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
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
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
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
Gangliocytic paraganglioma(GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindlelike/susten...Gangliocytic paraganglioma(GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindlelike/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using Pub Med, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled(30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain(55%) and gastrointestinal bleeding or sequelae(42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure(except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin(SYN) stained all epithelioid endocrine cells(18/18). Neuron specific enolase(NSE) and SYN stained most ganglion-like cells(7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells(21/21). Our literature review of published cases of GP with lymph node metastasis underscores the excellent prognosis of GP regardless of specific treatment modality. We question the necessity of aggressive surgical intervention in select patients, and argue that local resection of the mass and metastasis may be adequate. We also emphasize the importance of pre-surgical assessment with imaging studies, as well as post-surgical follow-up surveillance for disease recurrence.展开更多
AIM： To investigate the 152 cases of paragangliomas resected over the past 32 years in West China Hospital dinicopathologically.METHODS： All cases of paragangliomas diagnosed at the Department of Gastrointestinal Su...AIM： To investigate the 152 cases of paragangliomas resected over the past 32 years in West China Hospital dinicopathologically.METHODS： All cases of paragangliomas diagnosed at the Department of Gastrointestinal Surgery and Department of Pathology, West China Hospital, China were reviewed. The pathological documents were supplied by the Department of Pathology, West China Hospital, and other necessary data were extracted from the hospital records. The statistical analyses were performed by survival analysis （Kaplan-Meier method）, descriptive statistical analyses and Х^2 analysis.RESULTS： The neuroendocrine marker vimentin was found to be selectively expressed in the benign tumors, and there were significant differences in the expression of those markers in both benign and malignant tumors. The survival analysis revealed that survival correlated significantly with the malignancy, metastasis and nodal status.CONCLUSION： Vimentin may be useful in the differential diagnosis between malignant and benign tumors. The difference in the expression of this marker in the tumors could be a clue to the future clinical diagnosis. The malignancy, metastasis and the nodal status may predict the prognosis of this disease.展开更多
Paraganglioma is a rare tumor of paraganglia,derived from neural crest cells in sympathetic or parasympathetic ganglions.Primary paraganglioma originating from the pancreas is rare.We report two patients with paragang...Paraganglioma is a rare tumor of paraganglia,derived from neural crest cells in sympathetic or parasympathetic ganglions.Primary paraganglioma originating from the pancreas is rare.We report two patients with paraganglioma in the head of the pancreas,in whom computed tomography showed a sharply marginated,hypervascular tumor with cystic areas.Significant intratumoral vessels and early contrast filling of the draining veins from the mass were not found.Although the pancreatic paraganglioma was located at the pancreatic head,the bile ducts often revealed no dilation,and sometimes the main pancreatic duct was mildly dilated.These findings are helpful in differentiating pancreatic paraganglioma from other pancreatic neoplasms.It is often difficult to distinguish between nonfunctional pancreatic paragangliomas and pancreatic endocrine tumors.In many reports,pancreatic paragangliomas show the retroperitoneal extension of a paraganglioma into the pancreas rather than a true pancreatic neoplasm.In surgical treatment,we could select simple excision of the tumor rather than radical surgery.展开更多
A pancreatic paraganglioma is a rare neoplasm that is difficult to distinguish from a pancreatic neuroendocrine tumour. Here we present a case of pancreatic paraganglioma that was surgically resected following preoper...A pancreatic paraganglioma is a rare neoplasm that is difficult to distinguish from a pancreatic neuroendocrine tumour. Here we present a case of pancreatic paraganglioma that was surgically resected following preoperative diagnosis of a pancreatic neuroendocrine tumour. Careful evaluation of the endoscopic ultrasonography findings revealed abundant draining ves-sels,which could have led to a correct preoperative diagnosis of pancreatic paraganglioma.展开更多
BACKGROUND Pancreatic paragangliomas(PPGL)are rare benign neuroendocrine neoplasms but malignancy can occur.PPGL are often misdiagnosed as pancreatic neuroendocrine tumor or pancreatic adenocarcinoma.CASE SUMMARY We r...BACKGROUND Pancreatic paragangliomas(PPGL)are rare benign neuroendocrine neoplasms but malignancy can occur.PPGL are often misdiagnosed as pancreatic neuroendocrine tumor or pancreatic adenocarcinoma.CASE SUMMARY We reviewed 47 case reports of PPGL published in PubMed to date.Fifteen patients(15/47)with PPGL underwent endoscopic ultrasound-guided fine needle aspiration(EUS-FNA).Only six(6/15)were correctly diagnosed as PPGL.All patients with PPGL underwent surgical resection except three(one patient surgery was aborted because of hypertensive crisis,two patients had metastasis or involvement of major vessels).Our patient remained on close surveillance as she was asymptomatic.CONCLUSION Accurate preoperative diagnosis of PPGL can be safely achieved by EUS-FNA with immunohistochemistry.Multidisciplinary team approach should be considered to bring the optimal results in the management of PPGL.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.
