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
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
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: 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
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
Pheochromocytoma is a tumor arising from neuroectodermal chromaffin tissues in the adrenal gland or extra-adrenal paraganglia(paragangliomas). The prevalence of the tumor is 0.1%-0.6% in the hypertensive population, o...Pheochromocytoma is a tumor arising from neuroectodermal chromaffin tissues in the adrenal gland or extra-adrenal paraganglia(paragangliomas). The prevalence of the tumor is 0.1%-0.6% in the hypertensive population, of which 10%-20% are malignant. Pheochromocytoma produces, stores, and secretes catecholamines, as well as leads to hypertensive crisis, arrhythmia, angina, and acute myocardial infarction without coronary artery diseases. We report a case of acute coronary syndrome(ACS) with a final diagnosis of multiple endocrine neoplasia with pheochromocytoma and medullary thyroid carcinoma(MTC).展开更多
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
BACKGROUND Von Hippel-Lindau disease(also known as VHL syndrome),is an autosomal dominant inherited disease.We describe a sporadic case of VHL syndrome where bilateral pheochromocytomas were unexpectedly identified.Th...BACKGROUND Von Hippel-Lindau disease(also known as VHL syndrome),is an autosomal dominant inherited disease.We describe a sporadic case of VHL syndrome where bilateral pheochromocytomas were unexpectedly identified.The patient underwent selective laparoscopic resections of the pheochromocytomas,and the anesthetic management during surgery was complex and challenging.CASE SUMMARY A 22-year-old man presented to our hospital to seek medical advice for infertility without any other complaints.The results of computed tomography and catecholamine levels in blood and urine demonstrated adrenal gland masses which were diagnosed as pheochromocytomas.Further examination confirmed that the patient also had VHL syndrome.After thorough preparation,the patient underwent selective laparoscopic resection of the pheochromocytomas and was discharged 10 d after surgery.We describe the process of perioperative anesthesia management in this patient undergoing pheochromocytoma resection.CONCLUSION This case summaries specific clinical traits and considerations in perioperative anesthesia management for VHL syndrome patients undergoing bilateral pheochromocytoma resection.展开更多
Objective To investigate the clinical and genetic features of a Chinese family with yon Hippel- Lindau （VHL） disease revealed by bilateral pheochromocytoma. Methods The proband and other members in a Chinese family...Objective To investigate the clinical and genetic features of a Chinese family with yon Hippel- Lindau （VHL） disease revealed by bilateral pheochromocytoma. Methods The proband and other members in a Chinese family with familial pheochromocytoma were clinically evaluated and followed up. Genomic DNA extracted from the peripheral blood of 8 family members （including 3 patients） was amplified by polymerase chain reaction （PCR） and the PCR products were directly sequenced. Results The first presentation in the proband, his mother, and his sister was bilateral pheochromocytoma, and the missense mutation of 695G-A （Arg161Gln） in exon 3 of VHL gene was detected in the three patients. In the follow-up study, the proband and his mother were found to have other VHL tumors, induding retinal and cerebellar hemangioblastomas and pancreatic tumor. Neither clinical presentation of VHL disease nor gene mutation was found in other family members. Conclusion VHL disease should be suspected in some patients with familial pheochromocytoma, and VHL gene screening helps to achieve early diagnosis of the disease.展开更多
Objective:Clinical practice guidelines recommend open adrenalectomy(OA)for large pheochromocytoma(LPCC)>6 cm in size.Although laparoscopic adrenalectomy(LA)for the treatment of LPCC has been reported,its role remai...Objective:Clinical practice guidelines recommend open adrenalectomy(OA)for large pheochromocytoma(LPCC)>6 cm in size.Although laparoscopic adrenalectomy(LA)for the treatment of LPCC has been reported,its role remains unclear.This study aimed to compare the effectiveness of LA and OA,and summary the surgical treatment experience.Methods:Data concerning LPCC,from January 2010 to June 2019 of a single institution,were retrospectively reviewed.Altogether 82 patients with a tumor larger than 6 cm were included(52 patients in LA group and 30 patients in OA group).Groups were balanced by propensity score matching(PSM)into 