Objective To analyze the clinical characteristics of nonfunctioning pheochromocytoma, and to evaluate the efficacy of 131I-metaiodobenzylguanidine (MIBG) scan in the diagnosis and perioperative treatment of nonfunctio...Objective To analyze the clinical characteristics of nonfunctioning pheochromocytoma, and to evaluate the efficacy of 131I-metaiodobenzylguanidine (MIBG) scan in the diagnosis and perioperative treatment of nonfunctioning pheochromocy- toma. Methods The clinical data of 14 patients with nonfunctioning pheochromocytoma were analyzed retrospectively. Plasma free corticoid, renin, aldosterone, and urine catecholamines levels were estimated. B-mode ultrasonography, computed tomo- graphy scan, thoracic X-ray and 131I-MIBG were used. Results All patients with nonfunctioning pheochromocytoma had no hypertension and the tumors were found inciden- tally. The 24 hours urine catecholamines levels in 80% (8/10) patients were normal. The positive rate of 131I-MIBG was 80% (8/10) and the specificity was 100%. All patients underwent surgical operation of tumor resection. No preoperative volume expansion was given to all patients. All tumors were resected completely, and no death accident happened. There was no recurrence and metastasis after operation by long-term follow-up. Conclusion 131I-MIBG scan is the first choice technique for the diagnosis of nonfunctioning pheochromocytoma. Blood volume expansion is unnecessary before resection of pheochromocytoma.no recurrence and metastasis})展开更多
Objective To investigate the expression of cyclooxygenase-2 （ Cox-2 ） and microvessel density （MVD） in benign and malignant pheochromocytomas, and the relationship between Cox-2 and MVD. Methods Specimens and clin...Objective To investigate the expression of cyclooxygenase-2 （ Cox-2 ） and microvessel density （MVD） in benign and malignant pheochromocytomas, and the relationship between Cox-2 and MVD. Methods Specimens and clinical data from 38 patients （ 21 benign and 17 malignant pheochromocytomas ） were studied. Slides of normal adrenal glands in nephrectomy specimens from another 20 patients with benign renal tumors were used as control. Irnmunohistochemical technology was performed to detect the Cox-2 and MVD in all specimens. Results Expression of Cox-2 was observed in 5 of the 21 benign pheochromocytomas （23. 8% ） , and in 14 of the 17 malignant （82.4%）. No expression of Cox-2 was observed in control slides. There were significant differences of Cox-2 expression between benign and malignant pheochromocytomas, as well as between malignant pheochromocytomas and control （ P 〈0. 05）. Expressions of MVD were 36. 41 ±13. 00, 21.43 ±8. 05, and 13. 36 ±4.34 in malignant, benign pheochromocytomas, and in control, respectively. Conclusion Cox-2 may contribute to the invasive characteristics of malignant pheochromocytomas and be used as a marker to distinguish malignant from benign pheochromocytomas. Expression of MVD in malignant pheochromocytomas was directly correlated with Cox-2.展开更多
BACKGROUND Phaeochromocytoma and paragangliomas(PPGL)are rare neuroendocrine tumors usually caused by parasympathetic or sympathetic nerves,with an overall incidence of approximately 0.66 cases per 100000 people per y...BACKGROUND Phaeochromocytoma and paragangliomas(PPGL)are rare neuroendocrine tumors usually caused by parasympathetic or sympathetic nerves,with an overall incidence of approximately 0.66 cases per 100000 people per year.Most PPGLs are asymptomatic,and a few develop symptoms,such as elevated blood pressure and rapid heart rate,because of the release of catecholamines.According to the literature,surgical resection is the mainstay of PPGL treatment.However,the choice between minimally invasive surgery and open surgery remains controversial,particularly in cases involving complex anatomical relationships.We successfully resected a tumor located between the inferior vena cava and abdominal aorta using a minimally invasive approach with the assistance of computed tomography(CT)angiography and three-dimensional reconstruction,resulting in a favorable outcome.CASE SUMMARY A 56-year-old woman was admitted to the hospital with right upper abdominal discomfort for more than 6 months,experiencing occasional pins and needles sensation and radiation from the right shoulder and back.Prehospital CT of the upper abdomen revealed a retroperitoneal mass suspected to be a retroperitoneal ganglioneuroma.Surgery was recommended,and an enhanced CT scan of the upper abdomen,along with preoperative three-dimensional reconstruction,was performed after admission.The imaging indicated that the mass,measuring approximately 4.1 cm×3.8 cm×4.8 cm,was situated between the abdominal aorta and the inferior vena cava,extending downward to the level of the left renal vein.After ruling out any contraindications to surgery,a minimally invasive laparoscopy was performed to excise the mass precisely.The surgery was successful without any postoperative complications,and the 2-month follow-up revealed no abnormal signs of recurrence.CONCLUSION This case report describes successful and precise laparoscopic resection of a retroperitoneal tumor.The patient recovered well during the 2-month follow-up,and postoperative pathology revealed a paraganglioma.展开更多
BACKGROUND Pheochromocytoma(PHEO)is a type of tumor that originates from chromaffin cells in the adrenal medulla and is classified as an adrenal paraganglioma.PHEOs can secrete catecholamines,leading to a variety of s...BACKGROUND Pheochromocytoma(PHEO)is a type of tumor that originates from chromaffin cells in the adrenal medulla and is classified as an adrenal paraganglioma.PHEOs can secrete catecholamines,leading to a variety of symptoms.Accurate diagnosis and appropriate treatment selection are crucial for favorable outcomes in these cases.CASE SUMMARY The patient presented with unexplained chest tightness,palpitations,and pink sputum.Upon examination and analysis of laboratory results,a diagnosis of adrenal PHEO was established.The PHEO secreted high levels of catecholamines,causing sudden fluctuations in blood pressure and heart rate,leading to extre-mely unstable hemodynamics.Treatment with extracorporeal membrane oxygenation and intra-aortic balloon counterpulsation helped stabilize the patient’s vital signs,allowing for timely surgical intervention.CONCLUSION The combination of extracorporeal membrane oxygenation and intra-aortic balloon counterpulsation can enhance tissue perfusion,thus providing a solid foundation for the accurate diagnosis and effective surgical treatment of PHEO.展开更多
Pheochromocytoma (PHEO) is a rare endocrine tumor from the chromaffin cells in the adrenomedullary gland and sympathetic/parasympathetic ganglia, secreting one or more catecholamines. It is frequently associated with ...Pheochromocytoma (PHEO) is a rare endocrine tumor from the chromaffin cells in the adrenomedullary gland and sympathetic/parasympathetic ganglia, secreting one or more catecholamines. It is frequently associated with hypertension and described in the literature as a cause of secondary diabetes mellitus. In patients with known persistent uncontrolled diabetes, PHEO is rarely mentioned as the cause of uncontrolled diabetes. The authors report a rare case of PHEO diagnosed in a 64-year-old female patient treated for 10 years with type 2 diabetes mellitus and hypertension. She was treated using a combination of insulin (2 injections) and Metformin 1000 mg twice a day. The glycemic control was poor (HbA1c-11%), and persistent High Blood Pressure (HBP). She presented with unexplained weight loss associated with permanent hyperhidrosis (sweating), affecting her quality of life and diffuse abdominal pain. The investigations confirmed the diagnosis of PHEO, which resection led to improvement of glycemic control and hypertension.展开更多
