Management Of Paragangliomas Research Articles

Current peri-operative and surgical management of bladder paraganglioma

Introduction: In this article, we provide a narrative review of current peri-operative and surgical management of paraganglioma of the urinary bladder (PUB). Methods: Key articles relating to peri-operative optimisation, intra-operative considerations and post-operative follow-up of patients with PUB were identified for review. Discussion: Although the majority of PUBs are functionally active with potential for catecholamine release, typical catecholaminergic symptoms are often overshadowed by the presence of lower urinary tract symptoms such as painless visible haematuria. Consequently, many cases are diagnosed based on post-operative histopathology, resulting in inferior short- and long-term outcomes. Accurate pre-operative diagnosis is crucial as this informs pre-operative optimisation and choice of surgical approach. Partial cystectomy is the mainstay of treatment, but transurethral resection may be appropriate for non-functional tumours <3 cm. The presence of succinate dehydrogenase ( SDH) subunit B (SDHB) pathogenic variants may prompt upfront radical cystectomy. Long-term outcomes of surgical patients remain incompletely characterised and lifelong follow-up is recommended to exclude tumour recurrence. Conclusion: Patients with PUBs require meticulous peri-operative optimisation under the care of an experienced multidisciplinary team. A pre-operative diagnosis is crucial to ensure optimal outcomes and guide surgical decision-making. Level of evidence: 4

ASO Author Reflections: Shaping Paraganglioma Management from Operating Rooms to Genetic Labs.

ASO Author Reflections: Shaping Paraganglioma Management from Operating Rooms to Genetic Labs.

Multidisciplinary Management of Lateral Skull Base Paragangliomas: A 20-Year Experience.

Objectives Paragangliomas of the lateral skull base are rare, largely benign, neuroendocrine tumors. Little evidence exists to support clinicians in the management of these tumors. The present study evaluates considerations in the multidisciplinary workup and management of paragangliomas affecting the lateral skull base. Methods A STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) checklist compliant retrospective review of adult patients with lateral skull base paragangliomas over 20 years (2002-2021) was performed. Patient and tumor data were collected from patient health care records. Results Seventy patients were identified including 21 (30.0%) males and 49 (70.0%) females. The mean length of follow-up was 87.3 months (range: 12-239 months). Twenty-nine (41.4%) patients had Fisch A or B tumors. Overall, 57 patients (81.4%) were offered treatment upfront, with 13 patients (18.6%) initially undergoing active surveillance. Younger age and reduced American Society of Anesthesiologists (ASA) grade was significantly associated with a decision to offer treatment at presentation (both p = 0.03), while all patients with succinate dehydrogenase (SDH) mutations ( n = 10) and tumor secretion ( n = 4) were offered treatment. Patients with Fisch A/B tumors ( p = 0.01), cranial neuropathies ( p = 0.01), and smaller tumors ( p = 0.01) were more likely to undergo surgical resection. At the time of the last follow-up, the proportion of patients with a cranial neuropathy was notably lower in the observation (3/12, 25.0%) and radiotherapy (1/6, 16.7%) groups. Conclusion In our series, younger, fitter patients with SDH mutations, cranial neuropathies, or tumor secretion were more likely to be offered upfront treatment. There was a low incidence of new cranial neuropathy in the patients selected for observation during long-term follow-up.

Diagnosis and management of paraganglioma

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Multidisciplinary Management of Paragangliomas in a Reference Unit Hospital in Spain

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Intraoperative Corrected Diagnosis of a Right Renal Para-Hilar Paraganglioma, Taken Preoperatively for a Renal Tumor

Nonfunctional retroperitoneal paragangliomas are rare tumors. They are defined as extra-adrenal chromaffin tumors and represent about 20% of chromaffin tumors. They are often asymptomatic and can grow to large dimensions. We report the observation of a 67-year-old patient admitted to the operating room for right total nephrectomy for right hilar renal tumor and whose intraoperative dissection of the renal pedicle could demonstrate an exorenal mass. Anatomopathological analysis was in favor of a paraganglioma. Malignant forms, more frequent than benign forms, present a locoregional invasion and metastasize late. The management of paragangliomas must be multidisciplinary but only surgical treatment is curative. However, there is no consensus on the usefulness of complementary therapies, which can nevertheless be used as a symptomatic adjunct. Paragangliomas are genetic in 25% of cases. A genetic investigation must be systematically proposed.

Management of Complex Jugular Paragangliomas: Surgical Resection and Outcomes.

