Diagnosis, Genetics, and Management of 24 Patients With Cardiac Paragangliomas: Experience From a Single Center.

Context: Paragangliomas located within the pericardium represent a rare yet challenging clinical situation. Objective: The current analysis aimed to describe the clinical characteristics of cardiac paragangliomas, with emphasis on the diagnostic approach, genetic background, and multidisciplinary management. Methods: Twenty-four patients diagnosed with cardiac paraganglioma (PGL) in Peking Union Medical College Hospital, Beijing, China, between 2003 and 2021 were identified. Clinical data was collected from medical record. Genetic screening and succinate dehydrogenase subunit B immunohistochemistry were performed in 22 patients. Results: The median age at diagnosis was 38 years (range 11-51 years), 8 patients (33%) were females, and 4 (17%) had familial history. Hypertension and/or symptoms related to catecholamine secretion were present in 22 (92%) patients. Excess levels of catecholamines and/or metanephrines were detected in 22 (96%) of the 23 patients who have completed biochemical testing. Cardiac PGLs were localized with 131I-metaiodobenzylguanidine scintigraphy in 11/22 (50%), and 99mTc-hydrazinonicotinyl-tyr3-octreotide scintigraphy in 24/24 (100%) patients. Genetic testing identified germline SDHx mutations in 13/22 (59%) patients, while immunohistochemistry revealed succinate dehydrogenase (SDH) deficiency in tumors from 17/22 (77%) patients. All patients were managed by a multidisciplinary team through medical preparation, surgery, and follow-up. Twenty-three patients received surgical treatment and perioperative death occurred in 2 cases. Overall, 21 patients were alive at follow-up (median 7.0 years, range 0.6-18 years). Local recurrence or metastasis developed in 3 patients, all of whom had SDH-deficient tumors. Conclusion: Cardiac PGLs can be diagnosed based on clinical manifestations, biochemical tests, and appropriate imaging studies. Genetic screening, multidisciplinary approach, and long-term follow-up are crucial in the management of this disease.

A scientometric study of the top 100 most-cited publications based on Web-of-Science regarding robotic versus laparoscopic surgery.

Minimally invasive surgery includes traditional laparoscopic and robot-assisted surgery. Although many studies related to robotic surgery and laparoscopic surgery have been published, when doing our search, scientometric studies that focus on related robotic surgery versus laparoscopic surgery were limited. In this study, we aimed to analyze and review the research hots and research status of robotic surgery versus laparoscopic surgery. We searched publications that involved robotic surgery versus laparoscopic surgery in the Web of Science database from 1980 to May 23, 2020. The top 100 publications were published in 2012 with the number of 17 and citations ranged from 618 to 64. Published across 34 different journals, namely European urology (n = 17) and others, the greatest contribution among 36 institutes was made by the Cleveland Clinic (n = 11). Of the top 100 publications, a total of 429 unique words were identified and the most frequently occurring keyword was laparoscopy (n = 33). The co-occurrence of keywords in the top 100 publications indicated that the study of diseases mainly focused on prostatectomy, complications, prostate cancer, retropubic prostatectomy, nephron-sparing surgery, lymph-node dissection, total mesenteric excision, sexual function, rectal cancer, and assisted distal gastrectomy. In recent years, comparative research on robot and laparoscopic surgery has decreased and most studies focus on cancer.

A complicated case of innominate and right common arterial aneurysms due to Takayasu's arteritis.

BACKGROUND: Innominate artery aneurysms (IAAs) are relatively rare. Endovascular therapy has been an alternative to open surgery in some IAA cases, but open repair is still necessary in complicated cases. CASE SUMMARY: We report a 35-year-old female who suffered from Takayasu's arteritis. The patient did not get regular treatment, and IAA and right common carotid artery aneurysm developed, which complicated with occlusion of the left carotid artery, subclavian artery, and the initial part of the left vertebral artery. The patient also had moderate aortic valve insufficiency. With inflammation being controlled well, the patient received the surgery for arterial aneurysms of innominate and right common carotid arteries and aortic valve insufficiency. The shunts for cerebral blood supply were designed to protect the brain and the surgery was conducted successfully under extracorporeal circulation. CONCLUSION: The case illustrates that open surgery may be appropriate for some complicated IAAs, and brain protection is important.

Outcomes of surgery for functional cardiac paragangliomas: A single-center experience of 17 patients.