15 pairs.Patients’demographics,preoperative characteristics,and prognosis were analyzed.Results:Before PSM,the OA group had larger tumor sizes(median[interquartile range,IQR]:8.9[7.3-10.3]vs.7.2[6.7-8.0]cm;p=0.000)and higher vanillylmandelic acid level(median[IQR]:114.3[67.8-326.4]vs.66.6[37.8-145.8]μmol/24 h;p=0.004)and needed a higher cumulative dose of prazosin(median[IQR]:83.5[37.0-154.0]vs.38.0[21.0-81.0]mg;p=0.028).After PSM,the baseline data showed no significant differences between both groups.The LA group had relatively more stable blood pressure in surgery,with a lower fluctuation of systolic blood pressure(mean±standard deviation[SD]:70.9±25.1 vs.107.4±46.2 mmHg,p=0.012)and a lower percentage of hemodynamic instability(46.7%vs.86.7%,p=0.020).The LA group had shorter postoperative hospital stays(mean±SD:6.4±2.7 vs.10.1±3.4 days;p=0.003)than the OA group.Differences regarding metastasis rate(6.7%vs.0,p=1.000)were not statistically significant between LA and OA groups.The median(IQR)follow-up time of 82 patients was 72.5(47.0-103.5)months.Binary logistic regression showed that right-side tumors or those>8 cm in size were independent risk factors of OA.Conclusion:LA is a safe,minimally invasive procedure for LPCC and has relatively better perioperative characteristics in large medical centers.Patients with tumors on the right side or larger than 8 cm are more likely to undergo OA initially.展开更多
Vasoactive intestinal peptide-producing tumors （VIP-oma） usually originate in the pancreas and are chara-cterized by diarrhea, hypokalemia, and achlorhydria （WDHA syndrome）. In adults, nonpancreatic VIPoma is very...Vasoactive intestinal peptide-producing tumors （VIP-oma） usually originate in the pancreas and are chara-cterized by diarrhea, hypokalemia, and achlorhydria （WDHA syndrome）. In adults, nonpancreatic VIPoma is very rare. Herein, we report an unusual case of VIP-producing pheochromocytoma marked by persistent shock, fushing, and watery diarrhea and high sensitivity to octreotide. A 53-year-old woman was hospitalized for sudden-onset hypertension with convulsions, which then rapidly evolved to persistent shock, fushing, and watery diarrhea. Abdominal computed tomography indicated a left adrenal mass, accompanied by bleeding;and marked elevations of both plasma catecholamine and VIP concentrations were documented via laboratory testing. Surprisingly, all clinical symptoms responded swiftly to octreotide treatment. Once surgically treated, hormonal levels normalized in this patient, and the clinical symptoms dissipated. Postoperative pathological and immunohistopathological studies confrmed a VIP-secreting pheochromocytoma with strong, diffuse positivity for somatostatin receptor type 2. During a 6-mo follow-up period, she seemed in good health andwas symptom-free.展开更多
Bladder pheochromocytoma is the most common extra-adrenal genitourinary tumor.Endoscopic management is feared due to the risk of intra-operative hypertensive crisis.We described a case of successful endoscopic managem...Bladder pheochromocytoma is the most common extra-adrenal genitourinary tumor.Endoscopic management is feared due to the risk of intra-operative hypertensive crisis.We described a case of successful endoscopic management of a bladder pheochromocytoma and discussed its technical aspects.展开更多
AIM To investigate the occurrence of cardiomyopathy(CMP)in a cohort of patients with histologically proven pheochromocytoma(pheo),and to determine if catecholamine excess was causative of the left ventricular(LV)dysfu...AIM To investigate the occurrence of cardiomyopathy(CMP)in a cohort of patients with histologically proven pheochromocytoma(pheo),and to determine if catecholamine excess was causative of the left ventricular(LV)dysfunction.METHODS A retrospective chart review spanning years 1998through 2014 was undertaken and patients with a diagnosis of pheo confirmed with histopathologic examination were included.Presenting electrocardiograms and cardiac imaging studies were reviewed.Transthoracic echocardiography(TTE),ventriculography or single positron emission computed tomography imaging was evaluated and if significant abnormalities[left ventricular hypertrophy(LVH)or LV dysfunction]were noted in the pre operative period a follow up post-operative study was also analyzed.Multivariate analysis using logistic regression was used to investigate independent predictors for outcomes of interest,LV dysfunction and LVH.RESULTS We identified 18 patients with diagnosis of pheo confirmed on pathology.Mean age was 54.3±19.3years and 11(61.1%)patients were females.50%of such patients had either resistant hypertension or labile blood pressures during hospitalization,which