This study aimed to review tumor control and cranial nerve function outcomes in patients with complex jugular paragangliomas and to refine the surgical strategies for complex jugular paragangliomas. We describe our experience with 12 patients with complex jugular paragangliomas diagnosed in our institution from January 2013 to June 2020. The main outcomes included tumor control, complications, and function of facial nerve and lower cranial nerves, postoperatively. Gross-total resection was achieved for 9 (75%) patients, and subtotal resection was achieved for 3 (25%) patients. The surgical tumor control rate was 100% after a mean follow-up of 45.5 months (range, 13-111 months). Postoperatively, 10 patients (83.3%) obtained unchanged or improved facial nerve function. However, new lower cranial nerve deficits occurred in 2 patients (16.7%) due to surgical removal of the concurrent vagal paraganglioma and scar tissue enclosing the IX and XII nerves. Our refined surgical techniques, including tension-free anterior facial nerve rerouting, sigmoid sinus tunnel-packing, and pushpacking techniques, could be a choice for the treatment of complex jugular paragangliomas to achieve tumor control and cranial nerves preservation. A 2-stage surgery should be applied to minimize the risk of bilateral cranial neuropathies and the influence on cerebral circulation in patients with bilateral paragangliomas. The preoperative endovascular intervention such as coil embolization or internal carotid artery stenting can be employed for the management of paragangliomas with internal carotid artery-associated lesions.

Primary intrathyroidal paraganglioma: A case report of immunohistochemistry findings and a recommendation for surgical resection

Introduction: Thyroid paragangliomas are very rare tumors. Awareness of its presentation is important to differentiate it from other common thyroid neoplasms. We present a care report of a primary intrathyroidal paraganglioma and its histopathologic findings. Case Report: A 58-year-old asymptomatic female was referred to the General Surgery clinic following an incidental finding of thyroid nodules on imaging. She had no significant personal or family history of thyroid disease. Routine blood and thyroid function studies were within normal limits. Neck ultrasound demonstrated a multinodular goiter with a hypoechoic nodule in the right superior thyroid. Fine needle aspirate (FNA) showed atypia of unknown significance (Bethesda 3). She underwent a right hemithyroidectomy which confirmed on pathological examination to be a 19 mm intrathyroidal paraganglioma, which was positive for neuroendocrine markers and negative for calcitonin and cytokeratin. Serum metanephrine studies returned within normal limits. Conclusion: Given the difference in management of paragangliomas compared to its cytology mimics and association with familial cancer syndromes, awareness of this rare tumor, and use of immunohistochemical stains are critical in arriving at the diagnosis, which has implications for clinical management and surveillance of these patients.

Clinical presentations and management of paraganglioma in the jugular foramen

颈静脉孔区副神经节瘤（即颈静脉球瘤）起源于颈静脉球血管外膜的神经嵴细胞，其起病隐匿，临床症状不典型，早期易被忽视。病变可累及中耳、外耳道、面神经、颈静脉球、后组颅神经、颈内动脉甚至颅内，并引起相应临床症状。影像学检查以及SDHx相关基因检测对疾病的诊断和治疗方案的确立至关重要。处理策略包括随访观察、手术切除及立体定向放射治疗。治疗方案的制定需根据患者年龄、病变范围、全身情况及患者意愿等多方面综合考虑，制定个体化治疗方案，其手术治疗是耳鼻咽喉头颈外科领域最具挑战性手术之一。.

Contemporary management of paragangliomas of the head and neck.

Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors typically arising from nonsecretory head and neck parasympathetic ganglia. Historically thought of as aggressive tumors that warranted equally aggressive surgical intervention, evidence has emerged demonstrating that the vast majority of HNPGLs are slow growing and indolent. It is also now recognized that a large proportion of HNPGLs are hereditary with succinate dehydrogenase gene mutations typically implicated. These recent advances have led to significant changes in the way in which clinicians investigate and treat HNPGLs with most now opting for more conservative treatment strategies. However, a proportion of patients present with more aggressive disease and still require nonconservative treatment strategies. Recent studies have sought to determine in which groups of patients the morbidity associated with treatment is justified. We summarize the recent advances in the understanding and management of these tumors and we provide our recommendations regarding the management of HNPGLs.

Surgical Candidacy in Skull Base Paragangliomas: An Institutional Experience.

