine Neoplasia Type 2a/epidemiology , Multiple Endocrine Neoplasia Type 2a/surgery , Neck Dissection/statistics & numerical data , SEER Program , Sex Distribution , Thyroid Cancer, Papillary/epidemiology , Thyroid Cancer, Papillary/surgery , Thyroid Neoplasms/epidemiology , Thyroid Neoplasms/surgery , Thyroidectomy/statistics & numerical data , United States/epidemiology
8.
Gland Surg ; 7(Suppl 1): S86-S88, 2018 Aug.
Article in English | MEDLINE | ID: mdl-30178784
9.
Langenbecks Arch Surg ; 403(5): 599-606, 2018 Aug.
Article in English | MEDLINE | ID: mdl-29855800

ABSTRACT

BACKGROUND: Lymph node metastasis is not common among patients with gastrointestinal stromal tumors (GISTs) and its prognostic value is controversial. The purposes of this study are to identify predictors of lymph node metastasis and determine its prognostic associations. METHODS: A retrospective analysis of the surveillance, epidemiology and end results (SEER) database was performed. Patients with GISTs that underwent surgery and pathologic nodal staging were identified. Logistic regression and Cox regression were performed to identify independent predictors and prognostic factors, respectively. RESULTS: Of 1430 patients (age: 61.5 ± 14.5 years, 52% males), 140 (9.8%) had lymph node metastasis. On multivariable analysis, distant metastasis was the only independent predictor of lymph node metastasis (OR 4.95, 95% CI: 2.43-10.08, p < 0.001). In the entire cohort, lymph node metastasis did not reflect a worse overall survival (OS, HR 1.12, 95% CI: 0.49-2.58, p = 0.794) or disease-specific survival (DSS, HR 0.95, 95% CI: 0.31-2.88, p = 0.924), but was an independent predictor of worse OS in 51 patients (25.4% of 201 patients) who presented with both lymph node metastasis and synchronous distant metastasis (HR 2, 95% CI: 1.25-3.21, p = 0.004). Lymph node metastasis was also independently associated with worse survival among patients with small intestinal (OS: HR 1.88, 95% CI: 1.15-3.1, p = 0.013) and colorectal tumors (OS: HR 3.41, 95% CI: 1.56-7.46, p = 0.002, DSS: HR 3.58, 95% CI: 1.27-10.06, p = 0.016). CONCLUSIONS: Metastatic disease is an independent predictor of lymph node metastasis in patients with GISTs. Lymph node metastasis is also associated with worse overall survival in patients with metastatic GISTs.


Subject(s)
Gastrointestinal Neoplasms/pathology , Gastrointestinal Stromal Tumors/secondary , Aged , Female , Gastrointestinal Neoplasms/mortality , Gastrointestinal Stromal Tumors/mortality , Humans , Lymphatic Metastasis , Male , Middle Aged , Neoplasm Staging , Regression Analysis , Retrospective Studies , Risk Factors , SEER Program , Survival Rate
10.
Endocrine ; 61(3): 422-427, 2018 09.
Article in English | MEDLINE | ID: mdl-29923016

ABSTRACT

PURPOSE: It has been proposed that rebound hyperglycemia after resection of insulinoma indicates a biochemical cure. However, there is scant objective data in the literature on the rate and need for intervention in hyperglycemia in patients undergoing resection of insulinoma. The goal of our study was to evaluate the rate of postoperative hyperglycemia, any predisposing factors, and the need for intervention in a prospective cohort study of all patients undergoing routine glucose monitoring. METHODS: A retrospective analysis of 33 patients who had an insulinoma resected and who underwent routine postoperative monitoring of blood glucose (every hour for the first six hours then every four hours for the first 24 h) was performed. Hyperglycemia was defined as glucose greater than 180 mg/dL (10 mmol/l). RESULTS: Twelve patients (36%) developed hyperglycemia within 24 h (range 1-16 h). In patients with hyperglycemia, the mean maximum plasma glucose level was 221.5 mg/dL (range 97-325 mg/dL) (12.3 mmol/l), and four (33%) patients were treated with insulin. There was no significant difference in age, gender, body mass index (BMI), tumor size, biochemical profile, or surgical approach and extent of pancreatectomy between patients who developed hyperglycemia and those who did not. Pre-excision and post-excision intraoperative insulin levels were evaluated in 14 of 33 patients. The percentage decrease of the intraoperative insulin levels was not significantly different between patients who developed hyperglycemia and those who did not. All patients with postoperative hyperglycemia had normalization of their glucose levels, and none were discharged on anti-hyperglycemic agents. CONCLUSIONS: Hyperglycemia is common after insulinoma resection, and a subset of patients require transient treatment with insulin.