OBJECTIVE: Cardiac paragangliomas are rare neuroendocrine tumors. Early surgical treatment improves clinical symptoms and prolongs survival. We review our experience in 17 patients who underwent surgical resection for functional cardiac paraganglioma. METHODS: Seventeen patients underwent surgery for functional cardiac paraganglioma from 2004 to 2017 were identified. Clinical data and long-term outcomes were extracted and analyzed. RESULTS: All 17 patients with cardiac paraganglioma (11 males) with a median age of 35 years (range, 11 to 51 years) were hormonally functional and underwent operations. A 24-hour urine catecholamine assay documented elevation of norepinephrine, epinephrine, and dopamine. Tumors were determined with octreotide scintiscan in all 17 patients and metaiodoben-zylguanidine scintigraphy in 10 of 15 patients. Tumor sites were right atrioventricular groove in 4 patients, between the main arteries in 10 patients, and interatrial groove in 4 patients. All patients underwent complete resection. Concomitant surgeries were: structural reconstruction in 16 patients (94%) and coronary artery bypass graft in 8 patients (47%). One patient had a history of incomplete resection of an intra-atrial tumor. One patient died postoperatively. Operative mortality is 5.8%. During follow-up (mean, 6.5 years; range, 1.5 to 14.2 years), 16 patients are alive in functional class I or II. Two patients developed tumor recurrence and 14 remained symptom-free with normal urinary catecholamines. CONCLUSIONS: With a multidisciplinary treatment, early diagnosis, complete resection for the tumor along with complex reconstruction is achievable for most patients, and it offers promising long-term survival.

Leukocyte Telomere Length and All-Cause Mortality: A Between-Within Twin Study With Time-Dependent Effects Using Generalized Survival Models.

Although previous studies examining leukocyte telomere length (LTL) and all-cause mortality controlled for several confounders, the observed association could be biased due to unmeasured confounders, including familial factors. We aimed to examine the association of LTL with all-cause mortality in a Swedish twin sample while adjusting for familial factors and allowing for time-dependent effects. A total of 366 participants (174 twin pairs and 18 individuals) were recruited from the Swedish Twin Registry. LTL was assessed using the Southern blot method. All-cause mortality data were obtained through linkage with the Swedish Population Registry, updated through November 15, 2017. To control for familial factors within twin pairs, we applied a between-within shared frailty model based on generalized survival models. Overall, 115 (31.4%) participants were men and 251 (68.6%) were women. The average age of the study participants when blood was drawn was 79.1 years, and follow-up duration ranged from 10 days to 25.7 years (mean = 10.2 years). During the follow-up period, 341 (93.2%) participants died. Shorter LTL was associated with higher mortality rates when controlling for familial factors in the between-within shared frailty model. We found significant time-dependent effects of LTL on all-cause mortality, where the mortality rate ratios were attenuated with increasing age.

Parametric and penalized generalized survival models.

We describe generalized survival models, where g( S( t| z)), for link function g, survival S, time t, and covariates z, is modeled by a linear predictor in terms of covariate effects and smooth time effects. These models include proportional hazards and proportional odds models, and extend the parametric Royston-Parmar models. Estimation is described for both fully parametric linear predictors and combinations of penalized smoothers and parametric effects. The penalized smoothing parameters can be selected automatically using several information criteria. The link function may be selected based on prior assumptions or using an information criterion. We have implemented the models in R. All of the penalized smoothers from the mgcv package are available for smooth time effects and smooth covariate effects. The generalized survival models perform well in a simulation study, compared with some existing models. The estimation of smooth covariate effects and smooth time-dependent hazard or odds ratios is simplified, compared with many non-parametric models. Applying these models to three cancer survival datasets, we find that the proportional odds model is better than the proportional hazards model for two of the datasets.

CT-Guided Percutaneous Sclerotherapy for Low-Flow Retrobulbar Intraconal Venous Malformations.

BACKGROUND AND AIMS: Until now, no study has focused exclusively on low-flow retrobulbar intraconal venous malformations (RIVMs) which may require treatment due to cosmetic defect, pain, and visual dysfunction. The treatment for RIVMs which surround the optic nerve remains challenging. This case series aimed to evaluate the technical feasibility, effectiveness, and safety of percutaneous sclerotherapy with polidocanol for low-flow RIVMs, using local anesthesia. METHOD: This is a prospective, non-comparative, single-center, interventional case series. All patients signed informed consent forms. Seven patients with RIVMs were treated with percutaneous sclerotherapy with polidocanol/air foam using CT guidance. Primary endpoints are reduction in the volume of RIVMs and pain relief assessed by visual analog scale (VAS). Secondary endpoints are exophthalmos and recording adverse events obtained in clinical follow-up during outpatient visits. RESULTS: Results revealed that the mean volume of RIVMs was decreased from 12.05 ± 6.35 cm3 preoperatively to 1.56 ± 0.43 cm3 postoperatively, (p = 0.005), with a mean decrease of 87.05%. The intraocular pressure was decreased from 14.19 ± 2.99 to 11.79 ± 1.25 mmHg, (p = 0.043). The mean VAS score was decreased from 3.43 ± 2.37 preoperatively to 1.29 ± 0.76 postoperatively, (p = 0.023). The exophthalmos score was decreased from 1.75 ± 0.27 to 1.34 ± 0.31 cm, (p = 0.005). All patients were satisfied with the treatment, which did not leave a postoperative scar. CONCLUSIONS: The results of percutaneous intralesion injection of polidocanol for RIVMs are encouraging. The present results suggest that this method could be a safe and effective treatment option for patients with RIVMs.