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”.
文摘Gangliocytic paraganglioma(GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindlelike/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using Pub Med, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled(30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain(55%) and gastrointestinal bleeding or sequelae(42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure(except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin(SYN) stained all epithelioid endocrine cells(18/18). Neuron specific enolase(NSE) and SYN stained most ganglion-like cells(7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells(21/21). Our literature review of published cases of GP with lymph node metastasis underscores the excellent prognosis of GP regardless of specific treatment modality. We question the necessity of aggressive surgical intervention in select patients, and argue that local resection of the mass and metastasis may be adequate. We also emphasize the importance of pre-surgical assessment with imaging studies, as well as post-surgical follow-up surveillance for disease recurrence.
文摘AIM： To investigate the 152 cases of paragangliomas resected over the past 32 years in West China Hospital dinicopathologically.METHODS： All cases of paragangliomas diagnosed at the Department of Gastrointestinal Surgery and Department of Pathology, West China Hospital, China were reviewed. The pathological documents were supplied by the Department of Pathology, West China Hospital, and other necessary data were extracted from the hospital records. The statistical analyses were performed by survival analysis （Kaplan-Meier method）, descriptive statistical analyses and Х^2 analysis.RESULTS： The neuroendocrine marker vimentin was found to be selectively expressed in the benign tumors, and there were significant differences in the expression of those markers in both benign and malignant tumors. The survival analysis revealed that survival correlated significantly with the malignancy, metastasis and nodal status.CONCLUSION： Vimentin may be useful in the differential diagnosis between malignant and benign tumors. The difference in the expression of this marker in the tumors could be a clue to the future clinical diagnosis. The malignancy, metastasis and the nodal status may predict the prognosis of this disease.
文摘Paraganglioma is a rare tumor of paraganglia,derived from neural crest cells in sympathetic or parasympathetic ganglions.Primary paraganglioma originating from the pancreas is rare.We report two patients with paraganglioma in the head of the pancreas,in whom computed tomography showed a sharply marginated,hypervascular tumor with cystic areas.Significant intratumoral vessels and early contrast filling of the draining veins from the mass were not found.Although the pancreatic paraganglioma was located at the pancreatic head,the bile ducts often revealed no dilation,and sometimes the main pancreatic duct was mildly dilated.These findings are helpful in differentiating pancreatic paraganglioma from other pancreatic neoplasms.It is often difficult to distinguish between nonfunctional pancreatic paragangliomas and pancreatic endocrine tumors.In many reports,pancreatic paragangliomas show the retroperitoneal extension of a paraganglioma into the pancreas rather than a true pancreatic neoplasm.In surgical treatment,we could select simple excision of the tumor rather than radical surgery.
文摘A pancreatic paraganglioma is a rare neoplasm that is difficult to distinguish from a pancreatic neuroendocrine tumour. Here we present a case of pancreatic paraganglioma that was surgically resected following preoperative diagnosis of a pancreatic neuroendocrine tumour. Careful evaluation of the endoscopic ultrasonography findings revealed abundant draining ves-sels,which could have led to a correct preoperative diagnosis of pancreatic paraganglioma.
文摘BACKGROUND Pancreatic paragangliomas(PPGL)are rare benign neuroendocrine neoplasms but malignancy can occur.PPGL are often misdiagnosed as pancreatic neuroendocrine tumor or pancreatic adenocarcinoma.CASE SUMMARY We reviewed 47 case reports of PPGL published in PubMed to date.Fifteen patients(15/47)with PPGL underwent endoscopic ultrasound-guided fine needle aspiration(EUS-FNA).Only six(6/15)were correctly diagnosed as PPGL.All patients with PPGL underwent surgical resection except three(one patient surgery was aborted because of hypertensive crisis,two patients had metastasis or involvement of major vessels).Our patient remained on close surveillance as she was asymptomatic.CONCLUSION Accurate preoperative diagnosis of PPGL can be safely achieved by EUS-FNA with immunohistochemistry.Multidisciplinary team approach should be considered to bring the optimal results in the management of PPGL.
