VAGAL paraganglioma （VP） is an uncommon neoplasm originating from neural crest paraganglion cells located along the vagus nerve, repre-senting less than 5 % of all paragangliomas of the head and neck Despite improve...VAGAL paraganglioma （VP） is an uncommon neoplasm originating from neural crest paraganglion cells located along the vagus nerve, repre-senting less than 5 % of all paragangliomas of the head and neck Despite improvement in microsurgical techniques,展开更多
We reviewed the data of all patients managed for retroperitoneal paragangliomas(PGLs)between June 2010and June 2011 to present our experience concerning this uncommon entity to highlight diagnostic and therapeutic cha...We reviewed the data of all patients managed for retroperitoneal paragangliomas(PGLs)between June 2010and June 2011 to present our experience concerning this uncommon entity to highlight diagnostic and therapeutic challenges of retroperitoneal PGLs.All patients were admitted to the department of general and hepatobiliary surgery in the regional hospital of Jendouba,Tunisia.The size of the tumor was taken at its largest dimension,as determined in a computed tomography(CT)scan and pathological reports.There were 4 patients(all women)with a median age of 48 years(range46-56 years).Abdominal pain was the commonest presentation.CT showed and localized the tumors which were all retroperitoneal.All patients had successfu surgical resection of the tumors under invasive arterial blood pressure monitoring.One patient underwent surgery for a presumed tumor of the pancreatic head.The fresh-mount microscopic study of the peroperative biopsy yielded inflammatory tissue without malignancy and no resection was performed.Final histologica examination of the biopsy concluded PGL.A secondlaparotomy was performed and the tumor was entirely resected.The diagnosis was made after surgery by histology in all patients.The control of the blood pressure was improved after surgery in 3 patients.Paragangliomas are rare tumors.The retroperitoneal localization is uncommon.Complete surgical resection remains the only curative treatment but it is often challenging as these tumors are located near multiple vital blood vessels.展开更多
BACKGROUND Paraganglioma is a rare disease that can be lethal if undiagnosed.Thus,quick recognition is very important.Cardiac paragangliomas are found in patients who have hypertension.The classic symptoms are the tri...BACKGROUND Paraganglioma is a rare disease that can be lethal if undiagnosed.Thus,quick recognition is very important.Cardiac paragangliomas are found in patients who have hypertension.The classic symptoms are the triad of headaches,palpitations,and profuse sweating.We describe a very rare case of multiple paragangliomas of the heart and bilateral carotid artery without hypertension and outline the management strategies for this disease.CASE SUMMARY A 46-year-old man presented with the chief complaint of recently recurrent chest pain with a history of hemangioma of the bilateral carotid artery that had been surgically removed.He was found to have an intracardiac mass in the right atrioventricular groove and underwent successful excision.The final pathology demonstrated that the intracardiac mass was a cardiac paraganglioma,and the patient had an increased level of normetanephrine in the blood.The pathology and immunohistochemistry results showed that the bilateral carotid masses were also paragangliomas.During the 3 mo follow-up period,the patient did not experience recurrence of chest pain.CONCLUSION To our knowledge,this is the first case of multiple paragangliomas of the heart and neck without hypertension.This rare disease can be lethal if left undiagnosed.Thus,quick recognition is very important.The key to the diagnosis of cardiac paraganglioma is the presence of typical symptoms,including headaches,palpitations,profuse sweating,hypertension,and chest pain.Radiology can demonstrate the intracardiac mass.It is important to determine the levels of normetanephrine in the blood.The detection of genetic mutations is also recommended.Surgical resection is necessary to treat the disease and obtain pathological evidence.展开更多