Objective The aim of the study is to determine the surgical candidacy and nuances of skull base paraganglioma surgery in the era of radiotherapy. Materials and Methods This was a retrospective observational study conducted in patients who presented with skull base paragangliomas between January 2017 and December 2019. Primary data, including indication for surgery, the approach used, the extent of resection, complications, and postoperative lower cranial nerve status were studied. Results A total of 21 cases of skull base paragangliomas were analyzed, including seven cases of tympanic paraganglioma, 10 cases of jugular foramen paraganglioma, three cases of multiple paragangliomas, and one case of vagal paraganglioma. Indications for surgery were young age, bleeding from ear, neck mass with upper aerodigestive pressure symptoms, lower cranial nerve paralysis, and patients with intracranial pressure symptoms. Total excision was done in 11 patients, near-total excision in five patients, subtotal in three patients, and surgery was not done in two patients. Facial nerve paralysis was the most common complication observed, followed by bleeding and flap necrosis. Radiotherapy was considered as adjuvant treatment wherever indicated. Conclusion A thorough knowledge and understanding of the pathophysiology of the skull base paragangliomas and its management strategies can help to achieve excellent results in terms of tumor clearance and reduction in complications. A multidisciplinary team approach and meticulous skull base surgical techniques have a significant role to play in the management of paragangliomas, especially in developing countries where availability of radiosurgery is still a challenge.

Clinical Outcome of Carotid Body Paraganglioma Management: A Review of 10-Year Experience.

Background Carotid body paragangliomas are rare neoplasms usually benign, however sometimes presenting as highly aggressive tumors. Surgery is the main line of treatment. Purpose To study and describe clinical presentations, surgical approaches, postoperative complications, and treatment outcomes. Materials and Methods A single-institution retrospective analysis of 19 cases with carotid body paragangliomas who were candidates for surgery from January 2009 through January 2019 with a mean follow-up period of 58.8 months. Results The mean age was 46 years with the female predominance of 63%. The mean size of the tumor was 4.3 cm. All cases were presented with a painless pulsating neck lump located anteriorly at the level of the hyoid bone. Neck US was done in all cases as a primary screening investigation. CT scanning was the second main investigation performed in 17 cases (89.5%) revealing tumors attached to the carotid artery at its bifurcation. Urinary catecholamine metabolites were measured in all cases to rule out familial functioning types. 5 cases (26.3%) were malignant. All cases were surgically approached through transcervical transverse incision. 11, 5, and 3 cases were classified as Shamblin's type II, III, and I, respectively. All tumors were R0 resected with nodal neck dissection conducted in the malignant group. Major complications occurred in 4 cases (21%) during tumor dissection from the adventitia of carotid bifurcation. ECA ligation was performed in one case (5.3%). 2 patients (10.5%) suffered XII nerve paralysis. Carotid artery blowout occurred in one patient (5.3%) and was immediately controlled. No operative mortality occurred. All patients were free of disease during the follow-up period. 4 malignant cases (21%) suffered a systemic relapse to bone and lung metastasis justifying adjuvant chemotherapy, radiotherapy, or both. Conclusions Surgery is the treatment of choice for carotid body paragangliomas. Complete R0 resection should be justified especially in case of malignancy. Adjuvant chemotherapy or radiotherapy is an option for patients with primary malignancy or relapse.

Surgical management of mediastinal paraganglioma: All hands on deck!

Mediastinal paragangliomas are very uncommon neuroendocrine neoplasms. Due to their tissue of origin (sympathetic ganglia of the great vessels), they tend to arise deep within pericardial space and, more importantly, intimately attached to great vessels, which makes surgical resection, even with cardiopulmonary bypass, very challenging. This commentary accompanies the case report describing complex surgical management of a paraganglioma located in the anterior mediastinum that was initially thought to be a thymoma.

