Functional vagal paraganglioma: a case report illustrating diagnosis and management.
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ABSTRACT:
SUBMITTER: Hornbeek H
PROVIDER: S-EPMC3134809 | biostudies-other | 2010 Nov
REPOSITORIES: biostudies-other
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Project description:IntroductionRetroperitoneal paragangliomas are rare tumors, they arise from ganglia along the sympathetic and parasympathetic chain. We report a rare case of a non functional paraganglioma in whom surgical resection was performed.Presentation of caseA 35 years-old female presented with chronic abdominal pain, A contrast magnetic resonance imaging (MRI) of abdomen showed a well-defined Left latero-aortic cystic retro-peritoneal surgical resection using laparotomy was performed, The patiente recovered well and was discharged three days after surgery. Histological examination and immunohistochemical revealed a retroperitoneal paraganglioma.Discussion and conclusionNon-functioning retroperitoneal paragangliomas are rare and are most often Isolated. Radiological techniques including, Contrast-enhanced computed tomography (CT) and Magnetic resonance imaging (MRI) are useful for identifying and locating retroperitoneal paragangliomas. surgical excision is still the most effective treatment when it possible.
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Project description:Study design Case Report. Introduction and importance To report a case of a paraganglioma presenting in an uncommon location in the abdomen. Case presentation A 24-year-old man with an abdominal lesion presented with one-year history of severe headaches and palpitations. Interventions and outcome The tumor was surgically resected and was later diagnosed as an extra-adrenal paraganglioma. Conclusion The unique location of a paraganglioma could prove misleading, making it easier to confuse it with other malignant lesions, but it still can't be excluded, and with proper techniques it can be surgically excised. Highlights • Paragangliomas (PGLs) are slow-growing tumors of neuroendocrine origin.• PGLs in the portocaval region are considered extremely rare.• Their unusual location has its own implications that must be carefully considered.• Clinical findings with imaging techniques and laboratory tests are key to diagnosis.• Surgical resection remains the gold standard of treatment.
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Project description:BackgroundParagangliomas, also known as chemodectomas, are rare tumors arise from chemoreceptor tissue, and most commonly locate at the bifurcation of the common carotid, the jugular foramen, aortic arch, and retroperitoneum. Paragangliomas generally are considered to be benign tumors, and rarely produce local or distant metastases. Metastasis to liver is extremely rare.Case presentationWe report the case of a 39-year-old woman, who had undergone resection of a retroperitoneal paraganglioma at her local hospital for 12 years. She was referred to our hospital for further evaluation of a hepatic mass, which was misdignosed as hepatocellular carcinoma (HCC) and was treated by transarterial chemoembolization (TACE) in the local hospital 6 years ago. At admission, CT scan revealed a huge hypervascular mass with many feeding arteries, almost the same size as 5 years ago. Ultrasound-guided biopsy of the liver tumor was performed and immunohistochemical examination confirmed the diagnosis of hepatic metastatic paraganglioma. Though liver metastasis failed to achieve complete response or partial response to TACE treatment, it remained stable without progression during the 7-year follow-up.ConclusionParagangliomas are slow growing tumors and metastasis may develop decades after resection of the primary lesion. Long-term follow-up is necessary, and curative or palliative treatment should be considered to control symptoms, improve life quality, reduce complications and prolong survival.
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Project description:Background:Mediastinal paragangliomas (PGs) are rare and particularly challenging neuroendocrine tumours. Clinical presentation is heterogeneous and tumour resection can be challenging due to bleeding and the risk of catecholamine surges in functional tumours. Case Summary:A 36-year-old man with multiple cardiovascular risk factors was admitted with subacute heart failure. Investigations revealed a large non-metastatic functional mediastinal PG irrigated mainly by a left circumflex coronary anomalous feeder branch. The surgical risk was deemed very high due to patient comorbidities, tumour vascularization, and close relation to major thoracic structures. A multidisciplinary team decided to perform embolization of the anomalous coronary branch followed by peptide-receptor radionuclide therapy with 177-LuDOTATE aiming to decrease tumour size and perioperative risk. Follow-up studies showed a reduction in tumour vascularization, size, and hormonal production. Discussion:The innovative strategy of combining embolization of the anomalous feeder branch with radionuclide therapy proved to be a promising approach.