had raised suspicion for a pheo.Cardiac imaging studies were available for 12(66.7%)patients at the time of inclusion into study and preceding the adrenalectomy.7(58.3%)patients with a TTE available for review had mild or more severe LVH while 3(25%)patients had LV dysfunction of presumably acute onset.In a multivariate analysis,elevated catecholamine levels as assessed by urinary excretion of metabolites was not an independent predictor of development of LV systolic dysfunction or of presence of LVH on TTE.Two female patients with a preceding history of hypertension had marked LV hypertrophy and systolic anterior motion of the mitral valve.Prolongation of the QTc interval was noted in 5(27.8%)patients but no acute arrhythmias were observed in any patient.CONCLUSION This study adds to the growing body of literature on the predilection of patients with pheochromocytomas to develop non-ischemic CMP.Degree of catecholamine excess as measured by urinary secretion of metabolites did not predict the development of CMP but 2 of 3patients developed CMP in the setting of significant acute physiologic stress.Our findings provide support to the proposed etiologic role of elevated catecholamines in TC and other stress induced forms of CMP,however,activation of a brain-neural-cardiac axis from acute stress and local release of catecholamines but not chronic catecholamine elevations are likely to be responsible in pheo related CMP.展开更多
BACKGROUND Malignant pheochromocytoma with cerebral and skull metastasis is a very rare disease.Combining our case with 16 previously reported cases identified from a PubMed search,an analysis of 17 cases of malignant...BACKGROUND Malignant pheochromocytoma with cerebral and skull metastasis is a very rare disease.Combining our case with 16 previously reported cases identified from a PubMed search,an analysis of 17 cases of malignant cerebral pheochromocytoma was conducted.This literature review aimed to provide information on clinical manifestations,radiographic and histopathological features,and treatment strategies of this condition.CASE SUMMARY A 60-year-old man was admitted with a progressive headache and enlarging scalp mass lasting for 3 mo.Radiographic images revealed a left temporal biconvexshaped epidural mass and multiple lytic lesions.The patient underwent a left temporal craniotomy for resection of the temporal tumor.Histopathological analysis led to identification of the mass as malignant pheochromocytoma.The patient’s symptoms were alleviated at the postoperative 3-mo clinical follow-up.However,metastatic pheochromocytoma lesions were found on the right 6th rib and the 6th to 9th thoracic vertebrae on a 1-year clinical follow-up computed tomography scan.CONCLUSION Magnetic resonance spectroscopy and histopathological examination are necessary to make an accurate differential diagnosis between malignant cerebral pheochromocytoma and meningioma.Surgery is regarded as the first choice of treatment.展开更多
Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was refer...Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. After surgical resection of the tumor, diarrhea subsided and both electrolytes and affected hormone levels normalized. Immunohistochemical examination confirmed a diagnosis of pheochromocytoma, which contained VIP-positive ganglion-like cells. We herein present the clinical and histogenetic implications of this rare clinical entity, with literature review.展开更多
BACKGROUND Pheochromocytomas are rare endocrine tumors with various clinical manifestations,and few of them might present with profound,life-threatening conditions.CASE SUMMARY We report the case of a 65-year-old man ...BACKGROUND Pheochromocytomas are rare endocrine tumors with various clinical manifestations,and few of them might present with profound,life-threatening conditions.CASE SUMMARY We report the case of a 65-year-old man who complained of sudden dyspnea and hemoptysis for half a day.There was no obvious cause for the patient to have dyspnea,coughing,or coughing up to approximately 100 mL of fresh blood.Finally,he was diagnosed with pheochromocytoma crisis(PCC),coexisting with an abdominal aortic aneurysm(AAA).CONCLUSION We report a case of pheochromocytoma presenting with recurrent hemoptysis,dyspnea and hypotension coexisting with an AAA.It not only proved the uncommon manifestations of pheochromocytoma but also directed clinicians to consider PCC among the possible diagnoses when meeting similar cases.Moreover,surgical excision is the most beneficial method for the treatment of pheochromocytoma coexisting with AAA when the situation is stable.展开更多
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.