<strong>Background and Purpose:</strong><span><span><span style="font-family:""><span style="font-family:Verdana;"> Paragangliomas are rare tumors of the he...<strong>Background and Purpose:</strong><span><span><span style="font-family:""><span style="font-family:Verdana;"> Paragangliomas are rare tumors of the head and </span><span style="font-family:Verdana;">neck. Their management remains problematic and</span></span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">varies considerably de</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">pending on the center. This study reported 14 years of experience in the</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> management of head and neck paraganglioma (HNPGls)</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">.</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;"> We aimed to assess the therapeutic results of these tumors in terms of local control and overall survival. </span><b><span style="font-family:Verdana;">Materials and Methods</span></b><span style="font-family:Verdana;">: We included 16 patients followed for HNPGls and treated by radiotherapy from January 2006 to June 2018 in the National Institute of Oncology in Rabat. </span><b><span style="font-family:Verdana;">Results:</span></b><span style="font-family:Verdana;"> The median age was 44.5 years (15 - 67). 13 patients were female and three male with a sex ratio of 4.3. Cervical mass was the common sign (56</span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">.</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">3%). All patients received radiation therapy. This radiation was exclusive in 43.7% of cases or adjuvant to partial surgical resection in 56.3%. The median dose of radiotherapy was 54 Gy (46 - 60) and it was delivered by a three-dimensional conformal radiotherapy technique in 15 patients and volumetric modulated arc therapy in one. There were few acute complications such as grade I and II mucositis and dermatitis. After a median follow-up of 5.6 years (2 - 13.4), local control, defined by radiological stability or regression, was obtained in 14 patients, two patients progressed and one died. Progression-free survival rates at 5 and 7 years were 93.8% and 78.1% respectively, and overall survival at 5 and 7 years was 92.3%. </span><b><span style="font-family:Verdana;">Conclu</span><span style="font-family:Verdana;">sions:</span></b><span style="font-family:Verdana;"> Surgery is the first-line treatment for HNPGls. When surgery is not</span><span style="font-family:Verdana;"> possible or incomplete, radiotherapy has its place in the therapeutic strategy of this rare disease for long-term local control.</span></span></span></span>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 and paraganglioma(PGL)are a rare group of neuroendocrine neoplasms with characteristic genetic diversity and catecholamine secretion patterns.They arise from non-neuronal and non-epithelial...BACKGROUND Pheochromocytoma and paraganglioma(PGL)are a rare group of neuroendocrine neoplasms with characteristic genetic diversity and catecholamine secretion patterns.They arise from non-neuronal and non-epithelial neuroendocrine cells of the paraganglia,and have the highest rate of heritability among all tumors.CASE SUMMARY A 76-year-old woman presented with the complaint of dizziness that had persisted for one week.She had a 30-year history of hypertension.Despite longterm use of antihypertensive drugs,her blood pressure was not effectively controlled.A tumor was subsequently found in the head of the pancreas by computed tomography and magnetic resonance imaging and she was initially diagnosed with an aneurysm.On December 21,2021,she underwent resection of the retroperitoneal tumor and pancreatic repair surgery.However,after postoperative pathological analysis and immunohistochemistry,the diagnosis was revised to PGL.After two years and eight months of follow-up,the tumor did not recur or metastasize,and her blood pressure returned to normal without taking antihypertensive drugs.CONCLUSION The possibility of PGL should be considered when a tumor is identified and patients have catecholamine secretion related symptoms that are difficult to control with medications.展开更多
BACKGROUND Paraganglioma(PGL)is a neuroendocrine tumor originating from paraganglia that can occur in various locations,such as the head,neck,chest,abdomen,and pelvis.Retroperitoneal PGLs are rare,and recurrent cases ...BACKGROUND Paraganglioma(PGL)is a neuroendocrine tumor originating from paraganglia that can occur in various locations,such as the head,neck,chest,abdomen,and pelvis.Retroperitoneal PGLs are rare,and recurrent cases in this area are partic-ularly uncommon,posing considerable surgical complexities.Owing to their neu-roendocrine activity,PGLs are capable of secreting hormones like catecholamines,thereby presenting significant challenges in hemodynamic management during the perioperative period.CASE SUMMARY We report a 64-year-old man with a recurrent retroperitoneal PGL.The patient underwent retroperitoneal mass resection in 2013,with postoperative pathology revealing a PGL.Regular follow-up was not conducted until April 2024,when a computed tomography scan revealed a huge mass in the retroperitoneum,closely adjacent to the abdominal aorta.Laboratory examinations revealed elevated levels of catecholamines in the patient's blood serum.Upon admission,volume