Subject(s)
Digestive System Surgical Procedures/adverse effects , Hyperglycemia/epidemiology , Hypoglycemic Agents/therapeutic use , Insulin/therapeutic use , Insulinoma/surgery , Pancreatic Neoplasms/surgery , Adult , Aged , Blood Glucose , Female , Humans , Hyperglycemia/drug therapy , Hyperglycemia/etiology , Incidence , Male , Middle Aged , Postoperative Complications/drug therapy , Postoperative Complications/epidemiology , Postoperative Period , Retrospective Studies
11.
J Clin Endocrinol Metab ; 103(8): 2811-2814, 2018 08 01.
Article in English | MEDLINE | ID: mdl-29846662

ABSTRACT

Context: Patients taking exogenous glucocorticoids are at risk for gastrointestinal (GI) complications, including peptic ulcer disease with perforation and gastric bleeding. However, little is known about the GI comorbidity in patients with endogenous hypercortisolemia. Case Descriptions: We describe six patients with endogenous Cushing syndrome (CS) who developed sudden perforation of colonic diverticula necessitating urgent exploratory laparotomy. Most of these patients shared the following features of CS: skin thinning, severe hypercortisolemia (24-hour urinary free cortisol ≥10 times the upper limit of normal), ectopic secretion of ACTH, and severe hypokalemia. At the time of diagnosis of diverticular perforation (DP), these patients had minimal signs of peritonitis and lacked fever or marked leukocytosis. The diagnosis of DP was established by having a low threshold for obtaining an imaging study for evaluation of nonspecific abdominal pain. Conclusions: Patients with CS can develop spontaneous surgical abdomen with rapid decompensation within hours. Prompt recognition is critical in the successful treatment of these patients.


Subject(s)
Cushing Syndrome/complications , Diverticulum, Colon/etiology , Peptic Ulcer Perforation/etiology , Abdominal Pain/diagnosis , Abdominal Pain/etiology , Adult , Aged , Aged, 80 and over , Cushing Syndrome/pathology , Diverticulum, Colon/pathology , Duodenal Ulcer/complications , Duodenal Ulcer/pathology , Fatal Outcome , Female , Gastrointestinal Hemorrhage/etiology , Gastrointestinal Hemorrhage/pathology , Humans , Male , Middle Aged , Peptic Ulcer Perforation/pathology
12.
J Am Coll Surg ; 226(5): 933-938, 2018 05.
Article in English | MEDLINE | ID: mdl-29408534

