Jugular Foramen Paragangliomas.

Paragangliomas are the most common tumors at jugular foramen and pose a great surgical challenge. Careful clinical history and physical examination must be performed to adequately evaluate neurological deficits and its chronologic evolution, also to delineate an overview of the patient performance status. Complete imaging evaluation including MRI and CT scans should be performed, and angiography is a must to depict tumor blood supply and sigmoid sinus/internal jugular vein patency. Screening for multifocal paragangliomas is advisable, with a whole-body imaging. Laboratory investigation of endocrine function of the tumor is necessary, and adrenergic tumors may be associated with synchronous lesions. Preoperative prepare with alpha-blockage is advisable in norepinephrine/epinephrine-secreting tumors; however, it is not advisable in exclusively dopamine-secreting neoplasms. Best surgical candidates are young otherwise healthy patients with smaller lesions; however, treatment should be individualized each case. Variations of infratemporal fossa approach are employed depending on extensions of the mass. Regarding facial nerve management, we avoid to expose or reroute it if there is preoperative function preservation and prefer to work around facial canal in way of a fallopian bridge technique. If there is preoperative facial nerve compromise, the mastoid segment of the nerve is exposed, and it may be grafted if invaded or just decompressed. A key point is to preserve the anteromedial wall of internal jugular vein if there is preoperative preservation of lower cranial nerves. Careful multilayer closure is essential to avoid at most cerebrospinal fluid leakage. Residual tumors may be reoperated if growing and presenting mass effect or be candidate for adjuvant stereotactic radiosurgery.

Intraoperative radiofrequency ablation for unresectable abdominal paraganglioma: a case report.

For pheochromocytoma and paraganglioma (PPGL), the efficacy of percutaneous ablative therapies in achieving control of metastatic tumors measuring <3 cm had been demonstrated in only few reports, and intraoperative radiofrequency ablation (RFA) of locally invasive primary PPGLs has not been reported. We presented the case of a 31-year-old man who had a 9-cm functioning unresectable PPGL. He was treated with 13 cycles of cytotoxic chemotherapy without objective tumor response, according to the Response Evaluation Criteria in Solid Tumors (RECIST). Subsequently, magnetic resonance imaging revealed a 9.0 × 8.6 × 6.0-cm retroperitoneal mass that extended to the inferior portion of the inferior vena cava, the inferior mesenteric artery, and the infrarenal aorta. Biochemical evaluation demonstrated high level of plasma normetanephrine (20.2 nmol/L, normal range <0.9 nmol/L). Genetic investigation showed the germline pathogenic variant c.1591delC (p. Ser198Alafs*22) in the SDHB gene. I131-metaiodobenzylguanidine scintigraphy was negative and Ga68-dotatate PET-CT scan showed high tumor uptake without distant metastases. On open laparotomy, tumor debulking was not possible. Therefore, intraoperative RFA was performed by a highly experienced team of interventional radiologists. At 12 months after the RFA, the tumor volume decreased from 208 to 45 mL (78%), plasma normetanephrine decreased from 20.2 to 2.6 nmol/L (87%), and the doxazosin dose was reduced from 16 to 8 mg/day. To our best knowledge, this was the first report on intraoperative RFA that markedly reduced the size of a large primary unresectable PPGL, along with clinical and biochemical responses.

Management of Abdominal Paraganglioma: A Single Center's Experience.

Background and Objectives: Paragangliomas (PGLs) are rare neuroendocrine extra-adrenal tumors that could be secreting mass. The symptoms are the typical triad of paroxysmal headache, hypertension and sweating, but could also be accompanied by symptoms involving multiple organs. Surgery is the gold standard treatment for both PGLs and pheochromocytomas (PHEOs). Material and Methods: We used a computerized endocrine surgery registry to record the demographic and clinical data of 153 patients who underwent surgery for PPGL between 2010 and 2023 at our hospital. Results: Thirteen patients (8.43%) with paragangliomas underwent surgery at our institute. Five patients presented symptomatic syndrome. Preoperative investigations included enhanced abdominal CT (nine patients) and enhanced MRI (seven patients). In cases of suspicious mass, we performed 131I-MIBG scans (two patients) or 68GA-DOTATOC PET-CT scans (11 patients). Laparoscopic approach was used in four cases (30.7%) and abdominal laparotomy in the other nine (69.3%). Biochemical tests were performed on all patients. Conclusions: In this retrospective study, we discuss the multidisciplinary management in our institute of this rare disease, from its challenging diagnosis to the surgical strategy for PGLs. Laparoscopic surgery is the gold standard, but a tailored approach should be adopted for each patient.