BACKGROUND Pheochromocytoma,a rare catecholamine-secreting tumor,typically presents with the classic triad of headache,palpitations,and diaphoresis,often accompanied by cardiovascular manifestations.While vomiting occ...BACKGROUND Pheochromocytoma,a rare catecholamine-secreting tumor,typically presents with the classic triad of headache,palpitations,and diaphoresis,often accompanied by cardiovascular manifestations.While vomiting occurs in approximately 34.5%of cases,it is rarely the predominant and persistent presenting symptom.Pheochro-mocytoma-induced cardiomyopathy leading to heart failure is a recognized but uncommon complication.Due to its heterogeneous presentations,misdiagnosis and diagnostic delay are frequent.CASE SUMMARY A 53-year-old female presented predominantly with persistent and refractory vomiting as her chief complaint,accompanied by signs of acute heart failure[left ventricular ejection fraction(LVEF)30%].Initial evaluation at a primary hospital,including coronary angiography(revealing only mild stenosis),led to a misdia-gnosis of coronary artery disease.Despite standard anti-thrombotic,anti-heart failure,and anti-emetic therapy,her vomiting persisted and heart failure did not resolve.Subsequent hospitalization revealed dramatic paroxysmal hypertension(202/129 mmHg to 97/51 mmHg)and fever.Significantly elevated plasma meta-nephrines and normetanephrine,combined with abdominal computed tomogra-phy and magnetic resonance imaging,confirmed a right adrenal pheochromo-cytoma.This diagnosis was significantly delayed due to the atypical prominence of gastrointestinal symptoms masking the underlying endocrine crisis.CONCLUSION This case highlights a highly atypical presentation of pheochromocytoma domi-nated by refractory vomiting and complicated by acute catecholamine-induced cardiomyopathy.It emphatically underscores that pheochromocytoma must be considered in the differential diagnosis for patients presenting with unexplained,treatment-resistant vomiting,particularly when co-existing with acute heart failure.The presence of labile hypertension,even if not initially evident,provides a crucial diagnostic clue.Prompt biochemical screening(catecholamine metabolites)and adrenal imaging are essential to prevent diagnostic delay and enable timely,life-saving surgical intervention.展开更多
Nonfunctional pheochromocytoma is a particular type of pheochromocytoma without typical clinical features of catecholaminism. It is difficult to diagnose or differentially diagnose. This type of pheochromocytoma is ra...Nonfunctional pheochromocytoma is a particular type of pheochromocytoma without typical clinical features of catecholaminism. It is difficult to diagnose or differentially diagnose. This type of pheochromocytoma is rarely reported in the literature. From January 1, 1990 to June 1, 2004, we performed operations for 197 patients with pheochromocytomas, including 26 patients with nonfunctional pheochromocytoma. We analyzed the clinical data of the 26 patients.展开更多
BACKGROUND Multiple endocrine neoplasia type 2(MEN2)is a rare,autosomal dominant endocrine disease.Currently,the RET proto-oncogene is the only gene implicated in MEN2A pathogenesis.Once an RET carrier is detected,fam...BACKGROUND Multiple endocrine neoplasia type 2(MEN2)is a rare,autosomal dominant endocrine disease.Currently,the RET proto-oncogene is the only gene implicated in MEN2A pathogenesis.Once an RET carrier is detected,family members should be screened to enable early detection of medullary thyroid carcinoma,pheochromocytoma,and hyperparatitity.Among these,medullary thyroid carcinoma is the main factor responsible for patient mortality.Accordingly,delineating strategies to inform clinical follow-up and treatment plans based on genes is paramount for clinical practitioners.CASE SUMMARY Herein,we present RET proto-oncogene mutations,clinical characteristics,and treatment strategies in a family with MEN2A.A family study was conducted on patients diagnosed with MEN2A.DNA was extracted from the peripheral blood of family members,and first-generation exon sequencing of the RET protooncogene was conducted.The C634Y mutation was identified in three family members spanning three generations.Two patients were sequentially diagnosed with pheochromocytomas and bilateral medullary thyroid carcinomas.A 9-yearold child harboring the gene mutation was diagnosed with medullary thyroid carcinoma.Surgical resection of the tumors was performed.All family members were advised to undergo complete genetic testing related to the C634Y mutation,and the corresponding treatments administered based on test results and associated clinical guidelines.CONCLUSION Advancements in MEN2A research are important for familial management,assessment of medullary thyroid cancer invasive risk,and deciding surgical timing.展开更多
Approximately 40% of pheochromocytoma and paraganglioma(PPGL) cases are familial, typically presenting earlier with more complex symptoms. This paper synthesizes literature and guidelines to inform on clinical charact...Approximately 40% of pheochromocytoma and paraganglioma(PPGL) cases are familial, typically presenting earlier with more complex symptoms. This paper synthesizes literature and guidelines to inform on clinical characteristics and perioperative care for PPGL. Pheochromocytoma in von Hippel-Lindau(VHL) disease exhibits heightened secretion activity without significant perioperative hemodynamic changes. Tumors in multiple endocrine neoplasia type 2(MEN2) have a stronger endocrine function, which may induce hemodynamic fluctuations during surgery. Therefore, pheochromocytoma screening is essential at all stages of MEN2. Neurofibromatosis type 1(NF1) often presents multisystem lesions and can result in difficult airway. Pheochromocytoma should be evaluated when NF1 patients present hypertension. Pheochromocytoma and paraganglioma type 5 may present multiple lesions of pheochromocytoma or paraganglioma. In summary, hereditary PPGLs may present with severe lesions in other systems, beyond tumor function. A multi-disciplinary team(MDT) approach is often invaluable in perioperative management.展开更多
Pheochromocytomas and paragangliomas(PPGLs)cause symptoms by altering the circulation levels of catecholamines and peptide hormones.Currently,the diagnosis of PPGLs relies on diagnostic imaging and the detection of ca...Pheochromocytomas and paragangliomas(PPGLs)cause symptoms by altering the circulation levels of catecholamines and peptide hormones.Currently,the diagnosis of PPGLs relies on diagnostic imaging and the detection of catecholamines.In this study,we used ultra-performance liquid chromatography(UPLC)/quadrupole time-of-flight mass spectrometry(Q-TOF MS)analysis to identify and measure the perioperative differential metabolites in the plasma of adrenal pheochromocytoma patients.We identified differentially expressed genes by comparing the transcriptomic data of pheochromocytoma with the normal adrenal medulla.Through conducting two steps of metabolomics analysis,we identified 111 differential metabolites between the healthy group and the patient group,among which 53 metabolites were validated.By integrating the information of differential metabolites and differentially expressed genes,we inferred that the cysteine-methionine,pyrimidine,and tyrosine metabolism pathways were the three main metabolic pathways altered by the neoplasm.The analysis of transcription levels revealed that the tyrosine and cysteine-methionine metabolism pathways were downregulated in pheochromocytoma,whereas the pyrimidine pathway showed no significant difference.Finally,we developed an optimized diagnostic model of two metabolites,L-dihydroorotic acid and vanylglycol.Our results for these metabolites suggest that they may serve as potential clinical biomarkers and can be used to supplement and improve the diagnosis of pheochromocytoma.展开更多
