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Extra-adrenal paragangliomas are rare neuroendocrine tumours originating from paraganglia outside the adrenal glands. The present case series illustrates the Computed Tomography (CT) imaging features of three instances of extra-adrenal abdominal paragangliomas. The discussed cases include Retroperitoneal paraganglioma with Inferior Vena Cava (IVC) infiltration and associated hepatic, pancreatic, and skeletal metastasis; Inter-aortocaval paraganglioma; and Organ of Zuckerkandl Paraganglioma. The CT imaging features of the lesions and their relation with the adjacent vascular structures are described. The differential diagnosis and histopathological correlations are discussed. These cases underscore the importance of considering paragangliomas in the differential diagnosis of soft-tissue masses in the abdomen and highlight the role of Contrast-enhanced CT (CECT) imaging in their evaluation. Understanding the diverse imaging characteristics of extra-adrenal paragangliomas aids in accurate diagnosis and appropriate management. Further studies are needed to expand the authors knowledge of these rare tumours and optimise their imaging evaluation.

Extra-adrenal paragangliomas are rare neuroendocrine tumours originating from paraganglia outside the adrenal glands. The present case series illustrates the Computed Tomography (CT) imaging features of three instances of extra-adrenal abdominal paragangliomas. The discussed cases include Retroperitoneal paraganglioma with Inferior Vena Cava (IVC) infiltration and associated hepatic, pancreatic, and skeletal metastasis; Inter-aortocaval paraganglioma; and Organ of Zuckerkandl Paraganglioma. The CT imaging features of the lesions and their relation with the adjacent vascular structures are described. The differential diagnosis and histopathological correlations are discussed. These cases underscore the importance of considering paragangliomas in the differential diagnosis of soft-tissue masses in the abdomen and highlight the role of Contrast-enhanced CT (CECT) imaging in their evaluation. Understanding the diverse imaging characteristics of extra-adrenal paragangliomas aids in accurate diagnosis and appropriate management. Further studies are needed to expand the authors knowledge of these rare tumours and optimise their imaging evaluation. [ABSTRACT FROM AUTHOR]

Extra-adrenal pheochromocytomas are rare neuroendocrine tumors originating outside the adrenal glands and can pose significant diagnostic challenges due to their variable presentations. This report highlights a case of an extra-adrenal pheochromocytoma masquerading as a pancreatic head malignancy. We underscore the importance of considering extra-adrenal pheochromocytoma in the differential diagnosis of pancreatic masses, particularly when biochemical or clinical features suggest catecholamine excess. Early recognition and appropriate management are crucial to avoid unnecessary interventions and improve patient outcomes., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Habeb et al.)

Pheochromocytoma and paraganglioma are often associated with hereditary syndromes, particularly those involving genes such as RET , which is linked to multiple endocrine neoplasia type 2A. Genetic testing plays a crucial role in diagnosing these conditions, guiding treatment strategies, and providing early intervention options for the affected families. A 57-year-old man presenting with back pain was found to have a left adrenal tumor and a retroperitoneal tumor near the left renal hilum. Hormonal studies and imaging confirmed the diagnosis of pheochromocytoma and paraganglioma. The patient underwent robot-assisted laparoscopic surgery to remove the tumors. Postoperative multigene panel testing identified a mutation in RET  c.1901G > T (p.Cys634Phe) leading to the diagnosis of multiple endocrine neoplasia type 2A. Despite this genetic finding, no other endocrine tumors, such as medullary thyroid cancer or hyperparathyroidism, were detected at the time of diagnosis. The patient remains under close surveillance for the potential development of associated conditions. This case highlights the importance of comprehensive genetic testing in patients with pheochromocytoma and paraganglioma, particularly when hereditary syndromes are suspected. Genetic insights ensure precise management, allowing for tailored treatment and improved outcomes in patients with hereditary pheochromocytoma and paraganglioma., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. The ethics committee of the Daiyukai Health System issued approval 2024-016. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Takagi et al.)