Paraganglioma of the urinary bladder: A case report.

It is very un comm on to discover Paraga ngliomas in the uri nar y bladder. Thes e tu mo ur s origina te from the sympathetic nerves which supp ly chromaffin cells in the bladde r wall. They can be classified as functional or nonfunctional. If functional, the most common presentations are with hypertensive crisis or post-micturition syncope. A si lent pa raganglioma of th e bl adder ca n be easily m isdiagnosed w hich can resu lt in severe pe ri- oper ative morbidity. We pre sent a ca se of a male pati ent who was being m a naged fo r hype rt ension for 2-3 ye ars. He presented at The Indus Hospital, Karachi o n 7th August 2022 with gross painless haematuria. An ultrasound scan revealed an echogenic lesion aris ing from base of the urinar y bladder, wh ic h was trea ted via Transurethral Resection o f Bladde r Tumour (TURBT). Histopathological report revealed Paraganglioma of the Bladder. He was later scheduled for Partial Cystectomy (PC) and has been doing well ever since.

Retroperitoneal venous malformation mimics paraganglioma on triple imaging modalities.

Here, we report a case of a woman in her 50s who was referred for an incidentally discovered lesion anterior to the origin of inferior vena cava suspicious for a paraganglioma following a CT scan for vaginal bleeding. A follow-up 68Ga-DOTATATE PET-CT and MRI of the abdomen reinforced the radiological impression of an extra-adrenal paraganglioma (EAP). The EAP was deemed non-functional given normal urine biogenic amine, supine plasma fractionated metanephrines and chromogranin A levels. The mass was resected laparoscopically without perioperative blockade. Histological examination revealed a venous malformation. Extrahepatic abdominal venous malformations are rare and can be indistinguishable from an EAP on imaging preoperatively. Although benign, the lesion nevertheless warrants excision as it is associated with a risk of haemorrhage.

Intraoperative hybrid technique for excision of temporal bone paraganglioma: A case report.

BACKGROUND: Temporal bone paragangliomas are vascularized neoplasms. Although preoperative angioembolization serves as a valuable approach to reduce intraoperative blood loss, it comes with an elevated risk of cranial neuropathies, offers no assurance of complete hemostasis, and precludes real-time adjustments during surgery. METHODS: A 74-year-old patient presented with recurrent episodes of ear bleeding. On examination, a vascular lesion obstructed her external auditory canal. It had the clinical and radiological characteristics of a paraganglioma. Angiography revealed that it had three feeding vessels. RESULTS: The patient was successfully scheduled for hybrid, intraoperative angiography and temporary balloon occlusion of the feeding vessels supplying the lesion instead of preoperative angioembolization. CONCLUSIONS: Utilizing hybrid intraoperative angiography with temporary balloon occlusion during the surgical removal of temporal bone paragangliomas represents an innovative technique that reduces the risk of permanent cranial neuropathies while providing the capacity for real-time adjustments and improved hemostasis.

Imaging of Pheochromocytomas and Paragangliomas.