BACKGROUND Paraganglioma(PGL)located in the retroperitoneum presents challenges in diagnosis and treatment due to its hidden location,lack of specific symptoms in the early stages,and absence of distinctive manifestat...BACKGROUND Paraganglioma(PGL)located in the retroperitoneum presents challenges in diagnosis and treatment due to its hidden location,lack of specific symptoms in the early stages,and absence of distinctive manifestations on imaging.CASE SUMMARY A 56-year-old woman presented with a left upper abdominal mass discovered 1 wk ago during a physical examination.She did not have a history of smoking,alcohol consumption,or other harmful habits,no surgical procedures or infectious diseases,and had a 4-year history of hypertension.Upon admission,she did not exhibit fever,vomiting,or abdominal distension.Physical examination indicated mild percussion pain in the left upper abdomen,with no palpable enlargement of the liver or spleen.Laboratory tests and tumor markers showed no significant abnormalities.Enhanced computed tomography and magnetic resonance imaging of the upper abdomen revealed a cystic solid mass in the left epigastrium measuring approximately 6.5 cm×4.5 cm,with inhomogeneous enhancement in the arterial phase,closely associated with the lesser curvature of the stomach and the pancreas.The patient underwent laparoscopic resection of the retroperitoneal mass,which was successfully removed without tumor rupture.A 12-month postoperative follow-up period showed good recovery.CONCLUSION This case report details the successful laparoscopic resection of a retroperitoneal subclinical PGL,resulting in a good recovery observed at the 12-month follow-up.Interestingly,the patient also experienced unexpected cure of hypertensive disease.展开更多
Different types of neuroendocrine cancer,including medullary thyroid cancer(MTC)and thyroid C-cell hyperplasia,are part of multiple endocrine neoplasia type 2(MEN2).A proto-oncogene mutation of the rearranged during t...Different types of neuroendocrine cancer,including medullary thyroid cancer(MTC)and thyroid C-cell hyperplasia,are part of multiple endocrine neoplasia type 2(MEN2).A proto-oncogene mutation of the rearranged during transfection(RET)gene changes the way that receptor tyrosine kinases work.Multiple endocrine neoplasia,a pathological condition,involves these kinases.When the RET protooncogene changes,it can cause endocrine adenomas and hyperplasia to happen at the same time or one after the other.Pheochromocytoma,medullary thyroid carcinoma,and hyperparathyroidism,alone or in combination,are present in MEN2A patients.Some patients may also have skin lichen amyloidosis or Hirschsprung's disease.Patients with MEN2A often present with MTC.MTC is aggressive and has the worst prognosis,as most patients exhibit lymph node metastasis.MTC is one of the important causes of death in patients with MEN2A.RET mutation analysis aids in identifying MEN2A symptoms and monitoring levels of calcium,thyroid hormones,calcitonin,normetanephrine,fractionated metanephrines,and parathyroid hormone.The earlier diagnosis of MTC significantly improves survival and prompts better management of MEN2A.In this editorial,we will discuss the significance of molecular diagnostic approaches in detecting RET oncogene mutations in MEN2A.展开更多
Objective:The aim of the study was to investigate effective diagnostic molecular markers and the specific mechanisms of metastatic pheochromocytomas and paragangliomas(PPGLs).Methods:Data were collected from GEO datas...Objective:The aim of the study was to investigate effective diagnostic molecular markers and the specific mechanisms of metastatic pheochromocytomas and paragangliomas(PPGLs).Methods:Data were collected from GEO datasets GSE67066 and GSE60458.The R software and various packages were utilized for the analysis of differentially expressed genes,Gene Ontology analysis,Kyoto Encyclopedia of Genes and Genomes analysis,receiver operating characteristic curve assessment,logistic model construction,and correlation analysis.The NetworkAnalyst tool was used to analyze gene-miRNA interactions and signaling networks.In addition,the TIMER database was used to estimate the immune scores.Results:A total of 203 and 499 differentially expressed genes were identified in GSE67066 and GSE60458,respectively.These genes are implicated in cytokine and cytokine receptor interactions,extracellular matrix–receptor interactions,and platelet activation signaling pathways.Notably,MAMLD1,UST,MATN2,LPL,TWIST1,SFRP4,FRMD6,RBM24,PRIMA1,LYPD1,KCND2,CAMK2N1,SPOCK3,and ALPK3 were identified as the key genes.Among them,MATN2 and TWIST1 were found to be coexpressed with epithelial-mesenchymal transition–linked markers,whereas KCND2 and LPL exhibited associations with immune checkpoint expression and immune cell infiltration.Eight miRNAs were identified as potential regulators of key gene expression,and it was noted that TWIST1 might be regulated by SUZ12.Notably,the area under the curve of the 4-gene model for distinguishing between malignant and benign groups was calculated to be 0.918.Conclusions:The combined gene and mRNA expression model enhances the diagnostic accuracy of assessing PPGL metastatic potential.These findings suggest that multiple genes may play a role in the metastasis of PPGLs through the epithelial-mesenchymal transition and may influence the immune microenvironment.展开更多
Introduction: Adrenal tumours (AT) are commonly encountered in clinical practice. For any patient presenting an adrenal mass, there are two crucial points to consider: is the adrenal mass malignant and is it hormonall...Introduction: Adrenal tumours (AT) are commonly encountered in clinical practice. For any patient presenting an adrenal mass, there are two crucial points to consider: is the adrenal mass malignant and is it hormonally active? The objective of our study was to evaluate the epidemiological, diagnostic, therapeutic, and evolutionary aspects of AT in a developing country. Methodology: We conducted a retrospective study spanning 17 years (from January 2005 to October 2022) in four Dakar hospital services. We included all patient medical records with explored AT. Results: AT was diagnosed in 35 patients who had a mean age of 36.62 years (range: 12-79), and a female predominance (26 women to 9 men). Among these patients, 17.2 % had incidental AT. Hypertension was the commonest presenting symptom in 27 patients (77%), which was associated with Mnards triad in 14 patients (40%). Abdominal pain was reported by 20 patients (57%), and 14 presented with an abdominal mass. Among the patients, 29 had functional AT: 22 with pheochromocytoma, 5 with primary hyperaldosteronism, and 2 with hypercortisolism. Imaging evaluations revealed that 33 patients had unilateral AT and one had bilateral AT. Tumour sizes varied, 5 patients had tumours 10 cm. Two patients presented with metastases. The therapeutic approach involved adrenalectomy in 32 patients. Perioperative complications were observed in five patients, including haemorrhage (two patients), hypotension (two cases), and hypertensive crisis (one case). One patient with bilateral pheochromocytoma developed acute adrenal insufficiency postoperatively, followed by abdominal herniation. Histopathological examination confirmed pheochromocytoma in 21 patients, Conns adenoma in 5, adrenocortical carcinoma in 3, cortisol adenoma in 2, and adrenal cyst in 1 patient. Non-functional AT included adrenocortical carcinoma (three patients), adrenal cyst (one patient), and pheochromocytoma (two patients). After a one-year follow-up, 29 patients with benign tumours had favourable outcomes, while death occurred within six months of diagnosis in five cases. A patient who had bilateral pheochromocytomas, with complications, died before surgery. Conclusion: In our practice, AT are predominantly pheochromocytomas, being typically diagnosed when they become symptomatic large tumours and, due to this large size, laparotomy is the preferred surgical approach. Unfortunately, the prognosis for malignant tumours is poor.展开更多