ABSTRACT

BACKGROUND: Approximately 10% of patients with primary hyperparathyroidism (PHPT) have hereditary disease. Hereditary PHPT may be syndromic (MEN1, 2, and 4 and hyperparathyroidism-jaw tumor syndrome) or non-syndromic (familial isolated PHPT). There are limited data on the probability of testing positive for genetic mutation based on clinical presentation. The aim of this study was to determine potential associations between clinical and biochemical features and mutation in susceptibility genes for PHPT in patients with a family history of PHPT. STUDY DESIGN: A retrospective analysis of 657 patients who had an initial parathyroidectomy for PHPT at a tertiary referral center. Logistic regression analyses were performed in 205 patients with a family history of PHPT to identify factors associated with a positive genetic test. RESULTS: Of 657 patients, 205 (31.2%) had a family history of PHPT. Of those 205 patients, 123 (60%) had a germline mutation detected (91 MEN1, 14 CDC73, and 18 GCM2). In univariate analysis, younger age (45 years and younger), male sex, multigland disease, and parathyroid carcinoma were associated with positive germline mutation; biochemical cure after an initial parathyroidectomy was less frequent in patients with familial PHPT (96.2% vs 89.2%; p = 0.005). In multivariable analysis, age 45 years and younger, male sex, and multigland disease were independent factors associated with positive genetic testing. CONCLUSIONS: In addition to a family history of PHPT, male sex, age 45 years and younger, and presence of multigland disease, should prompt physicians to offer the opportunity for genetic counseling and testing, as it could influence the management of patients with PHPT.


Subject(s)
Genetic Testing , Hyperparathyroidism, Primary/genetics , Adult , Age Factors , Female , Genetic Predisposition to Disease , Germ-Line Mutation , Humans , Hyperparathyroidism, Primary/surgery , Male , Middle Aged , Parathyroidectomy , Probability , Retrospective Studies
13.
J Clin Endocrinol Metab ; 103(4): 1631-1638, 2018 04 01.
Article in English | MEDLINE | ID: mdl-29294023

ABSTRACT

Context: Patients with von Hippel-Lindau (vHL) disease caused by a missense VHL mutation have a more severe phenotype compared with other VHL mutation types. Objective: To define pancreatic neuroendocrine tumor (PNET) aggressiveness according to VHL genotype. Design: A prospective natural history study. Setting: The National Institutes of Health clinical center. Patients: Patients with vHL disease, pancreatic manifestations, and germline missense VHL gene mutations. Intervention: In-silico prediction of VHL mutation via five computational prediction models. Patients with >80% prediction for disease-causing mutations in all models [high predicted risk (HPR)] were compared with others [low predicted risk (LPR)]. Main Outcome Measure: Rates of metastases, surgical intervention, and disease progression. Results: Sixty-nine patients were included: 2 developed metastases, 12 needed surgery, and 31 had disease progression during a median follow-up of 60 months (range 13 to 84 months). Thirteen patients were excluded for low prediction reliability. In the remaining 56 patients (45 with PNETs, 11 with pancreatic cysts), the HPR group (n = 13) had a higher rate of disease progression than the LPR group (n = 43) in multivariable analysis (hazard ratio 3.6; 95% confidence interval, 1.1 to 11.9; P = 0.037). The HPR group also had a higher risk of developing metastases (P = 0.015). Among patients with codon 167 hotspot mutations (n = 26), those in the HPR group had a higher risk for disease progression (P = 0.03) than other patients. Conclusions: Computational models for predicting the impact of missense VHL gene mutations may be used as a prognostic factor in patients with PNETs in the context of vHL disease.


Subject(s)
DNA Mutational Analysis/methods , Mutation , Pancreatic Neoplasms/genetics , Von Hippel-Lindau Tumor Suppressor Protein/genetics , von Hippel-Lindau Disease/genetics , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/pathology , Adult , Carcinoma, Renal Cell/genetics , Carcinoma, Renal Cell/pathology , Computer Simulation , Disease Progression , Female , Hemangioblastoma/genetics , Hemangioblastoma/pathology , Humans , Male , Middle Aged , Pancreas/pathology , Pancreatic Neoplasms/pathology , Pheochromocytoma/genetics , Pheochromocytoma/pathology , Prognosis , Prospective Studies , Reproducibility of Results , von Hippel-Lindau Disease/pathology
14.
Surgery ; 163(1): 191-196, 2018 01.
Article in English | MEDLINE | ID: mdl-29126554