Background and Objectives: The aim of this study is to explore the long-term prognostic risk factors associated with patients diagnosed with retroperitoneal paraganglioma (RPGL) and examine their clinical and pathological characteristics., Methods: Expressions of biomarkers were identified using immunohistochemistry (IHC) and case databases were retrospectively searched. Survival analysis was performed using Kaplan-Meier and Cox risk regression to identify the factors that influence the postoperative progression-free survival of patients with RPGL., Results: A total of 105 patients, most of whom had tumors situated in the paraaortic region, and whose average tumor size was 8.6 cm, were enrolled in this study. The average follow-up duration was 51 months, with a mortality rate of 19% and a recurrence and metastasis rate of 41.9%. Tumors were assessed using the modified Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP), and SDHB, S-100, and Ki-67 were stained using IHC in all cases. Out of the total cases examined, negative in SDHB expression were observed in 18.1% of cases, S-100 expression was negative in 36.2% of cases, and endovascular tumor enboluswas present in approximately 25.7% of cases. The results of the univariate analysis indicated that several factors significantly influenced the progression-free survival of patients with PGL as follow: maximum tumor diameter (>5.5 cm), tumor morphological features, tumor grading (modified GAPP score > 6), SDHB negative, S-100 negative, and expression of proliferation index Ki-67 (>3%) (X 2  = 4.217-27.420, p < 0.05). The results of the multivariate analysis indicated that negative of S-100 (p = 0.021) and SDHB (p = 0.038), as well as intravascular tumor thrombus (p = 0.047) expression were independent risk factors for progression-free survival in patients., Conclusion: RPGL is characterized by diverse biological features and an elevated susceptibility to both recurrence and metastasis. Both SDHB and S-100 can be employed as traditional IHC indicators to predict the metastatic risk of PGL, whereas the tumor histomorphology-endovascular tumor enbolus assists in determining the metastasis risk of RPGL., (© 2024 Wiley Periodicals LLC.)

Retroperitoneal paraganglioma remains an extremely rare type of tumor that arises from either sympathetic or parasympathetic neural crest cells. It could be functional or non-functional. Non-functional paraganglioma may present as a diagnostic challenge since patients are usually asymptomatic and tend to present to the hospital with complications from the invasion of the tumor. Malignancy is usually determined by the degree of metastasis. The gold standard of diagnosis is biopsy and obtaining a sample for histological examination. This author presents a case of asymptomatic, non-functional retroperitoneal paraganglioma with distant metastasis., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Lingas et al.)

Paraganglioma is a rare type of neuroendocrine tumor with variable clinical presentations, making diagnosis relatively challenging. In this report, we present a case of retroperitoneal paraganglioma in a patient who experienced intermittent episodes of dizziness and chest pain. Imaging studies conducted during the patient's hospitalization revealed the presence of a lesion in the upper region of the right kidney, as well as a mass in the left retroperitoneal area that was suspected to be a paraganglioma. Biochemical studies were collected, including 24-hour urine metanephrines, urine catecholamines, urine cortisol, plasma metanephrines, renin, and aldosterone. However, it took an extended period of time for these results to come back. Given high clinical suspicion, alpha-blockade was initiated without a definite diagnosis of paraganglioma. Ultimately, the patient underwent tumor resection and the final pathology confirmed paraganglioma. The pathology of the contralateral renal mass showed oncocytoma. This case serves as an illustration of the difficulties that can arise when diagnosing and treating undiagnosed paragangliomas within a community healthcare setting., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Chen et al.)

Introduction: Paragangliomas are rare neuroendocrine tumors that arise from chromaffin cells. Often termed extra-adrenal pheochromocytomas, these tumors vary with regards to their functionality, location, and malignant potential. Mutations in the RET proto-oncogene are associated with multiple endocrine neoplasia syndrome type 2 (MEN-2) and paragangliomas. The phenotypes of the individual mutations are documented to help determine prognosis., Case Presentation: We report a case of a 64-year-old man with a history of parathyroid adenoma who developed a pancreatic retroperitoneal paraganglioma. Despite having laboratory evidence of excess circulating catecholamines, the patient's only presenting symptom was hip pain. The patient underwent resection, and histologic findings were consistent with paraganglioma with lymph node metastasis. Genetic testing revealed a variant of uncertain significance within the RET gene [c.731C>T (p.T244I)]., Conclusions: Paragangliomas are rare extra-adrenal neuroendocrine tumors that can be associated with germline mutations. Our patient was diagnosed with a pancreatic paraganglioma associated with a RET T244I mutation. Identifying patients with germline mutations is important for documenting phenotypic presentations of RET gene variants of uncertain significance, which will allow physicians to provide proper management and surveillance of paragangliomas and other associated tumors., Competing Interests: Conflict of Interests: The authors of this manuscript have no personal or financial conflicts of interest to declare. All authors are employed by Danbury Hospital, which is part of the Nuvance Health System, and have no further association with other organizations., (Copyright © 2022, International Journal of Endocrinology and Metabolism.)