Introduction: Pheochromocytoma is a rare cause of secondary arterial hypertension whose clinical presentation can be multifaceted. In particular, it may be revealed or complicated by cardiovascular manifestations inde...Introduction: Pheochromocytoma is a rare cause of secondary arterial hypertension whose clinical presentation can be multifaceted. In particular, it may be revealed or complicated by cardiovascular manifestations independent of hypertension. These include Tako-Tsubo cardiomyopathy, an acute reversible dyskinetic cardiomyopathy associated with ballooning of the left ventricular apex. Observation: We present the case of a 32-year-old woman, diabetic for 2 years. Her history included untreated labile hypertension and emotional stress. She was admitted to a cardiac intensive care unit for left heart failure. Paraclinical investigations confirmed the diagnosis of Tako-Tsubo cardiomyopathy induced by pheochromocytoma. After a few days of medical treatment in the ICU, the outcome was favorable, with well-compensated heart failure, stable hemodynamics and restoration of left ventricular function. Conclusion: This observation raises the issue of delayed diagnosis of a pheochromocytoma revealed by a TTC. Despite its spectacular initial presentation, the evolution of the latter was rather favorable under appropriate treatment.展开更多
Pheochromocytomas or paragangliomas are rare and the diagnosis remains a challenge for clinicians.We present a woman suffering from recurrent myocardial infarction and cardiomyopathy,who was finally diagnosed with par...Pheochromocytomas or paragangliomas are rare and the diagnosis remains a challenge for clinicians.We present a woman suffering from recurrent myocardial infarction and cardiomyopathy,who was finally diagnosed with paraganglioma.Pheochromocytomas or paraganglioma should be considered in the differential diagnosis of a patient with symptoms possibly referring to acute coronary syndrome and cardiomyopathy.展开更多
Background： Edaravone had been validated to effectively protect against ischemic injuries. In this study, we investigated the protective effect of edaravone by observing the effects on anti-apoptosis, regulation of B...Background： Edaravone had been validated to effectively protect against ischemic injuries. In this study, we investigated the protective effect of edaravone by observing the effects on anti-apoptosis, regulation of Bcl-2/Bax protein expression and recovering from damage to mitochondria after OGD （oxygen-glucose deprivation）-reperfusion. Methods： Viability of PC 12 cells which were injured at different time of OGD injury, was quantified by measuring MTT （2-（4,5-dimethylthia-zol-2-yl）-2,5-diphenyltetrazolium bromide） staining. In addition, PC 12 cells＇ viability was also quantified after their preincubation in different concentration of edaravone for 30 min followed by （OGD）. Furthermore, apoptotic population of PC 12 cells that reinsulted from OGD-reperfusion with or without preincubation with edaravone was determined by flow cytometer analysis, electron microscope and Hoechst/Pl staining. Finally, change of Bcl-2/Bax protein expression was detected by Western blot. Results： （1） The viability of PC12 cells decreased with time （1-12 h） after OGD. We regarded the model of OGD 2 h, then replacing DMEM （Dulbecco＇s Modified Eagle＇s Medium） for another 24 h as an OGD-reperfusion in this research. Furthermore, most PC 12 cells were in the state of apoptosis after OGD-reperfusion. （2） The viability of PC 12 cells preincubated with edaravone at high concentrations （1, 0.1, 0.01 μmol/L） increased significantly with edaravone protecting PC 12 cells from apoptosis after OGD-reperfusion injury. （3） Furthermore, edaravone attenuates the damage of OGD-reperfusion on mitochondria and regulated Bcl-2/Bax protein imbalance expression after OGD-reperfusion. Conclusion： Neuroprotective effects of edaravone on ischemic or other brain injuries may be partly mediated through inhibition of Bcl-2/Bax apoptotic pathways by recovering from the damage of mitochondria.展开更多
The measurement of urine catecholamine and metanephrine concentrations is important for biochemical screening and diagnosis of pheochromocytoma.The goal of this work was to develop a simple liquid chromatography-tande...The measurement of urine catecholamine and metanephrine concentrations is important for biochemical screening and diagnosis of pheochromocytoma.The goal of this work was to develop a simple liquid chromatography-tandem mass spectrometry(LC-MS/MS)method for determining catecholamines and metanephrines in urine to replace an existing liquid chromatographic method using electrochemical detection.Urine samples were prepared using Oasis weak-cation-exchange cartridges.The eluate was analyzed on an Agilent ZORBAX Eclipse Plus Phenyl-Hexyl column in 3 min.Adrenaline,noradrenaline,dopamine,metanephrine,normetanephrine,and their deuterated internal standards were monitored in positive electrospray ionization mode by multiple reaction monitoring(MRM).No evidence of ion suppression was observed.The assay was linear up to 5μmol/L for adrenaline,5μmol/L for noradrenaline,6.1μmol/L for dopamine,5.6μmol/L for metanephrine,and 34.6μmol/L for normetanephrine,with lower limits of quantification of 5,5,12,6 and 7nmol/L,respectively.The intra-day and inter-day precisions for all analytes ranged from 0.59%to 4.64%and1.98%to 4.80%,respectively.External quality assurance samples were assayed and showed excellent agreement with the target values.This simple method provides an improved assay for determining urine catecholamines and metanephrines.展开更多
Objective： To study the diagnosis and treatment ofpheochromocytoma in urinary bladder. Methods： Six cases of bladder pheochromocytoma were studied. Four cases showed hypertension, 3 of which were paroxysmal hyperten...Objective： To study the diagnosis and treatment ofpheochromocytoma in urinary bladder. Methods： Six cases of bladder pheochromocytoma were studied. Four cases showed hypertension, 3 of which were paroxysmal hypertension during urination. Catecholamine （CA） was increased in a case, and vanillymandelic acid （VMA） was increased in 2 cases. Bladder submucosal mass was detected by B-ultrasound in 5 cases （5/5）, computerized tomography （CT） in 3 cases （3/3）, cystoscopy in 5 cases （5/6）. Four cases took a-receptor blocker for 2 weeks, 1 case took β-receptor blocker to decrease heart rate. All patients were treated with surgical operation including 4 partial cystectomies, 2 excavations. Results： Three cases had manifestations including headache, excessive perspiration and hypertension during cystoscopy. Four cases were confirmed before operation. Two cases showed hypertension during operation. All patients were pathologically diagnosed as pheochromocytoma post- operatively. In five cases followed up, blood pressure returned to normal. No patient had relapse and malignancy. Conclusions： Typical hypertension during urination comprised the main symptoms. We should highly suspect bladder pheochromocytoma if a submucosal mass was discovered with B-ultrasound, CT, ^131I-M1BG （methyliodobenzylguanidine） and cystoscopy. The determination of CA in urine is valuable for qualitative diagnosis. The preoperative management of controlling blood pressure and expansion of the blood volume are very important. Surgical operation is a good method for effective treatment. Postoperative long-time followed up is necessary.展开更多