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Project description:Accurate diagnoses are crucial in determining the most effective treatment across different cancers. In challenging cases, morphology-based traditional pathology methods have important limitations, while molecular profiling can provide valuable information to guide clinical decisions. We present a 35-year female with lung cancer with choriocarcinoma features. Her disease involved the right lower lung, brain, and thoracic lymph nodes. The pathology from brain metastasis was reported as “metastatic choriocarcinoma” (a germ cell tumor) by local pathologists. She initiated carboplatin and etoposide, a regimen for choriocarcinoma. Subsequently, her case was assessed by pathologists from an academic cancer center, who gave the diagnosis of “adenocarcinoma with aberrant expression of β-hCG” and finally pathologists at our hospital, who gave the diagnosis of “poorly differentiated carcinoma with choriocarcinoma features”. Genomic profiling detected a KRAS G13R mutation and transcriptomics profiling was suggestive of lung origin. The patient was treated with carboplatin/paclitaxel/ipilimumab/nivolumab followed by consolidation radiation therapy. She had no evidence of progression to date, 13 months after the initial presentation. The molecular profiling could facilitate diagnosing of challenging cancer cases. In addition, chemoimmunotherapy and local consolidation radiation therapy may provide promising therapeutic options for patients with lung cancer exhibiting choriocarcinoma features.
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Project description:The coexistence of thyroid functioning nodules and Graves' disease is called Marine-Lenhart syndrome. This condition is estimated to occur in 0.8-2.7% of patients with Graves' disease with few cases reported in the literature. Criteria for the diagnosis are not well defined. Here, we present a case of hyperthyroidism characterized by the presence of stimulating TSH receptor antibodies and severe bilateral exophthalmos. A thyroid uptake and scan revealed an elevated 24-hour iodine-131 uptake and a discrete hot nodule in the upper pole of the right lobe which was also observed with a thyroid ultrasound. The patient was diagnosed with Marine-Lenhart syndrome complicated by thyroid eye disease and was treated with methimazole followed by thyroidectomy and orbital decompression. We review the existing literature and propose criteria for the diagnosis and treatment of this condition.
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Project description:INTRODUCTION: Paragangliomas are neuroendocrine tumors that most commonly originate in the adrenal gland, a type that is called pheochromocytoma; however, 5-10% of paragangliomas are extra-adrenal and may arise in any area between the neck and pelvic region along the sympathetic nervous system. Those located in the head and neck comprise 3% of extra-adrenal tumors, with the majority originating in the tympanic-jugular region and carotid body. OBJECTIVE: To present a rare case of nasal paraganglioma and review the literature. CASE REPORT: The patient was submitted to medial subtotal maxillectomy, and her clinical findings, diagnostic data, and treatment outcome were recorded. CONCLUSION: Paragangliomas are considered benign tumors, but they occasionally display a malignant character. The most important finding in this case was the need for total resection of the tumor to avoid recurrence.
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Project description:Cardiac paragangliomas (PGLs) are rare extra-adrenal tumors that arise from chromaffin cells of the sympathetic ganglia. PGLs are often diagnosed incidentally, in the absence of symptoms, or with symptoms related to cardiovascular dysfunction. Cardiac computed tomography (CCT) and cardiac magnetic resonance (CMR) can be used to accurately determine the lesion morphology and position as well as providing detailed tissue characterization. A multimodal imaging approach, not yet standardized, could be useful either in diagnosis and monitoring or in treatment planning. In the case reported here, CCT and CMR were performed to define lesion anatomy, and a reconstruction was generated using cinematic rendering (CR) to characterize the PGL angioarchitecture.
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Project description:Background:Absence of the pericardium is a rare congenital defect with an approximate incidence of <1/10 000. We review a case of complete pericardial agenesis in a symptomatic patient with gross cardiac mobility, for which pericardial reconstruction was undertaken successfully. Case summary:A 24-year-old otherwise fit and well patient, with debilitating exertional chest pain was found to have complete pericardial agenesis on the left side and on the diaphragmatic surface. There was gross cardiac mobility demonstrated on cardiac magnetic resonance imaging. His pericardium was reconstructed surgically using Gore-tex® patches. There were no complications, and the patient was discharged 8?days later. Three months later at follow-up, the patient required no analgesia and has had complete resolution of his chest pains. Discussion:Congenital hemi-pericardial agenesis is a very rare condition which often remains undetected due to its asymptomatic nature. It is important to consider this as a differential diagnosis of exertional chest pains. Cardiac magnetic resonance imaging remains the investigation of gold standard. There is no consensus on whether surgical intervention in symptomatic or asymptomatic patients has any prognostic value. However, we have demonstrated that by reconstructing the pericardium in a highly symptomatic patient, there has been a resolution in size of a previously dilated right ventricle and most importantly an improvement in quality of life.
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Project description:Paragangliomas are rare neuroendocrine tumors with 500 to 1600 new cases in the United States each year (1). The clinical presentation may range from asymptomatic to the classic triad of episodic diaphoresis, headache, and palpitations. Surgery is the hallmark of treatment when tumors are amenable to resection. When patients are found to have metastases, systemic therapies may be employed. In this case report, we present a patient found to have a large retroperitoneal paraganglioma with nodal metastases.
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