ABSTRACT

BACKGROUND: Our knowledge of the susceptibility genes for pheochromocytomas/paragangliomas has increased; however, data on its impact on surgical decision-making has not been described. The aim of this study was to determine the effect of routine preoperative genetic testing on the operative intervention in patients with pheochromocytomas/paragangliomas. METHODS: One-hundred-eight patients diagnosed with pheochromocytomas/paragangliomas who underwent 118 operations had preoperative genetic testing for 9 known pheochromocytoma/paraganglioma susceptibility genes. A retrospective analysis of a prospective database was performed to evaluate clinical factors associated with the surgical approach selected and the outcome of the surgical intervention. RESULTS: In 51 patients (47%), a germline mutation was detected and one-third had no family history of pheochromocytoma/paraganglioma. In 77 operations (65%), it was the first operative intervention for the disease site (60 laparoscopic, 17 open), and 41 (35%) were reoperative interventions (36 open, 5 laparoscopic). For initial operations, variables associated with whether an open or laparoscopic approach was used were tumor size (P = .009) and presence of germline mutation (P = .042). Sixty-eight adrenal operations were performed (54 total, 14 cortical-sparing). Variables significantly associated with a cortical-sparing adrenalectomy being performed were the presence of germline mutation (P = .006) and tumor size (P = .013). CONCLUSION: Preoperative knowledge of the germline mutation status affects the surgical approach and extent of adrenalectomy.


Subject(s)
Adrenal Gland Neoplasms/surgery , Adrenalectomy/statistics & numerical data , Genetic Testing/statistics & numerical data , Paraganglioma/surgery , Pheochromocytoma/surgery , Adolescent , Adrenal Gland Neoplasms/genetics , Adrenalectomy/methods , Adult , Aged , Child , Child, Preschool , Germ-Line Mutation , Humans , Middle Aged , Paraganglioma/genetics , Pheochromocytoma/genetics , Preoperative Care , Retrospective Studies , Young Adult
15.
Surgery ; 163(1): 31-34, 2018 01.
Article in English | MEDLINE | ID: mdl-29108698

ABSTRACT

BACKGROUND: Hereditary primary hyperparathyroidism may be syndromic or nonsyndromic (familial isolated hyperparathyroidism). Recently, germline activating mutations in the GCM2 gene were identified in a subset of familial isolated hyperparathyroidism. This study examined the clinical and biochemical characteristics and the treatment outcomes of GCM2 mutation-positive familial isolated hyperparathyroidism as compared to sporadic primary hyperparathyroidism. METHODS: We performed a retrospective analysis of clinical features, parathyroid pathology, and operative outcomes in 18 patients with GCM2 germline mutations and 457 patients with sporadic primary hyperparathyroidism. RESULTS: Age at diagnosis, sex distribution, race/ethnicity, and preoperative serum calcium concentrations were similar between the 2 groups. The preoperative serum levels of intact parathyroid hormone was greater in patients with GCM2-associated primary hyperparathyroidism (239 ± 394 vs 136 ± 113, P = .005) as were rates of multigland disease and parathyroid carcinoma in the GCM2 group (78% vs 14.3%, P < .001 and 5% vs 0%, P = .04, respectively), but the biochemical cure rate was less in the GCM2 group (86% vs 99%, P < .001). CONCLUSION: GCM2-associated primary hyperparathyroidism patients have greater preoperative parathyroid hormone levels, a greater rate of multigland disease, a lesser rate of biochemical cure, and a substantial risk of parathyroid carcinoma. Knowledge of these clinical characteristics could optimize the surgical management of GCM2-associated familial isolated hyperparathyroidism.