The coincidence of a gastrointestinal stromal tumor (GIST) and a neuroendocrine tumor (NET) in neurofibromatosis type 1 (NF1) is described only five times within the literature. We report on a 63 year old Caucasian fe...The coincidence of a gastrointestinal stromal tumor (GIST) and a neuroendocrine tumor (NET) in neurofibromatosis type 1 (NF1) is described only five times within the literature. We report on a 63 year old Caucasian female with the rare condition of neurofibromatosis type 1 coinciding with recurrent gastrointestinal stromal tumor plus bilateral pheochromocytoma (PCC). After a history of palpitations and dizziness that lasted for years, a left adrenal mass was detected by CT. Laparotomy revealed a pheochromocytoma of the left adrenal gland while an ileoterminal GIST was found incidentally intraoperatively. After six months contralateral PCC and multiple recurrent GIST were resected again. After four years the patient is doing well without any signs of further recurrent tumors. Discussion includes review of the literature.展开更多
文摘Objective To analyze the clinical characteristics of nonfunctioning pheochromocytoma, and to evaluate the efficacy of 131I-metaiodobenzylguanidine (MIBG) scan in the diagnosis and perioperative treatment of nonfunctioning pheochromocy- toma. Methods The clinical data of 14 patients with nonfunctioning pheochromocytoma were analyzed retrospectively. Plasma free corticoid, renin, aldosterone, and urine catecholamines levels were estimated. B-mode ultrasonography, computed tomo- graphy scan, thoracic X-ray and 131I-MIBG were used. Results All patients with nonfunctioning pheochromocytoma had no hypertension and the tumors were found inciden- tally. The 24 hours urine catecholamines levels in 80% (8/10) patients were normal. The positive rate of 131I-MIBG was 80% (8/10) and the specificity was 100%. All patients underwent surgical operation of tumor resection. No preoperative volume expansion was given to all patients. All tumors were resected completely, and no death accident happened. There was no recurrence and metastasis after operation by long-term follow-up. Conclusion 131I-MIBG scan is the first choice technique for the diagnosis of nonfunctioning pheochromocytoma. Blood volume expansion is unnecessary before resection of pheochromocytoma.no recurrence and metastasis})
文摘Objective To investigate the expression of cyclooxygenase-2 （ Cox-2 ） and microvessel density （MVD） in benign and malignant pheochromocytomas, and the relationship between Cox-2 and MVD. Methods Specimens and clinical data from 38 patients （ 21 benign and 17 malignant pheochromocytomas ） were studied. Slides of normal adrenal glands in nephrectomy specimens from another 20 patients with benign renal tumors were used as control. Irnmunohistochemical technology was performed to detect the Cox-2 and MVD in all specimens. Results Expression of Cox-2 was observed in 5 of the 21 benign pheochromocytomas （23. 8% ） , and in 14 of the 17 malignant （82.4%）. No expression of Cox-2 was observed in control slides. There were significant differences of Cox-2 expression between benign and malignant pheochromocytomas, as well as between malignant pheochromocytomas and control （ P 〈0. 05）. Expressions of MVD were 36. 41 ±13. 00, 21.43 ±8. 05, and 13. 36 ±4.34 in malignant, benign pheochromocytomas, and in control, respectively. Conclusion Cox-2 may contribute to the invasive characteristics of malignant pheochromocytomas and be used as a marker to distinguish malignant from benign pheochromocytomas. Expression of MVD in malignant pheochromocytomas was directly correlated with Cox-2.
文摘BACKGROUND Phaeochromocytoma and paragangliomas(PPGL)are rare neuroendocrine tumors usually caused by parasympathetic or sympathetic nerves,with an overall incidence of approximately 0.66 cases per 100000 people per year.Most PPGLs are asymptomatic,and a few develop symptoms,such as elevated blood pressure and rapid heart rate,because of the release of catecholamines.According to the literature,surgical resection is the mainstay of PPGL treatment.However,the choice between minimally invasive surgery and open surgery remains controversial,particularly in cases involving complex anatomical relationships.We successfully resected a tumor located between the inferior vena cava and abdominal aorta using a minimally invasive approach with the assistance of computed tomography(CT)angiography and three-dimensional reconstruction,resulting in a favorable outcome.CASE SUMMARY A 56-year-old woman was admitted to the hospital with right upper abdominal discomfort for more than 6 months,experiencing occasional pins and needles sensation and radiation from the right shoulder and back.Prehospital CT of the upper abdomen revealed a retroperitoneal mass suspected to be a retroperitoneal ganglioneuroma.Surgery was recommended,and an enhanced CT scan of the upper abdomen,along with preoperative three-dimensional reconstruction,was performed after admission.The imaging indicated that the mass,measuring approximately 4.1 cm×3.8 cm×4.8 cm,was situated between the abdominal aorta and the inferior vena cava,extending downward to the level of the left renal vein.After ruling out any contraindications to surgery,a minimally invasive laparoscopy was performed to excise the mass precisely.The surgery was successful without any postoperative complications,and the 2-month follow-up revealed no abnormal signs of recurrence.CONCLUSION This case report describes successful and precise laparoscopic resection of a retroperitoneal tumor.The patient recovered well during the 2-month follow-up,and postoperative pathology revealed a paraganglioma.
文摘BACKGROUND Pheochromocytoma(PHEO)is a type of tumor that originates from chromaffin cells in the adrenal medulla and is classified as an adrenal paraganglioma.PHEOs can secrete catecholamines,leading to a variety of symptoms.Accurate diagnosis and appropriate treatment selection are crucial for favorable outcomes in these cases.CASE SUMMARY The patient presented with unexplained chest tightness,palpitations,and pink sputum.Upon examination and analysis of laboratory results,a diagnosis of adrenal PHEO was established.The PHEO secreted high levels of catecholamines,causing sudden fluctuations in blood pressure and heart rate,leading to extre-mely unstable hemodynamics.Treatment with extracorporeal membrane oxygenation and intra-aortic balloon counterpulsation helped stabilize the patient’s vital signs,allowing for timely surgical intervention.CONCLUSION The combination of extracorporeal membrane oxygenation and intra-aortic balloon counterpulsation can enhance tissue perfusion,thus providing a solid foundation for the accurate diagnosis and effective surgical treatment of PHEO.
