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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]

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.)

Pheochromocytoma/paraganglioma (PPGL)-related hypercatecholaminemic crisis is a rare lethal condition caused by uncontrolled catecholamine secretion, occasionally leading to critical fluctuation in blood pressure (BP). Emergent transcatheter arterial embolization (TAE) has been employed for spontaneous PPGL rupture, but never, to our knowledge, for critical fluctuation in BP associated with PPGL-related hypercatecholaminemic crisis. We describe here our experience utilizing this method to control critical fluctuation in BP associated with this crisis in a 44-year-old man with an unruptured retroperitoneal paraganglioma. The patient experienced sudden severe left abdominal pain and came to our emergency department, where he exhibited severe fluctuation in BP and underwent laboratory testing that showed hypercatecholaminuria and computed tomography (CT) that revealed a left retroperitoneal tumor with no apparent intra- or retroperitoneal hematoma. We performed emergent TAE from the left inferior phrenic artery using gelatin sponge, which stabilized his BP and relieved his abdominal pain. Histologic examination following elective surgical resection of the tumor confirmed our diagnosis of unruptured retroperitoneal paraganglioma. We believe that TAE represents an important option for the emergent treatment of the critical BP fluctuation associated with PPGL-related hypercatecholaminemic crisis.

Introduction: paragangliomas are very rare extra-adrenal tumors originating from the autonomic nervous system. They are classified in functioning or non-functioning tumors, according to the production of catecholamines. Patient information: 40-year-old woman, with a history of severe arterial hypertension, who had been treated with three hypotensive drugs without obtaining control; she began to suffer diffuse abdominal pain that increased, associated with hyperpolymenorrhea and a periumbilical tumor of approximately 8 cm, painful and mobile. Laboratory tests were normal. Computed tomography and magnetic resonance imaging confirmed the diagnosis of an 8×5 cm retroperitoneal tumor attached to the aorta and displacing the inferior cava. It was necessary to carry out a highly complex surgical intervention. A tumor was found and the histological and immunohistochemical results confirmed the existence of a non-functioning extra-adrenal paraganglioma. Conclusions: retroperitoneal paraganglioma is a rare tumor, as shown by statistics. The diagnosis was made, fundamentally, by imaging, histological and immunohistochemical studies. Treatment is fundamentally surgical, as was performed in this patient. [ABSTRACT FROM AUTHOR]

Abstract Background Paragangliomas (PGLs) are extremely rare neuroendocrine tumours arising from extra-adrenal chromaffin cells. PGLs are clinically rare, difficult to diagnose and usually require surgical intervention. PGLs mostly present catecholamine-related symptoms. We report a case of Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal PGL. There has been only one similar case reported in literature. Case presentation We present a unique case of a 52-year-old female with acute abdomen induced by haemorrhagic retroperitoneal PGL. The patient had a 5-h history of sudden onset of serve right lower quadrant abdominal pain radiating to the right flank and right lumbar region. Patient had classic symptoms of acute abdomen. Abdominal ultrasound revealed a large abdominal mass with a clear boundary. A Computed Tomography Angiography (CTA) of superior mesenteric artery was also performed to in the emergency department. The CTA demonstrated a large retroperitoneal mass measured 9.0 × 7.3 cm with higher density inside. A provisional diagnosis of retroperitoneal tumour with haemorrhage was made. The patient received intravenous fluids, broad-spectrum antibiotics and somatostatin. On the 3rd day of admission, her abdominal pain was slightly relieved, but haemoglobin decreased from 10.9 to 9.4 g/dL in 12 h suggesting that there might be active bleeding in the abdominal cavity. Thus, we performed a midline laparotomy for the patient. Haemorrhage was successfully stopped during operation. The retroperitoneal tumour with haemorrhage was completely removed. The abdominal pain was significantly relieved after surgery. The patient initially presented with acute abdomen instead of catecholamine-related symptoms. The diagnosis of retroperitoneal PGL with haemorrhage was finally confirmed by postoperative pathological and immunohistochemical results. The postoperative course was uneventful. At the 1-year follow-up visit, no tumour recurrence was observed by Single Photon Emission Computed Tomography. A literature review was performed to further understand and analyse the aforementioned disease. Conclusion Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal paraganglioma is extremely rare. Abdominal Computed Tomography is essential to locate the lesion and differentiate between other causes of acute abdomen. PGLs are hypervascular tumours. We should be aware that ruptured retroperitoneal PGL with massive bleeding could be life threatening and require emergency laparotomy.

