AN UNUSUAL BILATERAL VAGAL PARAGANGLIOMAS: ONE CASE STUDY

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,

Retroperitoneal paragangliomas:Report of 4 cases

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.

Nonhypertensive male with multiple paragangliomas of the heart and neck:A case report

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.

Radiation Therapy of Head and Neck Paragangliomas: Experience from Radiotherapy Department at the National Institute of Oncology in Rabat (Morocco)

Background and Purpose: Paragangliomas are rare tumors of the head and neck. Their management remains problematic and varies considerably depending on the center. This study reported 14 years of experience in the management of head and neck paraganglioma (HNPGls). We aimed to assess the therapeutic results of these tumors in terms of local control and overall survival. Materials and Methods: 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. Results: 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 (56.3%). 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%. Conclusions: Surgery is the first-line treatment for HNPGls. When surgery is not possible or incomplete, radiotherapy has its place in the therapeutic strategy of this rare disease for long-term local control.

Endoscopic Transcanal Excision of Glomus Tympanicum: A Case Report and Literature Review

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.

Anesthesia Management in Hereditary Pheochromocytoma and Paraganglioma:Updated Insights into Clinical Features and Perioperative Care

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.

Subclinical paraganglioma of the retroperitoneum:A case report

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.

Treatment for paraganglioma with stereotactic radiotherapy

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.

Utilizing bioinformatics for integrated analysis of multiple genes in the diagnosis and pathogenesis of metastatic pheochromocytoma and paraganglioma

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.

Primary intra-abdominal paraganglioma:A case report

BACKGROUND Paragangliomas are rare neuroendocrine tumors.We hereby report a case of a localized paraganglioma found in the abdominal cavity,and review the relevant literature to improve the understanding of this disease.CASE SUMMARY A 29-year-old Chinese female patient was referred to our hospital due to an abdominal mass found on physical examination.Imaging revealed a mass in the left upper abdomen,suggestive of either a benign stromal tumor or an ectopic accessory spleen.Laparoscopic radical resection was subsequently performed,and histopathological analysis confirmed the diagnosis of a paraganglioma.The patient was followed up 3 months post-operation,and reported good recovery with no metastasis.CONCLUSION Radical resection can effectively treat intra-abdominal paragangliomas,with few side effects and low recurrence risk.In addition,early and accurate diagnosis and timely intervention are essential for the prognosis of this disease.

Intrapericardial Paraganglioma, an Exceedingly Rare Finding: A Case Report

Paragangliomas are rare tumors that arise from the sympathetic or the parasympathetic ganglia. Parasympathetic paragangliomas are usually nonfunctional. They are often found in the pre aortic and paravertebral sympathetic plexus or at the base of the skull. 80% of these are Glomus jugulare and carotid body paragangliomas. Intrapericardial paragangliomas are exceedingly rare. Less than 2% of paragangliomas are found in the chest and most of them are situated in the posterior mediastinum. As such, there are no clear guidelines on how to treat intrapericardial paragangliomas. We present here the case of a patient with an intrapericardial paraganglioma who was evaluated by cardiology, cardiac surgery, and endocrinology. In this case cardiac surgery opted for a conservative approach and did not offer surgery to the patient. There is some literature supporting a surgical approach but no specific guidelines about this have been written. The patient is currently being monitored since she did not have metastatic disease and her paraganglioma was nonfunctioning. With this case we want to add to the body of evidence that this type of cases does not need an urgent surgical approach.

Per-Operative Discovery of Tympanic Paraganglioma: A Case Report

Background: Tympanic paragangliomas are common middle ear benign neoplasias that develop from endocrines cells situated along the great blood vessels of the head and neck, as well as those of the thoracic and lumbar spine. Primary symptoms of tympanic locations include hearing loss and pulsatile tinnitus. Otoscopy often shows a retrotympanic reddish mass, causing tympanic bulging. Computerized tomography scan findings include a tissue density regular mass located at the promontory and limited within the middle ear cavity. Surgical management comprises tumour excision with control of bleeding, with or without prior embolization. Aim: The aim of this presentation is to report an unusual case of tympanic paraganglioma. Case Presentation: We report the case of a 56-year-old patient whose clinical and paraclinical presentations were consistent with cholesteatoma, though a tympanic paraganglioma was discovered per-operatively. Conclusion: In some rare cases, tympanic paraganglioma can be present like a cholesteatoma.

迷走神经主干副神经节瘤1例

1临床资料患者，女性，41岁，因发现左侧颈部肿块6年，伴疼痛1个月于2006-11-02入院。体检：左侧颈部触及一肿块，大小约3．5cm×3cm，局部无红肿，轻度压痛．可移动，无波动。颈部CT示左侧下颔骨后缘深面可见一结节状软组织密度影，大小约3．5cm×2．7cm，与周边组织界线清楚，增强后呈明显环形强化（图1）。颈部彩超：肿块位于左侧颈总动脉后外方，血流丰富。于2006-11-05全麻下行左侧颈部肿块切除术。术中见肿瘤位于下颌角内后方，胸锁乳突肌深面，

迷走神经副节瘤1例

头颈部副神经节瘤约占头颈肿瘤的0.6%,主要包含颈动脉体瘤、颈静脉球体瘤和迷走神经副节瘤。临床中迷走神经副节瘤很少见。我科于2014年2月收治1例迷走神经副节瘤患者,报道如下。

Recurrent Myocardial Infarction and Catecholamine-Induced Cardiomyopathy:A Case Report

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.

甲状腺周围副神经节瘤1例

1临床资料患者，女，32岁，于2013-09-16我院体检颈部B超提示：甲状腺右侧叶内见一低回声结节，大小1．9cm×1．3cm，境界清，内部回声欠均匀（图1A、B）。

甲状腺副神经节瘤合并双侧颈动脉体瘤的颈部多发性副神经节瘤1例

副神经节瘤是一种少见的神经内分泌肿瘤,多来自于自主神经系统及其分支[1]。头颈部副神经节瘤约占全部头颈肿瘤的0.6%,80%发生于颈动脉体、颈静脉球。原发于甲状腺内的副神经节瘤极少见,而同时发生于甲状腺和双侧颈动脉体的同期颈部多发性副神经节瘤,检索中英文文献尚无病例报道,因该病少见,临床表现不典型,容易出现误诊误治。

Linear Accelerator Based External Beam Radiotherapy in Glomus Jugulare Tumour: A Retrospective Review from a Tertiary Cancer Hospital in Kenya

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. 2/3rd of the patients were females in 5th and 6th 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.

Duodenal gangliocytic paraganglioma with lymph node metastases: a case report and comparative review of 31 cases

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.

Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course

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”.