文摘Pheochromocytoma (PHEO) is a rare endocrine tumor from the chromaffin cells in the adrenomedullary gland and sympathetic/parasympathetic ganglia, secreting one or more catecholamines. It is frequently associated with hypertension and described in the literature as a cause of secondary diabetes mellitus. In patients with known persistent uncontrolled diabetes, PHEO is rarely mentioned as the cause of uncontrolled diabetes. The authors report a rare case of PHEO diagnosed in a 64-year-old female patient treated for 10 years with type 2 diabetes mellitus and hypertension. She was treated using a combination of insulin (2 injections) and Metformin 1000 mg twice a day. The glycemic control was poor (HbA1c-11%), and persistent High Blood Pressure (HBP). She presented with unexplained weight loss associated with permanent hyperhidrosis (sweating), affecting her quality of life and diffuse abdominal pain. The investigations confirmed the diagnosis of PHEO, which resection led to improvement of glycemic control and hypertension.
基金Supported by National Natural Science Foundation of China,No.82200353Jiangsu Province Double Innovation Doctoral Program,No.JSSCBS20221948+4 种基金Suzhou Gusu Health Talent Program,No.(2022)043Suzhou Gusu Health Talent Plan Talent Research Project,No.GSWS2022014Suzhou Science and Technology Innovation Policy Funding Projectthe Jiangsu Province College Students’Innovation and Entrepreneurship Training Program Project,No.202410285087Z“Bo Xi”Talent Casting Plan of the First Affiliated Hospital of Soochow University.
文摘BACKGROUND Pheochromocytoma,a rare catecholamine-secreting tumor,typically presents with the classic triad of headache,palpitations,and diaphoresis,often accompanied by cardiovascular manifestations.While vomiting occurs in approximately 34.5%of cases,it is rarely the predominant and persistent presenting symptom.Pheochro-mocytoma-induced cardiomyopathy leading to heart failure is a recognized but uncommon complication.Due to its heterogeneous presentations,misdiagnosis and diagnostic delay are frequent.CASE SUMMARY A 53-year-old female presented predominantly with persistent and refractory vomiting as her chief complaint,accompanied by signs of acute heart failure[left ventricular ejection fraction(LVEF)30%].Initial evaluation at a primary hospital,including coronary angiography(revealing only mild stenosis),led to a misdia-gnosis of coronary artery disease.Despite standard anti-thrombotic,anti-heart failure,and anti-emetic therapy,her vomiting persisted and heart failure did not resolve.Subsequent hospitalization revealed dramatic paroxysmal hypertension(202/129 mmHg to 97/51 mmHg)and fever.Significantly elevated plasma meta-nephrines and normetanephrine,combined with abdominal computed tomogra-phy and magnetic resonance imaging,confirmed a right adrenal pheochromo-cytoma.This diagnosis was significantly delayed due to the atypical prominence of gastrointestinal symptoms masking the underlying endocrine crisis.CONCLUSION This case highlights a highly atypical presentation of pheochromocytoma domi-nated by refractory vomiting and complicated by acute catecholamine-induced cardiomyopathy.It emphatically underscores that pheochromocytoma must be considered in the differential diagnosis for patients presenting with unexplained,treatment-resistant vomiting,particularly when co-existing with acute heart failure.The presence of labile hypertension,even if not initially evident,provides a crucial diagnostic clue.Prompt biochemical screening(catecholamine metabolites)and adrenal imaging are essential to prevent diagnostic delay and enable timely,life-saving surgical intervention.
文摘Nonfunctional pheochromocytoma is a particular type of pheochromocytoma without typical clinical features of catecholaminism. It is difficult to diagnose or differentially diagnose. This type of pheochromocytoma is rarely reported in the literature. From January 1, 1990 to June 1, 2004, we performed operations for 197 patients with pheochromocytomas, including 26 patients with nonfunctional pheochromocytoma. We analyzed the clinical data of the 26 patients.
基金Supported by The Finance Bureau of Dongguan City,Guangdong Province.
文摘BACKGROUND Multiple endocrine neoplasia type 2(MEN2)is a rare,autosomal dominant endocrine disease.Currently,the RET proto-oncogene is the only gene implicated in MEN2A pathogenesis.Once an RET carrier is detected,family members should be screened to enable early detection of medullary thyroid carcinoma,pheochromocytoma,and hyperparatitity.Among these,medullary thyroid carcinoma is the main factor responsible for patient mortality.Accordingly,delineating strategies to inform clinical follow-up and treatment plans based on genes is paramount for clinical practitioners.CASE SUMMARY Herein,we present RET proto-oncogene mutations,clinical characteristics,and treatment strategies in a family with MEN2A.A family study was conducted on patients diagnosed with MEN2A.DNA was extracted from the peripheral blood of family members,and first-generation exon sequencing of the RET protooncogene was conducted.The C634Y mutation was identified in three family members spanning three generations.Two patients were sequentially diagnosed with pheochromocytomas and bilateral medullary thyroid carcinomas.A 9-yearold child harboring the gene mutation was diagnosed with medullary thyroid carcinoma.Surgical resection of the tumors was performed.All family members were advised to undergo complete genetic testing related to the C634Y mutation,and the corresponding treatments administered based on test results and associated clinical guidelines.CONCLUSION Advancements in MEN2A research are important for familial management,assessment of medullary thyroid cancer invasive risk,and deciding surgical timing.
文摘Approximately 40% of pheochromocytoma and paraganglioma(PPGL) cases are familial, typically presenting earlier with more complex symptoms. This paper synthesizes literature and guidelines to inform on clinical characteristics and perioperative care for PPGL. Pheochromocytoma in von Hippel-Lindau(VHL) disease exhibits heightened secretion activity without significant perioperative hemodynamic changes. Tumors in multiple endocrine neoplasia type 2(MEN2) have a stronger endocrine function, which may induce hemodynamic fluctuations during surgery. Therefore, pheochromocytoma screening is essential at all stages of MEN2. Neurofibromatosis type 1(NF1) often presents multisystem lesions and can result in difficult airway. Pheochromocytoma should be evaluated when NF1 patients present hypertension. Pheochromocytoma and paraganglioma type 5 may present multiple lesions of pheochromocytoma or paraganglioma. In summary, hereditary PPGLs may present with severe lesions in other systems, beyond tumor function. A multi-disciplinary team(MDT) approach is often invaluable in perioperative management.
基金supported by the National Natural Science Foundation of China(No.82072811).
文摘Pheochromocytomas and paragangliomas(PPGLs)cause symptoms by altering the circulation levels of catecholamines and peptide hormones.Currently,the diagnosis of PPGLs relies on diagnostic imaging and the detection of catecholamines.In this study,we used ultra-performance liquid chromatography(UPLC)/quadrupole time-of-flight mass spectrometry(Q-TOF MS)analysis to identify and measure the perioperative differential metabolites in the plasma of adrenal pheochromocytoma patients.We identified differentially expressed genes by comparing the transcriptomic data of pheochromocytoma with the normal adrenal medulla.Through conducting two steps of metabolomics analysis,we identified 111 differential metabolites between the healthy group and the patient group,among which 53 metabolites were validated.By integrating the information of differential metabolites and differentially expressed genes,we inferred that the cysteine-methionine,pyrimidine,and tyrosine metabolism pathways were the three main metabolic pathways altered by the neoplasm.The analysis of transcription levels revealed that the tyrosine and cysteine-methionine metabolism pathways were downregulated in pheochromocytoma,whereas the pyrimidine pathway showed no significant difference.Finally,we developed an optimized diagnostic model of two metabolites,L-dihydroorotic acid and vanylglycol.Our results for these metabolites suggest that they may serve as potential clinical biomarkers and can be used to supplement and improve the diagnosis of pheochromocytoma.