expan-sion and blood pressure(BP)monitoring were carried out for one week,with catecholamine levels reviewed and normalized.Adequate preoperative prepa-ration was conducted,including central venous access,arterial BP monitoring,and the preparation of vasoactive agents.During tumor resection,the patient ex-perienced acute,significant fluctuations in BP.The timely intervention of the anesthesiologist stabilized the BP,facilitating the successful resection of the tumor which was confirmed as a recurrent PGL.Postoperative follow-up revealed no evidence of tumor residual or recurrence.CONCLUSION PGL recurrence is rare but non-negligible.PGLs adjacent to major arteries com-plicate surgery,and perioperative hemodynamic stability demands meticulous attention.Core Tip:Recurrent retroperitoneal paragangliomas are infrequent but pose substantial surgical challenges,particularly when located adjacent to critical vascular structures such as the abdominal aorta.Effective perioperative management of he-modynamic fluctuations,driven by catecholamine secretion,requires meticulous preoperative planning,including volume expansion,blood pressure monitoring,and vasoactive agent preparation.Surgical intervention demands prompt and coordinated anesthetic support to stabilize hemodynamics,ensuring successful tumor resection.Given the potential for late recurrence,long-term follow-up is essential for early detection and management of asymptomatic recurrences.INTRODUCTION Paraganglioma(PGL)is a neuroendocrine tumor originating from paraganglia,which are associated with the autonomic nervous system[1].These tumors arise from chromaffin cells or similar cells capable of secreting catecholamines,such as adrenaline and noradrenaline.PGLs manifest in various anatomical locations,including the head,neck,chest,abdomen,and pelvis,and are characterized by neurosecretory and chief cells surrounded by prominent vascular stroma.Although typically benign,malignant forms of PGL are also recognized,and they may exhibit a tendency for recurrence or me-tastasis[2].PGLs located in the retroperitoneum are relatively uncommon and present greater surgical challenges because of their complex anatomical location near vital organs and major blood vessels.Their catecholamine-secreting nature further complicates the maintenance of hemodynamic stability during the perioperative period.In May 2024,a patient with recurrent retroperitoneal PGL was admitted to our hospital,and the details are reported below.展开更多
Glomus tumors are extremely rare tumors that arise from paraganglionic cells, which are derived from neural crest cells. These tumors are benign, slow-growing, locally invasive, and destructive. Glomus tumors are the ...Glomus tumors are extremely rare tumors that arise from paraganglionic cells, which are derived from neural crest cells. These tumors are benign, slow-growing, locally invasive, and destructive. Glomus tumors are the most common tumor of the middle ear cavity and the second most common tumor of the temporal bone. We present a case of a 49-year-old healthy female who, following diagnostic tools, received surgical intervention resulting in an excellent outcome. Our case report includes a comprehensive analysis of published cases in the literature.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
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
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
BACKGROUND Paragangliomas(PG)are rare neoplasms of neuroendocrine origin that tend to be highly vascularized,slow-growing,and usually sporadic.To date,common treatment options are surgical resection(SR),with or withou...BACKGROUND Paragangliomas(PG)are rare neoplasms of neuroendocrine origin that tend to be highly vascularized,slow-growing,and usually sporadic.To date,common treatment options are surgical resection(SR),with or without radiation therapy(RT),and a watch-and-wait approach.AIM To evaluate the local control and effectiveness of exclusive fractionated stereotactic RT(FSRT)treatment in unresectable PG(uPG).METHODS We retrospectively evaluated patients with uPG(medically inoperable or refused SR)treated with FSRT with a Cyberknife System(Accuray Incorporated,Sunnyvale,California).Toxicity and initial efficacy were evaluated.RESULTS From May 2009 to January 2023,6 patients with a median age of 68(range 20-84)were treated with FSRT.The median delivered dose was 21 Gy(range 20-30 Gy)at a median isodose line of 75.5%(range 70%-76%)in 4 fractions(range 3-5 fractions).The median volume was 13.6 mL(range 12.4-65.24 mL).The median cumulative biological effective dose and equivalent dose in 2-Gy fractions were 70 Gy and 37.10 Gy respectively.Site of origin involved were the timpa-nojugular glomus(4/6),temporal bone,and cervical spine.In 1 of the 6 patients,the follow-up was insufficient;5 of 6 patients showed a 5-year overall survival and 5-year progression-free survival of 100%.We observed negligible toxicities during and after RT.The majority of patients showed stable symptoms during follow-up.Only 1 patient developed spine metastases.CONCLUSION Our preliminary results on this small cohort of patients suggest that FSRT could be an effective and safe alternative to SR.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