Generalized survival models for correlated time-to-event data.

Our aim is to develop a rich and coherent framework for modeling correlated time-to-event data, including (1) survival regression models with different links and (2) flexible modeling for time-dependent and nonlinear effects with rich postestimation. We extend the class of generalized survival models, which expresses a transformed survival in terms of a linear predictor, by incorporating a shared frailty or random effects for correlated survival data. The proposed approach can include parametric or penalized smooth functions for time, time-dependent effects, nonlinear effects, and their interactions. The maximum (penalized) marginal likelihood method is used to estimate the regression coefficients and the variance for the frailty or random effects. The optimal smoothing parameters for the penalized marginal likelihood estimation can be automatically selected by a likelihood-based cross-validation criterion. For models with normal random effects, Gauss-Hermite quadrature can be used to obtain the cluster-level marginal likelihoods. The Akaike Information Criterion can be used to compare models and select the link function. We have implemented these methods in the R package rstpm2. Simulating for both small and larger clusters, we find that this approach performs well. Through 2 applications, we demonstrate (1) a comparison of proportional hazards and proportional odds models with random effects for clustered survival data and (2) the estimation of time-varying effects on the log-time scale, age-varying effects for a specific treatment, and two-dimensional splines for time and age.

Percutaneous intratumoral injection of gemcitabine plus cisplatin mixed with fibrin glue for advanced pancreatic carcinoma: Case Report.

RATIONALE: The aim of this study was to determine the effectiveness of intratumoral injection of chemotherapeutics in improving the quality of life and survival of patients with pancreatic carcinoma. PATIENT CONCERNS: We present a case series of 5 patients with unresectable pancreatic adenocarcinoma. DIAGNOSES: Patients diagnosed with unresectable poorly differentiated pancreatic ductal adenocarcinoma by intraoperative frozen biopsyor percutaneous biopsy. INTERVENTIONS: Five patients with unresectable pancreatic adenocarcinoma received a computed tomography-guided percutaneous intratumoral injection of gemcitabine plus cisplatin mixed with fibrin glue. OUTCOMES: Mean overall survival was 16.2 ±â€Š3.7 months. Local control rates were 100% and 80% at postoperative 3 and 6 months, respectively. Mean Visual Analogue Scale pain score decreased from 7.2 ±â€Š.84 preoperatively to 2 ±â€Š1.22 at postoperative 4 weeks. There were no complications associated with the procedure. LESSONS: Percutaneous intratumoral injection of gemcitabine plus cisplatin mixed with fibrin glue for advanced pancreatic may be safe and effective.

Surgical Treatment Strategies of Intravenous Leiomyomatosis withRight Cardiac Cavities Extension.

Objective To investigate the diagnosis and surgical treatment strategies of intravenous leiomyomatosis(IVL)extending through inferior vena cava into the right cardiac cavities. Methods Thirty patients of IVL extending through inferior vena cava into the right cardiac cavities were treated in Peking Union Medical College Hospital from November 2002 to January 2015.The following variables were studied: age,cardiopulmonary bypass time,deep hypothermic circulatory arrest time,origins of IVL,blood loss,duration of post-operative hospital stay,hospitalization expenses,edema of lower extremity,blood transfusion,postoperative complication,residual IVL,and re-grow or recurrence. Results Thirteen of 30 patients reported double lower limb edema. The cardiopulmonary bypass was applied in 27 cases,and the average duration of cardiopulmonary bypass was(106.9±53.7)min. Then,21 patients were treated with the deep hypothermic circulatory arrest,and the mean time was(28.2±11.6) min. The tumors originated from the genital veins in 9 cases,the iliac vein in 13 cases,and both veins in 8 cases. The average intra-operative blood loss volume was (2060.5±2012.3)ml,and 21 patients received blood transfusion. The average hospitalization time was(18.9±8.3)days and the average hospitalization expenses was (80 840.4±28 264.2)RMB yuan. While 14 patients had postoperative complications,there was no serious postoperative complication or death.All patients have shown a favorable outcome.Conclusions Tumor embolus extending through inferior vena cava into the right cardiac cavities should be suspected in patients with multiple hysteromyoma. Successful therapy for IVL with right cardiac cavities extension is dependent on reasonable surgical treatment strategies. Surgical removal of the ovaries is vital to avoid IVL re-grow or recurrence.