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.)

Introduction: Retroperitoneal paraganglioma (RPGL) is a rare clinical tumor derived from the retroperitoneal sympathetic paraganglion tissue. Since RPGLs are locate deeply and have no specific symptoms and imaging manifestations at the early stage, which easily causes missed diagnosis or misdiagnosis. In addition, reports on totally laparoscopic resection of RPGLs are scarce due to their close proximity to large vessels, giant size, uncertain location, and unknown malignant status.Case Presentation: We present here the case of totally laparoscopic resection of a 6.4 × 5.4 cm RPGL that was discovered during a workup for discomfort and upper abdominal pain in a 68-year-old female patient, mimicking a gastrointestinal stromal tumor (GIST) of the duodenum, Which was confirmed as a RPGL based on the histopathological and immunohistochemical findings.Conclusions: RPGL is a rare tumor, and the transperitoneal laparoscopic approach for the RPGL is a safe, applicable method with less trauma and quick recovery, which is worth clinical popularizing and application. Moreover, the survival prognosis of RPGL patients are related to metastasis, and lifelong follow-up should be emphasized.

Laparoscopic resection of a retroperitoneal paraganglioma is considered challenging because of variable anatomical presentation and potential vascular injury during surgical manipulation. Due to the rarity of the entity, only a few studies using this technique have been reported. Furthermore, a ruptured paraganglioma which manifests in traumatic retroperitoneal hemorrhage is exceedingly uncommon.Herein, we reported a case of a 12-year-old girl who presented with a ruptured retroperitoneal paraganglioma due to abdominal blunt trauma. Her contrast-enhanced computed tomography scan revealed a retroperitoneal hemorrhage and an abdominal mass of approximately 4 cm in diameter surrounded by the aorta, inferior vena cava, and left renal vein. A preoperative diagnosis of retroperitoneal paraganglioma was made from her 24 hour urinary catecholamine level, a clonidine loading test, and 123I-MIBG scintigraphy and elective laparoscopic resection was performed. Despite of dense peritumoral adhesion, a clear and magnified view of the surgical field and meticulous manipulation of the tumor allowed this laparoscopic resection to be successful, minimally invasive, and safe.

A 38 years old male worker presented with an advanced left side retroperitoneal mass, with metastatic involvement of 11th Thoracic Vertebra (T11) and resultant paraparesia and paresthesia . The histopathologic examination and immunohistochemical analysis confirmed the diagnosis of paraganglioma. Decompression laminectomy and fixation of involved vertebra resulted to considerable symptomatic relief. Due to unresectability at presentation, chemotherapy with palliative intent performed, and resulted to mild response. Palliative debulking performed after chemotherapy and resulted to better quality of patient's life during next 8 months. Remaining mass at pelvic area increased in size and patient received (131)I-MIBG therapy, 8 months post-operatively. A second debulking surgery, as well as other palliative measures will be considered for future.

Extra-adrenal retroperitoneal paragangliomas (PGLs) are rare tumors causing considerable difficulty in both, diagnosis and treatment. They can be unicentric or multicentric, tend to be locally invasive and therefore have a high incidence of local recurrence. PGLs shows somatostatin receptor positivity, which can be imaged with technetium-99m (Tc-99m)-hydrazinonicotinyl-Tyr3-octreotide (HYNIC-TOC) and can be treated with lutetium-177 (Lu-177) DOTATATE. We present a case of recurrent unresectable retroperitoneal PGL with nodal and bone metastases in a 27-year-old male, 6 months postsurgery detected with Tc-99m-HYNIC-TOC and was administered with peptide receptor radionuclide therapy using Lu-177 DOTATATE. [ABSTRACT FROM AUTHOR]