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: 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.
文摘Pheochromocytoma is a tumor arising from neuroectodermal chromaffin tissues in the adrenal gland or extra-adrenal paraganglia(paragangliomas). The prevalence of the tumor is 0.1%-0.6% in the hypertensive population, of which 10%-20% are malignant. Pheochromocytoma produces, stores, and secretes catecholamines, as well as leads to hypertensive crisis, arrhythmia, angina, and acute myocardial infarction without coronary artery diseases. We report a case of acute coronary syndrome(ACS) with a final diagnosis of multiple endocrine neoplasia with pheochromocytoma and medullary thyroid carcinoma(MTC).
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BACKGROUND Von Hippel-Lindau disease(also known as VHL syndrome),is an autosomal dominant inherited disease.We describe a sporadic case of VHL syndrome where bilateral pheochromocytomas were unexpectedly identified.The patient underwent selective laparoscopic resections of the pheochromocytomas,and the anesthetic management during surgery was complex and challenging.CASE SUMMARY A 22-year-old man presented to our hospital to seek medical advice for infertility without any other complaints.The results of computed tomography and catecholamine levels in blood and urine demonstrated adrenal gland masses which were diagnosed as pheochromocytomas.Further examination confirmed that the patient also had VHL syndrome.After thorough preparation,the patient underwent selective laparoscopic resection of the pheochromocytomas and was discharged 10 d after surgery.We describe the process of perioperative anesthesia management in this patient undergoing pheochromocytoma resection.CONCLUSION This case summaries specific clinical traits and considerations in perioperative anesthesia management for VHL syndrome patients undergoing bilateral pheochromocytoma resection.
基金Supported by the "tenth five-years " National Science and Technology Tackle Key Project (2004BA720A29)
文摘Objective To investigate the clinical and genetic features of a Chinese family with yon Hippel- Lindau （VHL） disease revealed by bilateral pheochromocytoma. Methods The proband and other members in a Chinese family with familial pheochromocytoma were clinically evaluated and followed up. Genomic DNA extracted from the peripheral blood of 8 family members （including 3 patients） was amplified by polymerase chain reaction （PCR） and the PCR products were directly sequenced. Results The first presentation in the proband, his mother, and his sister was bilateral pheochromocytoma, and the missense mutation of 695G-A （Arg161Gln） in exon 3 of VHL gene was detected in the three patients. In the follow-up study, the proband and his mother were found to have other VHL tumors, induding retinal and cerebellar hemangioblastomas and pancreatic tumor. Neither clinical presentation of VHL disease nor gene mutation was found in other family members. Conclusion VHL disease should be suspected in some patients with familial pheochromocytoma, and VHL gene screening helps to achieve early diagnosis of the disease.