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
Objective: Tumours originating from jugular bulb, carotid bifurcation, Vagus nerve are collectively called Paragangliomas. They are slow growing, essentially benign tumours, but can be detrimental if untreated. There ...Objective: Tumours originating from jugular bulb, carotid bifurcation, Vagus nerve are collectively called Paragangliomas. They are slow growing, essentially benign tumours, but can be detrimental if untreated. There is limited evidence on the effectiveness of fractionated radiotherapy in the management ofs Glomus jugulare tumours. The aim of this study is to determine the efficacy of Linear accelerator based fractionated external beam radiotherapy on unilateral inoperable Glomus jugulare paragangliomas. Method: This is a retrospective analysis of all the 12 cases of inoperable, unilateral Glomus jugulare tumours treated during the period 2011-2016 at a tertiary cancer centre in Kenya. Minimum follow up duration was 3 years. Patient characteristics, disease staging, immediate complications and therapeutic efficacy were analysed from the case files. Results: The 12 patients diagnosed with inoperable Glomus jugulare tumours reported in this period were treated with external beam radiotherapy to a tumour dose of 54 Gy in 30 fractions over a period of 6 weeks using IMRT technique in 6 MV Linear accelerator. 2/3<sup>rd </sup>of the patients were females in 5<sup>th</sup> and 6<sup>th</sup> decade of life. Onset of first symptom to initiation of treatment was found to be 1.7 years. Headache, earache, and tinnitus were the main complaints. No major side effects were recorded during therapy. Mean length of the tumour in its maximum dimension at the time of diagnosis was 4.5 cm. At the end of one-year post therapy, a mean reduction of 6.5 mm in the tumour length was observed, (Range: 0 - 15 mm). Tumour size remained static for a year and thereafter a slow growth pattern of 1mm per year was observed. Conclusion: Fractionated external beam radiotherapy is an effective and non-invasive treatment for advanced, inoperable Glomus jugulare paragangliomas. Clinical stability through tumour control was observed. Though newer radiation techniques like Cyberknife, Proton therapy offer better tumour control, conventional external beam radiotherapy is an effective tool in disease containment in resource limited countries.展开更多
Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patien...Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of “1”, rather than a benign tumor of “0”.展开更多
文摘VAGAL paraganglioma （VP） is an uncommon neoplasm originating from neural crest paraganglion cells located along the vagus nerve, repre-senting less than 5 % of all paragangliomas of the head and neck Despite improvement in microsurgical techniques,
文摘We reviewed the data of all patients managed for retroperitoneal paragangliomas(PGLs)between June 2010and June 2011 to present our experience concerning this uncommon entity to highlight diagnostic and therapeutic challenges of retroperitoneal PGLs.All patients were admitted to the department of general and hepatobiliary surgery in the regional hospital of Jendouba,Tunisia.The size of the tumor was taken at its largest dimension,as determined in a computed tomography(CT)scan and pathological reports.There were 4 patients(all women)with a median age of 48 years(range46-56 years).Abdominal pain was the commonest presentation.CT showed and localized the tumors which were all retroperitoneal.All patients had successfu surgical resection of the tumors under invasive arterial blood pressure monitoring.One patient underwent surgery for a presumed tumor of the pancreatic head.The fresh-mount microscopic study of the peroperative biopsy yielded inflammatory tissue without malignancy and no resection was performed.Final histologica examination of the biopsy concluded PGL.A secondlaparotomy was performed and the tumor was entirely resected.The diagnosis was made after surgery by histology in all patients.The control of the blood pressure was improved after surgery in 3 patients.Paragangliomas are rare tumors.The retroperitoneal localization is uncommon.Complete surgical resection remains the only curative treatment but it is often challenging as these tumors are located near multiple vital blood vessels.