A 38 years old male worker presented with an advanced left side retroperitoneal mass, with metastatic involvement of 11th Thoracic Vertebra (T11) and resultant paraparesia and paresthesia. The histopathologic examination and immune-histochemical analysis confirmed the diagnosis of paraganglioma. Decompression laminectomy and fixation of involved vertebra resulted to considerable symptomatic relief. Due to inability of resection at presentation, chemotherapy with palliative intent performed, and resulted to mild response. Palliative debulking performed after chemotherapy and resulted to better quality of patient's life during next 8 months. Remaining mass at pelvic area increased in size and patient received (131) I-MIBG therapy, 8 months post-operatively. A second debulking surgery, as well as other palliative measures will be considered for future. [ABSTRACT FROM AUTHOR]

Background. Paraganglioma develop from embryological neural crest cells from the base of the skull, mediastinum, retroperitoneum, urinary bladder and scrotum. The most common location of the retroperitoneal form is situated between the aorta and vena cava, at the level of left renal vein, or more distally, to the aortic bifurcation. The care of patients with paraganglioma is a challenge for many physicians and surgeons because of its clinical features and therapeutic implications. Case presentation. We report the case of a 22-years old female patient with symptomatic retroperitoneal paraganglioma who was successfully treated with complete surgical excision. The pathological report confirmed the presence of chromaffin tumours. Postoperative care was uneventful, the patient being discharged after 10 days. Follow-up evaluation showed no recurrence after 4 years. Conclusion. Although a very rare condition, retroperitoneal paraganglioma has to be considered in the differential diagnosis of retroperitoneal tumours. Complete surgical resection is crucial for treatment and histological assessment as these tumours are potentially curable if they are diagnosed correctly. In our experience, the best surgical approach is open surgical excision for large masses located in the interaortocaval region, with a clear operatory field and good control over the major blood vessels. Follow-up is strongly recommended. [ABSTRACT FROM AUTHOR]

Gastrointestinal stromal tumor (GIST) and paraganglioma are uncommon tumors; mostly occur in a sporadic and isolated form. They are occasionally components of 3 clinical syndromes, familial paraganglioma and gastric GIST, neurofibromatosis type 1 and Carney triad. The latter does not show genetic mutations and affects young women predominantly. This article reports the occurrence of GIST and retroperitoneal paraganglioma in young Saudi woman aged 22 years. The initial clinical presentation was acute severe gastric bleeding. Gastric mass with retroperitoneal mass was discovered during surgical exploration. Histological and immunohistochemical study of both masses showed features of GIST and paraganglioma. The combination of GIST and retroperitoneal paraganglioma in the reported case may be incomplete expression of Carney triad or be coincidental occurrence. [ABSTRACT FROM AUTHOR]

Laparoscopic resection of a retroperitoneal paraganglioma is considered challenging because of variable anatomical presentation and potential vascular injury during surgical manipulation. Due to the rarity of the entity, only a few studies using this technique have been reported. Furthermore, a ruptured paraganglioma which manifests in traumatic retroperitoneal hemorrhage is exceedingly uncommon. Herein, we reported a case of a 12-year-old girl who presented with a ruptured retroperitoneal paraganglioma due to abdominal blunt trauma. Her contrast-enhanced computed tomography scan revealed a retroperitoneal hemorrhage and an abdominal mass of approximately 4 cm in diameter surrounded by the aorta, inferior vena cava, and left renal vein. A preoperative diagnosis of retroperitoneal paraganglioma was made from her 24 hour urinary catecholamine level, a clonidine loading test, and 123I-MIBG scintigraphy and elective laparoscopic resection was performed. Despite of dense peritumoral adhesion, a clear and magnified view of the surgical field and meticulous manipulation of the tumor allowed this laparoscopic resection to be successful, minimally invasive, and safe. [ABSTRACT FROM AUTHOR]