Subject(s)
Hyperparathyroidism, Primary/genetics , Nuclear Proteins/genetics , Transcription Factors/genetics , Adolescent , Adult , Aged , Female , Germ-Line Mutation , Humans , Hyperparathyroidism, Primary/pathology , Male , Middle Aged , Parathyroid Glands/pathology , Retrospective Studies , Young Adult
16.
Ann Thorac Surg ; 103(6): 1700-1709, 2017 Jun.
Article in English | MEDLINE | ID: mdl-28433224

ABSTRACT

BACKGROUND: Older patients have an increased incidence of paraesophageal hernia (PEH) and can be denied surgical assessment due to the perception of increased complications and mortality. This study examines the influence of age and comorbidities on early complications and other short-term outcomes of PEH repair. METHODS: From 2000 to 2016, data of surgically treated patients with PEH were prospectively recorded in an Institutional Review Board-approved database. Only patients whose hernia involved over 50% of the stomach were included. Patients were stratified by age (<70, 70 to 79, ≥80 years of age) and compared in univariate and multivariate analyses. RESULTS: Overall, 524 patients underwent surgical PEH repair (<70: 261 [50%]; 70 to 79: 163 [31%]; ≥80: 100 [19%]). Patients greater than or equal to 80 years of age had higher American Society of Anesthesiologists class, more comorbidities, larger hernias, and higher incidences of type IV PEH and acute presentation. Patients greater than or equal to 80 years of age had more postoperative complications, but not higher grade complications (Clavien-Dindo grade ≥IIIa). Median length of stay was 1 day longer for patients greater than or equal to 80 years of age (5 days versus 4 days for patients <70 and 70 to 79 years of age, respectively). Objective, radiologic hernia recurrence at 4.3 months postoperation was 17.3% and was not increased in the greater than or equal to 80 years of age group. After adjustment for comorbidities and other factors, age greater than or equal to 80 years was not a significant factor in predicting severe complications, readmission within 30 days, or early recurrence. CONCLUSIONS: PEH repair is safe in physiologically stable patients, irrespective of age. Incidence of complications is higher in older patients, but complication severity and mortality are similar to those of younger patients. Patients with giant PEH should be given the opportunity to review treatments options with an experienced surgeon.


Subject(s)
Hernia, Hiatal/surgery , Herniorrhaphy/adverse effects , Postoperative Complications/epidemiology , Age Factors , Aged , Aged, 80 and over , Female , Follow-Up Studies , Hernia, Hiatal/pathology , Herniorrhaphy/methods , Herniorrhaphy/mortality , Humans , Incidence , Laparoscopy , Male , Middle Aged , Operative Time , Risk Factors
17.
Surg Endosc ; 31(3): 1005-1011, 2017 03.
Article in English | MEDLINE | ID: mdl-27387181

ABSTRACT

BACKGROUND: Portomesenteric vein thrombosis following laparoscopic bariatric surgical procedures is a serious and potentially lethal complication. It is quite rare, and its clinical presentation, management, and sequelae remain poorly understood. METHODS: We searched PubMed, Medline, Google Scholar, Ovid, and Cochrane databases for articles reporting case series and systematic reviews in the English language on patients who underwent laparoscopic bariatric surgery and had a subsequent portal or mesenteric vein thrombosis. Articles discussing laparoscopic gastric banding were excluded. RESULTS: A total of 14 articles reporting on 36 cases were found. We analyzed the pooled data from these case reports and series with emphasis on number of reported patients, demographics, time of diagnosis, risk factors, symptoms, management, complications, and sequelae. CONCLUSIONS: Portomesenteric vein thrombosis is not uncommon following laparoscopic bariatric surgery and appears to occur more after laparoscopic sleeve gastrectomy. Bariatric surgeons should have a high index of suspicion for early detection and treatment of this potentially lethal complication. Obese patients at high risk for venous thrombosis should be screened for genetic predisposition for hypercoagulable state and should be considered for extended thromboprophylaxis postoperatively.


Subject(s)
Gastrectomy/methods , Gastric Bypass/methods , Laparoscopy/methods , Mesenteric Veins , Obesity, Morbid/surgery , Portal Vein , Postoperative Complications/epidemiology , Venous Thrombosis/epidemiology , Bariatric Surgery/methods , Humans , Mass Screening , Risk Factors , Thrombophilia/diagnosis , Thrombophilia/genetics
18.
Thrombosis ; 2016: 4965458, 2016.
Article in English | MEDLINE | ID: mdl-27478635

ABSTRACT

Congenital malformations of the inferior vena cava (IVC) are rare and underreported. They can be a risk factor for deep venous thrombosis (DVT) as a result of inadequate venous drainage of the lower extremities through collateral circulation. The significant number of cases reported in the literature highlights their importance, warranting investigating their existence in younger individuals with idiopathic DVT of the lower extremities and pelvic veins. In this systematic review, we depict the typical presentation of IVC malformations, their management, and the management of their associated DVT.