基金Supported by the Youth Fund Program in Zhongshan Hospital,Fudan University,No.2018ZSQH49.
文摘BACKGROUND Paraganglioma is a rare disease that can be lethal if undiagnosed.Thus,quick recognition is very important.Cardiac paragangliomas are found in patients who have hypertension.The classic symptoms are the triad of headaches,palpitations,and profuse sweating.We describe a very rare case of multiple paragangliomas of the heart and bilateral carotid artery without hypertension and outline the management strategies for this disease.CASE SUMMARY A 46-year-old man presented with the chief complaint of recently recurrent chest pain with a history of hemangioma of the bilateral carotid artery that had been surgically removed.He was found to have an intracardiac mass in the right atrioventricular groove and underwent successful excision.The final pathology demonstrated that the intracardiac mass was a cardiac paraganglioma,and the patient had an increased level of normetanephrine in the blood.The pathology and immunohistochemistry results showed that the bilateral carotid masses were also paragangliomas.During the 3 mo follow-up period,the patient did not experience recurrence of chest pain.CONCLUSION To our knowledge,this is the first case of multiple paragangliomas of the heart and neck without hypertension.This rare disease can be lethal if left undiagnosed.Thus,quick recognition is very important.The key to the diagnosis of cardiac paraganglioma is the presence of typical symptoms,including headaches,palpitations,profuse sweating,hypertension,and chest pain.Radiology can demonstrate the intracardiac mass.It is important to determine the levels of normetanephrine in the blood.The detection of genetic mutations is also recommended.Surgical resection is necessary to treat the disease and obtain pathological evidence.
文摘<strong>Background and Purpose:</strong><span><span><span style="font-family:""><span style="font-family:Verdana;"> Paragangliomas are rare tumors of the head and </span><span style="font-family:Verdana;">neck. Their management remains problematic and</span></span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">varies considerably de</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">pending on the center. This study reported 14 years of experience in the</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> management of head and neck paraganglioma (HNPGls)</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">.</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;"> We aimed to assess the therapeutic results of these tumors in terms of local control and overall survival. </span><b><span style="font-family:Verdana;">Materials and Methods</span></b><span style="font-family:Verdana;">: We included 16 patients followed for HNPGls and treated by radiotherapy from January 2006 to June 2018 in the National Institute of Oncology in Rabat. </span><b><span style="font-family:Verdana;">Results:</span></b><span style="font-family:Verdana;"> The median age was 44.5 years (15 - 67). 13 patients were female and three male with a sex ratio of 4.3. Cervical mass was the common sign (56</span></span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">.</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">3%). All patients received radiation therapy. This radiation was exclusive in 43.7% of cases or adjuvant to partial surgical resection in 56.3%. The median dose of radiotherapy was 54 Gy (46 - 60) and it was delivered by a three-dimensional conformal radiotherapy technique in 15 patients and volumetric modulated arc therapy in one. There were few acute complications such as grade I and II mucositis and dermatitis. After a median follow-up of 5.6 years (2 - 13.4), local control, defined by radiological stability or regression, was obtained in 14 patients, two patients progressed and one died. Progression-free survival rates at 5 and 7 years were 93.8% and 78.1% respectively, and overall survival at 5 and 7 years was 92.3%. </span><b><span style="font-family:Verdana;">Conclu</span><span style="font-family:Verdana;">sions:</span></b><span style="font-family:Verdana;"> Surgery is the first-line treatment for HNPGls. When surgery is not</span><span style="font-family:Verdana;"> possible or incomplete, radiotherapy has its place in the therapeutic strategy of this rare disease for long-term local control.</span></span></span></span>
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.
基金Supported by the Guangxi Medical and Health Care Appropriate Technology Development and Popularization and Application Project,No.S2023034.