文摘BACKGROUND Paraganglioma(PGL)located in the retroperitoneum presents challenges in diagnosis and treatment due to its hidden location,lack of specific symptoms in the early stages,and absence of distinctive manifestations on imaging.CASE SUMMARY A 56-year-old woman presented with a left upper abdominal mass discovered 1 wk ago during a physical examination.She did not have a history of smoking,alcohol consumption,or other harmful habits,no surgical procedures or infectious diseases,and had a 4-year history of hypertension.Upon admission,she did not exhibit fever,vomiting,or abdominal distension.Physical examination indicated mild percussion pain in the left upper abdomen,with no palpable enlargement of the liver or spleen.Laboratory tests and tumor markers showed no significant abnormalities.Enhanced computed tomography and magnetic resonance imaging of the upper abdomen revealed a cystic solid mass in the left epigastrium measuring approximately 6.5 cm×4.5 cm,with inhomogeneous enhancement in the arterial phase,closely associated with the lesser curvature of the stomach and the pancreas.The patient underwent laparoscopic resection of the retroperitoneal mass,which was successfully removed without tumor rupture.A 12-month postoperative follow-up period showed good recovery.CONCLUSION This case report details the successful laparoscopic resection of a retroperitoneal subclinical PGL,resulting in a good recovery observed at the 12-month follow-up.Interestingly,the patient also experienced unexpected cure of hypertensive disease.
文摘Different types of neuroendocrine cancer,including medullary thyroid cancer(MTC)and thyroid C-cell hyperplasia,are part of multiple endocrine neoplasia type 2(MEN2).A proto-oncogene mutation of the rearranged during transfection(RET)gene changes the way that receptor tyrosine kinases work.Multiple endocrine neoplasia,a pathological condition,involves these kinases.When the RET protooncogene changes,it can cause endocrine adenomas and hyperplasia to happen at the same time or one after the other.Pheochromocytoma,medullary thyroid carcinoma,and hyperparathyroidism,alone or in combination,are present in MEN2A patients.Some patients may also have skin lichen amyloidosis or Hirschsprung's disease.Patients with MEN2A often present with MTC.MTC is aggressive and has the worst prognosis,as most patients exhibit lymph node metastasis.MTC is one of the important causes of death in patients with MEN2A.RET mutation analysis aids in identifying MEN2A symptoms and monitoring levels of calcium,thyroid hormones,calcitonin,normetanephrine,fractionated metanephrines,and parathyroid hormone.The earlier diagnosis of MTC significantly improves survival and prompts better management of MEN2A.In this editorial,we will discuss the significance of molecular diagnostic approaches in detecting RET oncogene mutations in MEN2A.
基金supported by the Project of the 940 Hospital of the Joint Logistics Support Force of the Chinese PLA(no.2021yxky057).
文摘Objective:The aim of the study was to investigate effective diagnostic molecular markers and the specific mechanisms of metastatic pheochromocytomas and paragangliomas(PPGLs).Methods:Data were collected from GEO datasets GSE67066 and GSE60458.The R software and various packages were utilized for the analysis of differentially expressed genes,Gene Ontology analysis,Kyoto Encyclopedia of Genes and Genomes analysis,receiver operating characteristic curve assessment,logistic model construction,and correlation analysis.The NetworkAnalyst tool was used to analyze gene-miRNA interactions and signaling networks.In addition,the TIMER database was used to estimate the immune scores.Results:A total of 203 and 499 differentially expressed genes were identified in GSE67066 and GSE60458,respectively.These genes are implicated in cytokine and cytokine receptor interactions,extracellular matrix–receptor interactions,and platelet activation signaling pathways.Notably,MAMLD1,UST,MATN2,LPL,TWIST1,SFRP4,FRMD6,RBM24,PRIMA1,LYPD1,KCND2,CAMK2N1,SPOCK3,and ALPK3 were identified as the key genes.Among them,MATN2 and TWIST1 were found to be coexpressed with epithelial-mesenchymal transition–linked markers,whereas KCND2 and LPL exhibited associations with immune checkpoint expression and immune cell infiltration.Eight miRNAs were identified as potential regulators of key gene expression,and it was noted that TWIST1 might be regulated by SUZ12.Notably,the area under the curve of the 4-gene model for distinguishing between malignant and benign groups was calculated to be 0.918.Conclusions:The combined gene and mRNA expression model enhances the diagnostic accuracy of assessing PPGL metastatic potential.These findings suggest that multiple genes may play a role in the metastasis of PPGLs through the epithelial-mesenchymal transition and may influence the immune microenvironment.
文摘Introduction: Adrenal tumours (AT) are commonly encountered in clinical practice. For any patient presenting an adrenal mass, there are two crucial points to consider: is the adrenal mass malignant and is it hormonally active? The objective of our study was to evaluate the epidemiological, diagnostic, therapeutic, and evolutionary aspects of AT in a developing country. Methodology: We conducted a retrospective study spanning 17 years (from January 2005 to October 2022) in four Dakar hospital services. We included all patient medical records with explored AT. Results: AT was diagnosed in 35 patients who had a mean age of 36.62 years (range: 12-79), and a female predominance (26 women to 9 men). Among these patients, 17.2 % had incidental AT. Hypertension was the commonest presenting symptom in 27 patients (77%), which was associated with Mnards triad in 14 patients (40%). Abdominal pain was reported by 20 patients (57%), and 14 presented with an abdominal mass. Among the patients, 29 had functional AT: 22 with pheochromocytoma, 5 with primary hyperaldosteronism, and 2 with hypercortisolism. Imaging evaluations revealed that 33 patients had unilateral AT and one had bilateral AT. Tumour sizes varied, 5 patients had tumours 10 cm. Two patients presented with metastases. The therapeutic approach involved adrenalectomy in 32 patients. Perioperative complications were observed in five patients, including haemorrhage (two patients), hypotension (two cases), and hypertensive crisis (one case). One patient with bilateral pheochromocytoma developed acute adrenal insufficiency postoperatively, followed by abdominal herniation. Histopathological examination confirmed pheochromocytoma in 21 patients, Conns adenoma in 5, adrenocortical carcinoma in 3, cortisol adenoma in 2, and adrenal cyst in 1 patient. Non-functional AT included adrenocortical carcinoma (three patients), adrenal cyst (one patient), and pheochromocytoma (two patients). After a one-year follow-up, 29 patients with benign tumours had favourable outcomes, while death occurred within six months of diagnosis in five cases. A patient who had bilateral pheochromocytomas, with complications, died before surgery. Conclusion: In our practice, AT are predominantly pheochromocytomas, being typically diagnosed when they become symptomatic large tumours and, due to this large size, laparotomy is the preferred surgical approach. Unfortunately, the prognosis for malignant tumours is poor.
