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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. [ABSTRACT FROM AUTHOR]

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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: 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. [ABSTRACT FROM AUTHOR]