文摘Objective:Clinical practice guidelines recommend open adrenalectomy(OA)for large pheochromocytoma(LPCC)>6 cm in size.Although laparoscopic adrenalectomy(LA)for the treatment of LPCC has been reported,its role remains unclear.This study aimed to compare the effectiveness of LA and OA,and summary the surgical treatment experience.Methods:Data concerning LPCC,from January 2010 to June 2019 of a single institution,were retrospectively reviewed.Altogether 82 patients with a tumor larger than 6 cm were included(52 patients in LA group and 30 patients in OA group).Groups were balanced by propensity score matching(PSM)into 15 pairs.Patients’demographics,preoperative characteristics,and prognosis were analyzed.Results:Before PSM,the OA group had larger tumor sizes(median[interquartile range,IQR]:8.9[7.3-10.3]vs.7.2[6.7-8.0]cm;p=0.000)and higher vanillylmandelic acid level(median[IQR]:114.3[67.8-326.4]vs.66.6[37.8-145.8]μmol/24 h;p=0.004)and needed a higher cumulative dose of prazosin(median[IQR]:83.5[37.0-154.0]vs.38.0[21.0-81.0]mg;p=0.028).After PSM,the baseline data showed no significant differences between both groups.The LA group had relatively more stable blood pressure in surgery,with a lower fluctuation of systolic blood pressure(mean±standard deviation[SD]:70.9±25.1 vs.107.4±46.2 mmHg,p=0.012)and a lower percentage of hemodynamic instability(46.7%vs.86.7%,p=0.020).The LA group had shorter postoperative hospital stays(mean±SD:6.4±2.7 vs.10.1±3.4 days;p=0.003)than the OA group.Differences regarding metastasis rate(6.7%vs.0,p=1.000)were not statistically significant between LA and OA groups.The median(IQR)follow-up time of 82 patients was 72.5(47.0-103.5)months.Binary logistic regression showed that right-side tumors or those>8 cm in size were independent risk factors of OA.Conclusion:LA is a safe,minimally invasive procedure for LPCC and has relatively better perioperative characteristics in large medical centers.Patients with tumors on the right side or larger than 8 cm are more likely to undergo OA initially.
基金Supported by National Clinical Specialty Construction Project,No.2012649
文摘Vasoactive intestinal peptide-producing tumors （VIP-oma） usually originate in the pancreas and are chara-cterized by diarrhea, hypokalemia, and achlorhydria （WDHA syndrome）. In adults, nonpancreatic VIPoma is very rare. Herein, we report an unusual case of VIP-producing pheochromocytoma marked by persistent shock, fushing, and watery diarrhea and high sensitivity to octreotide. A 53-year-old woman was hospitalized for sudden-onset hypertension with convulsions, which then rapidly evolved to persistent shock, fushing, and watery diarrhea. Abdominal computed tomography indicated a left adrenal mass, accompanied by bleeding;and marked elevations of both plasma catecholamine and VIP concentrations were documented via laboratory testing. Surprisingly, all clinical symptoms responded swiftly to octreotide treatment. Once surgically treated, hormonal levels normalized in this patient, and the clinical symptoms dissipated. Postoperative pathological and immunohistopathological studies confrmed a VIP-secreting pheochromocytoma with strong, diffuse positivity for somatostatin receptor type 2. During a 6-mo follow-up period, she seemed in good health andwas symptom-free.
文摘Bladder pheochromocytoma is the most common extra-adrenal genitourinary tumor.Endoscopic management is feared due to the risk of intra-operative hypertensive crisis.We described a case of successful endoscopic management of a bladder pheochromocytoma and discussed its technical aspects.