Surgical treatment of pulmonary artery aneurysm: an institutional experience and literature review.

OBJECTIVES: The occurrence of pulmonary artery aneurysm (PAA) is extremely rare in the clinical setting. Careful treatment should be considered because of the possibility of fatal complications including rupture, dissection, pulmonary embolism and heart failure. Our goal is to contribute a better understanding of this disease and its treatment. METHODS: Information on patients diagnosed with PAA was retrieved from the institute's database. The detailed data including aetiology, clinical presentation, diagnostic methods, operation methods and long-term outcomes of the surgical cases were outlined and analysed. RESULTS: In total, 21 patients were diagnosed with PAA in Peking Union Medical College Hospital from 1980 to 2015, among whom 5 patients received surgical treatment, including 2 patients with giant PAAs. The complete remission rate of surgical cases was 80%, and the average postoperative hospital stay was 8.5 ± 1.29 days. One postoperative death occurred due to distributive shock. CONCLUSIONS: PAAs must be seriously classified by aetiology to be treated appropriately. Patients with giant-size PAAs, and those with pulmonary hypertension, anatomical anomalies, and rapid growth and compression of neighbouring critical structures, are proper candidates for surgery. Surgical options include aneurysm repair and replacement with allogeneic/synthetic grafts, depending on the situation. Additionally, the correction of associated abnormalities should be performed simultaneously during surgery. Surgical outcomes are effective, and long-term prognoses are satisfactory.

[Endoscopic Saphenous Vein Harvesting versus Open Vein Harvesting Techniques].

OBJECTIVE: To investigate whether endoscopic saphenous vein harvesting (EVH )reduces leg wound morbidity and improves rehabilitation of lower extremity as compared to open vein harvesting (OVH) in patients with coronary artery disease. METHODS: The clinical data of 333 patients who underwent isolated coronary artery bypass graft surgery (CABG) between January 2007 and December 2011 were retrospectively analyzed.These patients were divided into EVH group (n=210) and OVH group (n=123). Parameters including age,sex,with/without diabetes mellitus,leg wound complications rate,postoperative hospital stay,hospitalization expense,and vein graft patency were analyzed. RESULTS: The age,sex,with/without diabetes mellitus were not significantly different between two groups (P>0.05). However,the incidences of leg wound complications, poor wound healing, wound infections, wound pain,wound numbness, and edema of lower extremity were significantly lower in the EVH group than the OVH group (P<0.05). The ecchymoma and deep vein thrombosis incidences were not significantly different between these two groups (P>0.05). The postoperative hospital stay showed no significant difference (P>0.05), while the hospitalization expense was significantly higher in the EVH group than in the OVH group (P<0.05). There was no statistical difference of vein graft patency (P>0.05). CONCLUSION: The EVH system is a safe and effective alternative to OVH because it can markedly reduce postoperative leg wound morbidity and the EVH vein graft has good patency.

[Analyzing the production of organic acid of different genotype streptococcus mutans isolated from children with different caries experience].

OBJECTIVE: To compare the production of organic acid of different genotype Streptococcus mutans (S. mutans) isolated from children with different caries experience. METHODS: 66 strains of S.mutans isolated from dental plaques of children aged from 3 to 5 with different caries experience were chosen as test bacteria. The quantities of organic acid include formic acid, acetic acid and lactic acid which produced by different genotype of S. mutans, were measured by gas chromatograph. RESULTS: There were significant difference in production of organic acid among the different genotypes of S. mutans isolated from children with different caries susceptibility, and so were the strains isolated from children within the same caries susceptibility (P < 0.05). The more genotypes the strain had, the more organic acid it produced (P < 0.05). Among all the organic acid, the quantity of lactic acid was much more than that of formic acid and acetic acid. CONCLUSIONS: There were significant difference in the ability of the strains with different genotypes to produce organic acid, and the more genotypes it has, the more organic acid it produced.

[Establishment and application of a RT-PCR system for quantifying mRNA of 4 hsp genes in human cells].

OBJECTIVE: To establish a RT-PCR system for detecting mRNA expression level of 4 hsp genes in human cells. METHODS: RT-PCR system was established with gene cloning, gene recombination, in vitro transcription and RT-PCR techniques; The detection of expression level of 4 hsp genes in SW13 cell was carried out with this system. RESULTS: In SW13 cell, hsp70 and hsp90 alpha were typical heat shock induced genes, while hsp60 and hsp90 beta were efficiently expressed and further induced by heat-shock to various extent. CONCLUSIONS: In our hands novel RT-PCR system can be used to detect mRNA expression level of 4 human hsp genes.