Pheochromocytomas/paragangliomas are unique in their highly variable molecular landscape driven by genetic alterations, either germline or somatic. These mutations translate into different clusters with distinct tumor locations, biochemical/metabolomic features, tumor cell characteristics (eg, receptors, transporters), and disease course. Such tumor heterogeneity calls for different imaging strategies in order to provide proper diagnosis and follow-up. This also warrants selection of the most appropriate and locally available imaging modalities tailored to an individual patient based on consideration of many relevant factors including age, (anticipated) tumor location(s), size, and multifocality, underlying genotype, biochemical phenotype, chance of metastases, as well as the patient's personal preference and treatment goals. Anatomical imaging using computed tomography and magnetic resonance imaging and functional imaging using positron emission tomography and single photon emission computed tomography are currently a cornerstone in the evaluation of patients with pheochromocytomas/paragangliomas. In modern nuclear medicine practice, a multitude of radionuclides with relevance to diagnostic work-up and treatment planning (theranostics) is available, including radiolabeled metaiodobenzylguanidine, fluorodeoxyglucose, fluorodihydroxyphenylalanine, and somatostatin analogues. This review amalgamates up-to-date imaging guidelines, expert opinions, and recent discoveries. Based on the rich toolbox for anatomical and functional imaging that is currently available, we aim to define a customized approach in patients with (suspected) pheochromocytomas/paragangliomas from a practical clinical perspective. We provide imaging algorithms for different starting points for initial diagnostic work-up and course of the disease, including adrenal incidentaloma, established biochemical diagnosis, postsurgical follow-up, tumor screening in pathogenic variant carriers, staging and restaging of metastatic disease, theranostics, and response monitoring.

Tumor progression in tympanojugular paragangliomas: the role of radiotherapy and wait and scan.

INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.

The Great Imitator: Pulmonary Paraganglioma Mimicking Pulmonary Metastasis of Breast Cancer on 18 F-FDG PET/CT.

ABSTRACT: Primary pulmonary paragangliomas are rare neuroendocrine tumors and are often discovered incidentally as nonfunctioning masses. We report a case of 58-year-old woman recently diagnosed with multifocal right breast cancer and suspicious right axillary lymph nodes. 18 F-FDG PET/CT showed a well-defined marginated lesion in the lower lobe of the right lung with intense FDG uptake. Findings were suspicious for pulmonary metastases. CT-guided biopsy results were consistent with a paraganglioma. Paragangliomas can mimic metastases, especially if they are large or have a high degree of FDG uptake, and careful evaluation of imaging features and clinical findings can help differentiate these entities. Most of the time biopsy is necessary to confirm the diagnosis.

Assessing the safety and efficacy of EVOH Pre-Embolization in head and neck paraganglioma tumors: A comprehensive analysis of current literature.

BACKGROUND: Head and Neck Paragangliomas are characterized by having a rich blood supply. Presurgical embolization with Onyx as a neoadjuvant treatment is not a consensus regarding its efficacy and safety. Our study aimed to answer this matter through a single-arm meta-analysis. METHODS: We systematically reviewed 4 databases. Sixteen studies were described and suitable papers were selected for meta-analysis of estimated intraoperative blood loss (EBL), percentage of tumor devascularization, and complications associated with embolization. RESULTS: The study identified 198 patients with 203 tumors, aged between 8 and 70 years. Commonly reported symptoms included neck mass perception and cranial nerve impairment. Carotid Body Tumors were most prevalent (127, 62.5 %), followed by jugular (48, 23.6 %), or vagal (29, 14.2 %) tumors. Eight studies reported estimated intraoperative blood loss (EBL) averaging 261.89 ml (95 %CI: 128.96 to 394.81 ml). In an analysis of 9 studies, 99 % (95 %CI: 96 to 100 %) achieved 70 % or more devascularization, and 79 % (95 %CI: 58 to 100 %) achieved 90 % or more devascularization. Complications from endovascular procedures were observed in 3 % (95 %CI: 0 to 8 %) of 96 patients across 10 studies, including 4 facial nerve deficits. Eighteen postoperative neurological deficits were reported across 15 articles. CONCLUSION: Despite acknowledged limitations, with refined indications, EVOH, especially Onyx embolization may significantly bolster patient safety, decreasing EBL and easing surgical resection. Further research with larger studies will refine criteria, optimize techniques, and improve patient care and treatment outcomes in the management of head and neck paragangliomas.

Cauda equina neuroendocrine tumors: A single institutional imaging review of cases over two decades.