19.
Asian Pac J Cancer Prev ; 17(7): 3521-6, 2016.
Article in English | MEDLINE | ID: mdl-27510003

ABSTRACT

Digital breast tomosynthesis (DBT) as a breast cancer screening modality, through generation of three dimensional images during standard mammographic compression, can reduce interference from breast tissue overlap, increasing conspicuity of invasive cancers while concomitantly reducing falsepositive results. We here conducted a systematic review on previous studies to synthesize the evidence of DBT efficacy, eventually 18 articles being included in the analysis. The most commonly emerging topics were advantages of DBT screening tool in terms of recall rates, cancer detection rates and costeffectiveness, preventing unnecessary burdens on women and the healthcare system. Further research is needed to evaluate the potential impact of DBT on longerterm outcomes, such as interval cancer rates and mortality, to better understand the broader clinical and economic implications of its adoption.


Subject(s)
Breast Neoplasms/diagnosis , Cost-Benefit Analysis/economics , Early Detection of Cancer/economics , Early Detection of Cancer/methods , Mammography/economics , Mammography/methods , Breast/pathology , Breast Neoplasms/pathology , Cost-Benefit Analysis/methods , Female , Humans , Imaging, Three-Dimensional/economics , Imaging, Three-Dimensional/methods
20.
Ann Thorac Surg ; 102(3): 931-939, 2016 Sep.
Article in English | MEDLINE | ID: mdl-27283109

ABSTRACT

BACKGROUND: After esophagectomy, some patients exceed targeted discharge goal within enhanced recovery after surgery programs. This study reviews the demographics, outcomes, cost, readmission rates, and patient satisfaction for the accelerated recovery (AR) group. METHODS: Between 2010 and 2013, 137 consecutive esophagectomy patients were compared according to the length of hospital stay: AR 5 to 6 days, targeted recovery (TR) 7 to 8 days, and delayed recovery (DR) 9 days or more. RESULTS: The AR patients increased from 3% to 46% during the study period. The AR patients were younger, but all groups were comparable regarding comorbidities (Charlson, American Society of Anesthesiologists, and Eastern Cooperative Oncology Group score), cancer stage, and treatment approach. The AR patients were more likely to have neoadjuvant therapy, shorter operations, and less blood loss. The DR patients were more likely to have complications (40% AR versus 45% TR versus 90% DR, p < 0.001). Inhospital and 90-day mortality was 1.5%. All AR patients were discharged home (100% AR versus 87% TR versus 63% DR, p < 0.001), and 30-day readmission rates were comparable between groups (14% AR versus 19% TR versus 5% DR, p = 0.122). Overall mean costs ($38,385 AR versus $41,607 TR versus $61,199 DR, p < 0.001) as well as readmission costs ($7,470 AR versus $27,695 TR versus $33,398 DR, p = 0.202) were lower in the AR group. Patient satisfaction scores were comparable between groups. CONCLUSIONS: Accelerated recovery is achievable in a significant proportion of patients undergoing esophagectomy. Accelerated recovery is associated with decreased treatment costs but does not lead to increased readmissions or decreased patient satisfaction. Enhanced recovery after surgery programs should be designed to accommodate patients appropriate for AR.


Subject(s)
Esophagectomy , Health Care Costs , Patient Discharge , Patient Satisfaction , Adult , Aged , Aged, 80 and over , Cohort Studies , Esophagectomy/economics , Female , Humans , Length of Stay , Male , Middle Aged , Patient Readmission , Prospective Studies , Time Factors
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