文摘Introduction: Pheochromocytoma is a rare cause of secondary arterial hypertension whose clinical presentation can be multifaceted. In particular, it may be revealed or complicated by cardiovascular manifestations independent of hypertension. These include Tako-Tsubo cardiomyopathy, an acute reversible dyskinetic cardiomyopathy associated with ballooning of the left ventricular apex. Observation: We present the case of a 32-year-old woman, diabetic for 2 years. Her history included untreated labile hypertension and emotional stress. She was admitted to a cardiac intensive care unit for left heart failure. Paraclinical investigations confirmed the diagnosis of Tako-Tsubo cardiomyopathy induced by pheochromocytoma. After a few days of medical treatment in the ICU, the outcome was favorable, with well-compensated heart failure, stable hemodynamics and restoration of left ventricular function. Conclusion: This observation raises the issue of delayed diagnosis of a pheochromocytoma revealed by a TTC. Despite its spectacular initial presentation, the evolution of the latter was rather favorable under appropriate treatment.
文摘Pheochromocytomas or paragangliomas are rare and the diagnosis remains a challenge for clinicians.We present a woman suffering from recurrent myocardial infarction and cardiomyopathy,who was finally diagnosed with paraganglioma.Pheochromocytomas or paraganglioma should be considered in the differential diagnosis of a patient with symptoms possibly referring to acute coronary syndrome and cardiomyopathy.
基金Project (No. 2005C30059) supported by the Science and TechnologyProgram of Zhejiang Province, China
文摘Background： Edaravone had been validated to effectively protect against ischemic injuries. In this study, we investigated the protective effect of edaravone by observing the effects on anti-apoptosis, regulation of Bcl-2/Bax protein expression and recovering from damage to mitochondria after OGD （oxygen-glucose deprivation）-reperfusion. Methods： Viability of PC 12 cells which were injured at different time of OGD injury, was quantified by measuring MTT （2-（4,5-dimethylthia-zol-2-yl）-2,5-diphenyltetrazolium bromide） staining. In addition, PC 12 cells＇ viability was also quantified after their preincubation in different concentration of edaravone for 30 min followed by （OGD）. Furthermore, apoptotic population of PC 12 cells that reinsulted from OGD-reperfusion with or without preincubation with edaravone was determined by flow cytometer analysis, electron microscope and Hoechst/Pl staining. Finally, change of Bcl-2/Bax protein expression was detected by Western blot. Results： （1） The viability of PC12 cells decreased with time （1-12 h） after OGD. We regarded the model of OGD 2 h, then replacing DMEM （Dulbecco＇s Modified Eagle＇s Medium） for another 24 h as an OGD-reperfusion in this research. Furthermore, most PC 12 cells were in the state of apoptosis after OGD-reperfusion. （2） The viability of PC 12 cells preincubated with edaravone at high concentrations （1, 0.1, 0.01 μmol/L） increased significantly with edaravone protecting PC 12 cells from apoptosis after OGD-reperfusion injury. （3） Furthermore, edaravone attenuates the damage of OGD-reperfusion on mitochondria and regulated Bcl-2/Bax protein imbalance expression after OGD-reperfusion. Conclusion： Neuroprotective effects of edaravone on ischemic or other brain injuries may be partly mediated through inhibition of Bcl-2/Bax apoptotic pathways by recovering from the damage of mitochondria.
文摘The measurement of urine catecholamine and metanephrine concentrations is important for biochemical screening and diagnosis of pheochromocytoma.The goal of this work was to develop a simple liquid chromatography-tandem mass spectrometry(LC-MS/MS)method for determining catecholamines and metanephrines in urine to replace an existing liquid chromatographic method using electrochemical detection.Urine samples were prepared using Oasis weak-cation-exchange cartridges.The eluate was analyzed on an Agilent ZORBAX Eclipse Plus Phenyl-Hexyl column in 3 min.Adrenaline,noradrenaline,dopamine,metanephrine,normetanephrine,and their deuterated internal standards were monitored in positive electrospray ionization mode by multiple reaction monitoring(MRM).No evidence of ion suppression was observed.The assay was linear up to 5μmol/L for adrenaline,5μmol/L for noradrenaline,6.1μmol/L for dopamine,5.6μmol/L for metanephrine,and 34.6μmol/L for normetanephrine,with lower limits of quantification of 5,5,12,6 and 7nmol/L,respectively.The intra-day and inter-day precisions for all analytes ranged from 0.59%to 4.64%and1.98%to 4.80%,respectively.External quality assurance samples were assayed and showed excellent agreement with the target values.This simple method provides an improved assay for determining urine catecholamines and metanephrines.
文摘Objective： To study the diagnosis and treatment ofpheochromocytoma in urinary bladder. Methods： Six cases of bladder pheochromocytoma were studied. Four cases showed hypertension, 3 of which were paroxysmal hypertension during urination. Catecholamine （CA） was increased in a case, and vanillymandelic acid （VMA） was increased in 2 cases. Bladder submucosal mass was detected by B-ultrasound in 5 cases （5/5）, computerized tomography （CT） in 3 cases （3/3）, cystoscopy in 5 cases （5/6）. Four cases took a-receptor blocker for 2 weeks, 1 case took β-receptor blocker to decrease heart rate. All patients were treated with surgical operation including 4 partial cystectomies, 2 excavations. Results： Three cases had manifestations including headache, excessive perspiration and hypertension during cystoscopy. Four cases were confirmed before operation. Two cases showed hypertension during operation. All patients were pathologically diagnosed as pheochromocytoma post- operatively. In five cases followed up, blood pressure returned to normal. No patient had relapse and malignancy. Conclusions： Typical hypertension during urination comprised the main symptoms. We should highly suspect bladder pheochromocytoma if a submucosal mass was discovered with B-ultrasound, CT, ^131I-M1BG （methyliodobenzylguanidine） and cystoscopy. The determination of CA in urine is valuable for qualitative diagnosis. The preoperative management of controlling blood pressure and expansion of the blood volume are very important. Surgical operation is a good method for effective treatment. Postoperative long-time followed up is necessary.
文摘The coincidence of a gastrointestinal stromal tumor (GIST) and a neuroendocrine tumor (NET) in neurofibromatosis type 1 (NF1) is described only five times within the literature. We report on a 63 year old Caucasian female with the rare condition of neurofibromatosis type 1 coinciding with recurrent gastrointestinal stromal tumor plus bilateral pheochromocytoma (PCC). After a history of palpitations and dizziness that lasted for years, a left adrenal mass was detected by CT. Laparotomy revealed a pheochromocytoma of the left adrenal gland while an ileoterminal GIST was found incidentally intraoperatively. After six months contralateral PCC and multiple recurrent GIST were resected again. After four years the patient is doing well without any signs of further recurrent tumors. Discussion includes review of the literature.