Cauda Equina Neuroendocrine Tumors (CE-NET), previously referred to as paragangliomas are a rare subset of spinal tumors, with limited data on imaging. Herein, we present a retrospective review of clinical and imaging findings of CE-NETs in ten patients who were evaluated at our institution over the past two decades. All patients had well-defined intradural lesions in the lumbar spine which demonstrated slow growth. A review of imaging findings revealed the presence of an eccentric vascular pedicle along the dorsal aspect of the tumor in 8 of the 10 patients (eccentric vessel sign), a distinctive finding that has not previously been reported with this tumor and may help improve the accuracy of imaging-based diagnosis. In all cases, a gross-total resection was performed, with resolution of symptoms in most of the cases.

Complete Response to 177 Lu-DOTATATE PRRT in a 9-Year-Old Child With Metastatic Carotid Body Paraganglioma.

ABSTRACT: We present a case involving a 9-year-old boy diagnosed with metastatic carotid body paraganglioma. The metastases were detected in cervical lymph nodes and lungs using 68 Ga-DOTANOC PET/CT imaging. The patient received peptide receptor radionuclide therapy with 177 Lu-DOTATATE. Following 3 treatment cycles, a significant improvement was observed in the metastatic lesions. After 4 cycles, the patient achieved a complete response, with a cumulative administered activity of 16.65 GBq during the therapy. This case underscores the effectiveness of using 177 Lu-DOTATATE in managing metastatic carotid body paraganglioma, offering promising results in terms of tumor regression and overall therapeutic response.

68 Ga-DOTATATE PET to Characterize Lesions in the Neuroaxis.

AIM: The differentiation of paragangliomas, schwannomas, meningiomas, and other neuroaxis tumors in the head and neck remains difficult when conventional MRI is inconclusive. This study assesses the utility of 68 Ga-DOTATATE PET/CT as an adjunct to hone the diagnosis. PATIENTS AND METHODS: This retrospective study considered 70 neuroaxis lesions in 52 patients with 68 Ga-DOTATATE PET/CT examinations; 22 lesions (31%) had pathologic confirmation. Lesions were grouped based on pathological diagnosis and best radiologic diagnosis when pathology was not available. Wilcoxon rank sum tests were used to test for differences in SUV max among paragangliomas, schwannomas, and meningiomas. Receiver operator characteristic curves were constructed. RESULTS: Paragangliomas had a significantly greater 68 Ga-DOTATATE uptake (median SUV max , 62; interquartile range [IQR], 89) than nonparagangliomas. Schwannomas had near-zero 68 Ga-DOTATATE uptake (median SUV max , 2; IQR, 1). Intermediate 68 Ga-DOTATATE uptake was seen for meningiomas (median SUV max , 19; IQR, 6) and other neuroaxis lesions (median SUV max , 7; IQR, 9). Receiver operator characteristic analysis demonstrated an area under the curve of 0.87 for paragangliomas versus all other lesions and 0.97 for schwannomas versus all other lesions. CONCLUSIONS: Marked 68 Ga-DOTATATE uptake (>50 SUV max ) favors a diagnosis of paraganglioma, although paragangliomas exhibit a wide variability of uptake. Low to moderate level 68 Ga-DOTATATE uptake is nonspecific and may represent diverse pathophysiology including paraganglioma, meningioma, and other neuroaxis tumors but essentially excludes schwannomas, which exhibited virtually no uptake.

Endoscopic papillectomy combined with endoscopic retrograde cholangio-pancreatography for duodenal gangliocytic paraganglioma: A case report.

RATIONALE: Gangliocytic paraganglioma is a rare tumor that can occur in several organs throughout the body. Gangliocytic paraganglioma of the main duodenal papilla is even rarer. This study analyzes and discusses the endoscopic management of a case of gangliocytic paraganglioma of the main duodenal papilla and reviews the relevant literature. It is hoped that this study will increase clinicians' awareness of this disease. PATIENT CONCERNS: Electron endoscopy reveals a duodenal main papillary tumor, and the patient desires further clarification of the nature of the tumor and the next step in the treatment plan. DIAGNOSES: Duodenal gangliocytic paraganglioma. INTERVENTIONS: As the patient lesion was located in the main duodenal papilla, we successfully performed endoscopic minimally invasive treatment of the tumor by endoscopic papillectomy combined with endoscopic retrograde cholangiopancreatography. OUTCOMES: The patient was discharged after the postoperative removal of the nasobiliary drain and returned to the hospital 2 months later to have the biliary stent removed; the patient was in good general condition at follow-up. LESSONS: For duodenal main papillary tumor, we need to be alert to the possibility of gangliocytic paraganglioma. Since the tumor is located in the submucosa of the juxta-abdominal region, the preoperative biopsy positivity rate is low, and the tumor is often adjacent to or involves the biliopancreatic duct, endoscopic resection combined with endoscopic retrograde cholangiopancreatography can be considered for diagnosis and treatment.