HIGH BLOOD PRESSURE (HYPERTENSION) REVEALING PARAGANGLIOMA

Objective: Paraganglioma is a catecholamine-producing neuroendocrine tumor. Management of paraganglioma including its diagnosis is difficult, because it has no characteristic symptoms and many diseases can manifest as headache and high blood pressure. Herein, we report a 2 cases of paraganglioma of the abdomen with resitant arterial hypertension Design and method: Herein, we report a 2 cases of paraganglioma of the abdomen with resitant arterial hypertension Results: Case 1 A 24-year-old female presented with a history of resistant arterial hypertension. At the physical examination, we found high blood pressure. Full laboratory tests were requested and the following relevant data were found: noradrenaline elevated in blood and urine. An adrenal CT showed a retroperitoneal tissue mass on the inferior pole of the left kidney with heterogeneous enhancement. The scintigraphy concluded a left lumbar paravertebral paraganglioma. The diagnosis was confirmed by anatomopathological examination after surgery. We have noted a normalization of blood pressure after surgery. Case 2 A 46-year-old female with a history of recent arterial hypertension. She was receiving treatment with at three antihypertensive medications, including a diuretic and the blood pressure remained high. Full laboratory tests were requested and the following relevant data were found: metanephrine and noradrenaline elevated in blood and urine. A CT scann showed a retroperitoneal tissue. The scintigraphy concluded a paraganglioma. The diagnosis was confirmed by anatomopathological examination after surgery. The blood pressure became normal after surgery. Conclusions: Paragangliomas are relatively common. They constitute a cause of hypertension not to be ignored considering the serious consequences in the absence of surgical management.

A meta-analysis on the surgical management of paraganglioma of the carotid body per Shamblin class.

The aim of this study was to evaluate the risk associated with different types of surgery for carotid body paraganglioma of different Shamblin class. A meta-analysis was conducted to evaluate per tumour class, the local control, cranial nerve damage and complication rates of different techniques using internal carotid artery (ICA) and external carotid artery (ECA) ligation, clamping or bypassing, as well as the craniocaudal vs caudocranial techniques. A meta-analysis is conducted after a systematic search in PubMed and the Cochrane library, in accordance with the PRISMA guidelines. Local control, cranial nerve damage, complications, function recovery. Out of 3565 articles, 27 were selected. The overall quality of evidence of studies was low. Cranial nerve damage (3%, 17% and 39%) and complication rates (0%, 1% and 10%) were significantly related to Shamblin class (class 1, 2 and 3, respectively, P<.01). For class 3 tumours, an increased risk of complications was found associated with routine ICA manipulation/reconstruction (RR 3.12 with a 95% CI of 1.29-7.59), as well as a trend towards enhanced risk of routine ECA ligation (RR 3.48 with a 95% CI of 0.88-13.81). For class 1 and 2 tumours, surgery seems a viable treatment option. For class 3 tumours, morbidity in terms of cranial nerve deficit and complications is considerable; particularly, the use of ICA manipulation/reconstruction and potentially ECA ligation seem to be accompanied by high stroke incidence.

Partial Cystectomy of Paraganglioma of the Urinary Bladder Before Living Kidney Transplantation: Case Report

BackgroundParaganglioma (extra-adrenal pheochromocytoma) of the bladder is a very rare disease, accounting for 0.06% of all bladder tumors. Optimal management of bladder paraganglioma before kidney transplantation is unknown. We report a case of partial cystectomy for urinary bladder paraganglioma before living kidney transplantation. Case PresentationA 59-year-old man with a 27-year history of hemodialysis was referred to our department for further examination of a bladder tumor detected during pre-transplantation testing. Cystoscopy revealed a submucosal tumor on the right side of the bladder. The patient experienced a hypertensive crisis during transurethral resection of the bladder tumor. Endocrinologic and pathologic examinations confirmed the diagnosis of paraganglioma in the urinary bladder. A partial cystectomy was performed before kidney transplantation. Nine months after partial cystectomy, the patient underwent AB0-incompatible living kidney transplantation from his spouse. No disease recurrence or graft rejection was observed 12 months after the transplantation. ConclusionsTo our knowledge, this is the 1st report on the management of paraganglioma in the urinary bladder before living kidney transplantation. Kidney transplantation after partial cystectomy is an option that may be considered in patients with paraganglioma of the urinary bladder, with careful observations of bladder function and vesicoureteral reflux to the grafts.

Results of a systematic literature review of treatment modalities for jugulotympanic paraganglioma, stratified per Fisch class.

Key for successful jugulotympanic paraganglioma management is a personalised approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control and complication rates for the different treatment modalities stratified by the broadly accepted Fisch classification. A systematic literature review according to the PRISMA statement was performed. A detailed overview of individual treatment outcomes per Fisch class is provided. Local control, cranial nerve damage, complications, function recovery. Eighteen studies were selected, resembling 83 patients treated with radiotherapy and 299 with surgery. Excellent local control was found post-surgery for class A and B tumours, and risk of cranial nerve damage was <1%. For class C1-4 tumours, local control was 80%-95% post-surgery (84% post-radiotherapy), and cranial nerve damage was found in 71%-76% (none post-radiotherapy; P<.05). There was no difference in treatment outcomes between tumours of different C class. For class C1-4De/Di tumours, local control was 38%-86% (98% post-radiotherapy; P<.05) and cranial nerve damage/complication rates were 67%-100% (3% post-radiotherapy; P<.05). C1-4DeDi tumours showed lesser local control and cranial nerve damage rates when compared to C1-4De tumours. An individual risk is constituted for surgery and radiotherapy, stratified per Fisch class. For class A and B tumours, surgery is a suitable treatment option. For class C and D tumours, radiotherapy results in lower complication rates and similar or better local control rates when compared to the surgical group.