文摘BACKGROUND Pheochromocytoma and paraganglioma(PGL)are a rare group of neuroendocrine neoplasms with characteristic genetic diversity and catecholamine secretion patterns.They arise from non-neuronal and non-epithelial neuroendocrine cells of the paraganglia,and have the highest rate of heritability among all tumors.CASE SUMMARY A 76-year-old woman presented with the complaint of dizziness that had persisted for one week.She had a 30-year history of hypertension.Despite longterm use of antihypertensive drugs,her blood pressure was not effectively controlled.A tumor was subsequently found in the head of the pancreas by computed tomography and magnetic resonance imaging and she was initially diagnosed with an aneurysm.On December 21,2021,she underwent resection of the retroperitoneal tumor and pancreatic repair surgery.However,after postoperative pathological analysis and immunohistochemistry,the diagnosis was revised to PGL.After two years and eight months of follow-up,the tumor did not recur or metastasize,and her blood pressure returned to normal without taking antihypertensive drugs.CONCLUSION The possibility of PGL should be considered when a tumor is identified and patients have catecholamine secretion related symptoms that are difficult to control with medications.
基金Supported by Natural Science Foundation of Zhejiang Province,China,No.LQ21H020007.
文摘BACKGROUND Paraganglioma(PGL)is a neuroendocrine tumor originating from paraganglia that can occur in various locations,such as the head,neck,chest,abdomen,and pelvis.Retroperitoneal PGLs are rare,and recurrent cases in this area are partic-ularly uncommon,posing considerable surgical complexities.Owing to their neu-roendocrine activity,PGLs are capable of secreting hormones like catecholamines,thereby presenting significant challenges in hemodynamic management during the perioperative period.CASE SUMMARY We report a 64-year-old man with a recurrent retroperitoneal PGL.The patient underwent retroperitoneal mass resection in 2013,with postoperative pathology revealing a PGL.Regular follow-up was not conducted until April 2024,when a computed tomography scan revealed a huge mass in the retroperitoneum,closely adjacent to the abdominal aorta.Laboratory examinations revealed elevated levels of catecholamines in the patient's blood serum.Upon admission,volume expan-sion and blood pressure(BP)monitoring were carried out for one week,with catecholamine levels reviewed and normalized.Adequate preoperative prepa-ration was conducted,including central venous access,arterial BP monitoring,and the preparation of vasoactive agents.During tumor resection,the patient ex-perienced acute,significant fluctuations in BP.The timely intervention of the anesthesiologist stabilized the BP,facilitating the successful resection of the tumor which was confirmed as a recurrent PGL.Postoperative follow-up revealed no evidence of tumor residual or recurrence.CONCLUSION PGL recurrence is rare but non-negligible.PGLs adjacent to major arteries com-plicate surgery,and perioperative hemodynamic stability demands meticulous attention.Core Tip:Recurrent retroperitoneal paragangliomas are infrequent but pose substantial surgical challenges,particularly when located adjacent to critical vascular structures such as the abdominal aorta.Effective perioperative management of he-modynamic fluctuations,driven by catecholamine secretion,requires meticulous preoperative planning,including volume expansion,blood pressure monitoring,and vasoactive agent preparation.Surgical intervention demands prompt and coordinated anesthetic support to stabilize hemodynamics,ensuring successful tumor resection.Given the potential for late recurrence,long-term follow-up is essential for early detection and management of asymptomatic recurrences.INTRODUCTION Paraganglioma(PGL)is a neuroendocrine tumor originating from paraganglia,which are associated with the autonomic nervous system[1].These tumors arise from chromaffin cells or similar cells capable of secreting catecholamines,such as adrenaline and noradrenaline.PGLs manifest in various anatomical locations,including the head,neck,chest,abdomen,and pelvis,and are characterized by neurosecretory and chief cells surrounded by prominent vascular stroma.Although typically benign,malignant forms of PGL are also recognized,and they may exhibit a tendency for recurrence or me-tastasis[2].PGLs located in the retroperitoneum are relatively uncommon and present greater surgical challenges because of their complex anatomical location near vital organs and major blood vessels.Their catecholamine-secreting nature further complicates the maintenance of hemodynamic stability during the perioperative period.In May 2024,a patient with recurrent retroperitoneal PGL was admitted to our hospital,and the details are reported below.