文摘AIM To investigate the occurrence of cardiomyopathy(CMP)in a cohort of patients with histologically proven pheochromocytoma(pheo),and to determine if catecholamine excess was causative of the left ventricular(LV)dysfunction.METHODS A retrospective chart review spanning years 1998through 2014 was undertaken and patients with a diagnosis of pheo confirmed with histopathologic examination were included.Presenting electrocardiograms and cardiac imaging studies were reviewed.Transthoracic echocardiography(TTE),ventriculography or single positron emission computed tomography imaging was evaluated and if significant abnormalities[left ventricular hypertrophy(LVH)or LV dysfunction]were noted in the pre operative period a follow up post-operative study was also analyzed.Multivariate analysis using logistic regression was used to investigate independent predictors for outcomes of interest,LV dysfunction and LVH.RESULTS We identified 18 patients with diagnosis of pheo confirmed on pathology.Mean age was 54.3±19.3years and 11(61.1%)patients were females.50%of such patients had either resistant hypertension or labile blood pressures during hospitalization,which had raised suspicion for a pheo.Cardiac imaging studies were available for 12(66.7%)patients at the time of inclusion into study and preceding the adrenalectomy.7(58.3%)patients with a TTE available for review had mild or more severe LVH while 3(25%)patients had LV dysfunction of presumably acute onset.In a multivariate analysis,elevated catecholamine levels as assessed by urinary excretion of metabolites was not an independent predictor of development of LV systolic dysfunction or of presence of LVH on TTE.Two female patients with a preceding history of hypertension had marked LV hypertrophy and systolic anterior motion of the mitral valve.Prolongation of the QTc interval was noted in 5(27.8%)patients but no acute arrhythmias were observed in any patient.CONCLUSION This study adds to the growing body of literature on the predilection of patients with pheochromocytomas to develop non-ischemic CMP.Degree of catecholamine excess as measured by urinary secretion of metabolites did not predict the development of CMP but 2 of 3patients developed CMP in the setting of significant acute physiologic stress.Our findings provide support to the proposed etiologic role of elevated catecholamines in TC and other stress induced forms of CMP,however,activation of a brain-neural-cardiac axis from acute stress and local release of catecholamines but not chronic catecholamine elevations are likely to be responsible in pheo related CMP.
文摘BACKGROUND Malignant pheochromocytoma with cerebral and skull metastasis is a very rare disease.Combining our case with 16 previously reported cases identified from a PubMed search,an analysis of 17 cases of malignant cerebral pheochromocytoma was conducted.This literature review aimed to provide information on clinical manifestations,radiographic and histopathological features,and treatment strategies of this condition.CASE SUMMARY A 60-year-old man was admitted with a progressive headache and enlarging scalp mass lasting for 3 mo.Radiographic images revealed a left temporal biconvexshaped epidural mass and multiple lytic lesions.The patient underwent a left temporal craniotomy for resection of the temporal tumor.Histopathological analysis led to identification of the mass as malignant pheochromocytoma.The patient’s symptoms were alleviated at the postoperative 3-mo clinical follow-up.However,metastatic pheochromocytoma lesions were found on the right 6th rib and the 6th to 9th thoracic vertebrae on a 1-year clinical follow-up computed tomography scan.CONCLUSION Magnetic resonance spectroscopy and histopathological examination are necessary to make an accurate differential diagnosis between malignant cerebral pheochromocytoma and meningioma.Surgery is regarded as the first choice of treatment.
文摘Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. After surgical resection of the tumor, diarrhea subsided and both electrolytes and affected hormone levels normalized. Immunohistochemical examination confirmed a diagnosis of pheochromocytoma, which contained VIP-positive ganglion-like cells. We herein present the clinical and histogenetic implications of this rare clinical entity, with literature review.
基金Supported by the Peking Union Medical Foundation-Rui E(Rui Yi)Emergency Medical Research Special Fund,No.R2018001the Beijing Science and Technology Association Jinqiao Project Seed Fund,No.JQ18057.
文摘BACKGROUND Pheochromocytomas are rare endocrine tumors with various clinical manifestations,and few of them might present with profound,life-threatening conditions.CASE SUMMARY We report the case of a 65-year-old man who complained of sudden dyspnea and hemoptysis for half a day.There was no obvious cause for the patient to have dyspnea,coughing,or coughing up to approximately 100 mL of fresh blood.Finally,he was diagnosed with pheochromocytoma crisis(PCC),coexisting with an abdominal aortic aneurysm(AAA).CONCLUSION We report a case of pheochromocytoma presenting with recurrent hemoptysis,dyspnea and hypotension coexisting with an AAA.It not only proved the uncommon manifestations of pheochromocytoma but also directed clinicians to consider PCC among the possible diagnoses when meeting similar cases.Moreover,surgical excision is the most beneficial method for the treatment of pheochromocytoma coexisting with AAA when the situation is stable.