An incidentally discovered paraganglioma that caused sinus arrest after resection.

Paragangliomas are neural-crest-derived nonepithelial neuroendocrine tumors distributed along the parasympathetic and sympathetic nerves. To our knowledge, no studies were reported regarding sinus arrest on day 4 after paraganglioma resection. A 66-year-old female patient with a history of pulmonary vein isolation visited our department for sigmoid colon cancer treatment. Enhanced computed tomography revealed an enhanced small nodule-like lymph node near the root of the inferior mesenteric artery. The patient underwent laparoscopic colectomy with regional lymph node dissection. Postoperatively, paroxysmal atrial fibrillation attacks developed, and the patient resumed oral medication. Additionally, sinus arrest after tachycardia developed. Changing the oral medication could maintain her circulatory dynamics. Pathological examination revealed that differentiated tubular adenocarcinoma infiltrated the submucosa. Immunohistochemically, the excised nodule as a lymph node was considered a functional paraganglioma. Our case indicates that paraganglioma resection and oral medication resumption may contribute to sinus arrest. When arrhythmias affecting the circulation occur perioperatively, the presence of a catecholamine-producing tumor should be considered in addition to cardiac disease. J. Med. Invest. 70 : 503-507, August, 2023.

Incidentally detected retroperitoneal paraganglioma in a 10-year-old French bulldog.

This article reports a case of a 10-year-old French bulldog initially seen for reluctance to move and episodes of pain. A magnetic resonance imaging study was undertaken in order to rule out a herniated disc. A large, retroperitoneal mass was visualized and cytological analysis suggested a neoplastic proliferation. The mass appeared to compress the caudal vena cava when viewed by abdominal CT scan. The mass was surgically removed. A nephrectomy was also carried out and aortic bleeding identified after dissection of adhesions. Despite these complications, the dog did well after the procedures. Postoperative checkups were normal. Histological and immunohistochemical analyses of the mass were compatible with a retroperitoneal paraganglioma. Key clinical message: This type of tumor is poorly described in the veterinary literature. As the behavior of this tumor type is not yet fully understood, each new description adds to our knowledge and should help in diagnosing and treating it more effectively in the future.

Paragangliome rétropéritonéal de découverte fortuite chez un bouledogue Français de 10 ans. Cet article expose le cas d'un chien mâle entier bouledogue Français de 10 ans présenté initialement pour des difficultés locomotrices et des manifestations algiques. Un examen d'imagerie par résonnance magnétique (IRM) est rapidement réalisé afin d'explorer l'hypothèse d'une hernie discale. Une volumineuse masse rétropéritonéale est alors mise en évidence. L'analyse cytologique de cette dernière est compatible avec un processus néoplasique. Après la réalisation d'un examen par tomodensitométrie de l'abdomen et la mise en évidence d'une compression marquée de la veine cave caudale par la masse, une prise en charge chirurgicale avec exérèse de la masse est décidée. Lors de l'intervention chirurgicale une néphrectomie est réalisée et un saignement aortique est identifié après la dissection des adhérences. Malgré ces complications, le chien se réveille bien et les contrôles post opératoires effectués sont satisfaisants. Les analyses histologiques et immunohistochimiques de la masse seront en faveur d'un paragangliome rétropéritonéal extra surrénalien.Message clinique clé :Ce type tumoral fait l'objet de peu de descriptions dans la littérature vétérinaire. Toute la lumière n'a pas encore été faite sur son comportement et chaque nouvelle description permet d'en enrichir les connaissances et donc de mieux comprendre ce type tumoral, ce qui, à l'avenir, pourra aider à le diagnostiquer plus facilement et à le traiter plus efficacement.(Traduit par les auteurs).