文摘Glomus tumors are extremely rare tumors that arise from paraganglionic cells, which are derived from neural crest cells. These tumors are benign, slow-growing, locally invasive, and destructive. Glomus tumors are the most common tumor of the middle ear cavity and the second most common tumor of the temporal bone. We present a case of a 49-year-old healthy female who, following diagnostic tools, received surgical intervention resulting in an excellent outcome. Our case report includes a comprehensive analysis of published cases in the literature.
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.
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BACKGROUND Paragangliomas(PG)are rare neoplasms of neuroendocrine origin that tend to be highly vascularized,slow-growing,and usually sporadic.To date,common treatment options are surgical resection(SR),with or without radiation therapy(RT),and a watch-and-wait approach.AIM To evaluate the local control and effectiveness of exclusive fractionated stereotactic RT(FSRT)treatment in unresectable PG(uPG).METHODS We retrospectively evaluated patients with uPG(medically inoperable or refused SR)treated with FSRT with a Cyberknife System(Accuray Incorporated,Sunnyvale,California).Toxicity and initial efficacy were evaluated.RESULTS From May 2009 to January 2023,6 patients with a median age of 68(range 20-84)were treated with FSRT.The median delivered dose was 21 Gy(range 20-30 Gy)at a median isodose line of 75.5%(range 70%-76%)in 4 fractions(range 3-5 fractions).The median volume was 13.6 mL(range 12.4-65.24 mL).The median cumulative biological effective dose and equivalent dose in 2-Gy fractions were 70 Gy and 37.10 Gy respectively.Site of origin involved were the timpa-nojugular glomus(4/6),temporal bone,and cervical spine.In 1 of the 6 patients,the follow-up was insufficient;5 of 6 patients showed a 5-year overall survival and 5-year progression-free survival of 100%.We observed negligible toxicities during and after RT.The majority of patients showed stable symptoms during follow-up.Only 1 patient developed spine metastases.CONCLUSION Our preliminary results on this small cohort of patients suggest that FSRT could be an effective and safe alternative to SR.
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.
文摘Objective: Tumours originating from jugular bulb, carotid bifurcation, Vagus nerve are collectively called Paragangliomas. They are slow growing, essentially benign tumours, but can be detrimental if untreated. There is limited evidence on the effectiveness of fractionated radiotherapy in the management ofs Glomus jugulare tumours. The aim of this study is to determine the efficacy of Linear accelerator based fractionated external beam radiotherapy on unilateral inoperable Glomus jugulare paragangliomas. Method: This is a retrospective analysis of all the 12 cases of inoperable, unilateral Glomus jugulare tumours treated during the period 2011-2016 at a tertiary cancer centre in Kenya. Minimum follow up duration was 3 years. Patient characteristics, disease staging, immediate complications and therapeutic efficacy were analysed from the case files. Results: The 12 patients diagnosed with inoperable Glomus jugulare tumours reported in this period were treated with external beam radiotherapy to a tumour dose of 54 Gy in 30 fractions over a period of 6 weeks using IMRT technique in 6 MV Linear accelerator. 2/3<sup>rd </sup>of the patients were females in 5<sup>th</sup> and 6<sup>th</sup> decade of life. Onset of first symptom to initiation of treatment was found to be 1.7 years. Headache, earache, and tinnitus were the main complaints. No major side effects were recorded during therapy. Mean length of the tumour in its maximum dimension at the time of diagnosis was 4.5 cm. At the end of one-year post therapy, a mean reduction of 6.5 mm in the tumour length was observed, (Range: 0 - 15 mm). Tumour size remained static for a year and thereafter a slow growth pattern of 1mm per year was observed. Conclusion: Fractionated external beam radiotherapy is an effective and non-invasive treatment for advanced, inoperable Glomus jugulare paragangliomas. Clinical stability through tumour control was observed. Though newer radiation techniques like Cyberknife, Proton therapy offer better tumour control, conventional external beam radiotherapy is an effective tool in disease containment in resource limited countries.
文摘Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of “1”, rather than a benign tumor of “0”.