Perioperative management of paraganglioma and catecholamine-induced cardiomyopathy in child\u2013 a case report and review of the literature

BackgroundParagangliomas are catecholamine-secreting tumors of the paraganglia. Perioperative mortality of children with paraganglioma is high, but preoperative therapy and anesthetic management of paraganglioma resection are controversial in children. The literatures on catecholamine-induced cardiomyopathy are limited to several case reports,with few reports of studies on children.Case presentationHere we report the anesthetic management of a child with paraganglioma and catecholamine-induced cardiomyopathy, and the possible perioperative anesthesia problems of the paraganglioma resection are discussed.ConclusionPreoperative and intraoperative anesthetic management of Pheochromocytomas children should follow the same principles as for adults, The most important aspects are the control of blood pressure liability and maintenance of adequate blood volume. Pheochromocytomas patient may have cardiomoyopathy due to myocardial toxicity of excessive circulating catecholamines level. The perioperative management of catecholamine-induced cardiomyopathy should include lowering sympathetic activation by means of α-and β-adrenergic receptor blocker and diuretics administration in case of volume overload.

Head and Neck Paragangliomas: An Update on Evaluation and Management.

Head and neck paragangliomas are a group of slow-growing hypervascular tumors associated with the paraganglion system. The approach to evaluate and treat these lesions has evolved over the last 2 decades. While radical surgery had been the traditional approach, improvements in diagnostic imaging as well as radiation therapy techniques have led to an emphasis on observation and nonsurgical therapy in many patients. This article reviews the contemporary approach to the workup and management of head and neck paragangliomas. Articles were identified from PubMed. PubMed searches with the following keywords were performed: carotid body paraganglioma management, vagal paraganglioma management, jugulotympanic paraganglioma management, imaging of head and neck paragangliomas, head and neck paraganglioma embolization, paraganglioma radiation, head and neck paraganglioma management, observation of head and neck paragangliomas, bilateral carotid body paragangliomas, and genetics of paragangliomas. Review and original research articles available in the English language and published during or after 2009 were selected on the basis of their clinical relevance and scientific strength. Certain articles published prior to 2009 were also included if they provided background information that was relevant. Workup and treatment of head and neck paragangliomas are changing. With more now known regarding the longitudinal behavior of these tumors, observation and nonsurgical therapy are indicated in many instances. For patients where surgery is the most appropriate option, improved diagnostic and perioperative techniques are allowing patients to tolerate resection, often with reduced morbidity.

The Challenges of Treating Paraganglioma Patients with (177)Lu-DOTATATE PRRT: Catecholamine Crises, Tumor Lysis Syndrome and the Need for Modification of Treatment Protocols.

A high percentage of paragangliomas express somatostatin receptors that can be utilized for targeted radioisotope therapy. The aim of this study was to describe and discuss the challenges of treating these tumors with (177)Lu-[DOTA(0),Tyr(3)]octreotate (DOTATATE) radioisotope therapy using established protocols. Three paraganglioma patients were treated with 4-5 cycles of (177)Lu-DOTATATE and were evaluated for side effects and response to therapy. Two of the three patients developed severe adverse reactions following their first (177)Lu-DOTATATE treatment. One patient developed a catecholamine crisis and tumor lysis syndrome within hours of treatment, requiring intensive care unit (ICU) support, and another developed a catecholamine crisis 3days after treatment, requiring hospitalization. The treatment protocols at our institution were subsequently modified by increasing the radioisotope infusion time from 15 to 30min, as recommended in the literature, to 2-4h and by reducing the administered dose of (177)Lu-DOTATATE. Subsequent (177)Lu-DOTATATE treatments utilizing the modified protocols were well tolerated, and response to therapy was achieved in all three patients, resulting in significantly improved quality of life. (177)Lu-DOTATATE is an exciting new therapeutic option in the management of paragangliomas; however, current treatment protocols described in the literature may need to be modified by lengthening the infusion time and/or lowering the initial treatment dose to prevent or reduce the severity of adverse reactions.