Paraganglioma is a rare neoplasm originating from extra-adrenal pheochromocytes of the sympathetic and parasympathetic nervous system. It is usually benign and the treatment method of choice is a complete resection of the tumour. The authors present a case of 66-year-old female patient with a multifocal benign retroperitoneal paraganglioma, which was completely removed during surgery.
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Recent progress in the pathologic classification of pheochromocytomas and paragangliomas.
Best Pract Res Clin Endocrinol Metab
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Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden; Department of Pathology and Cancer Diagnostics, Karolinska University Hospital, Stockholm, Sweden.
Article Synopsis
- * These tumors have a potential risk of metastasis and the 2022 WHO classification emphasizes combining morphology with functional and genetic studies for more accurate diagnosis and prognosis.
- * This review aims to update pathologists on modern diagnostic strategies, focusing on biomarkers and genetic profiling, while addressing challenges in identifying metastatic disease.
Hormonally Silent Multifocal Pheochromocytoma in the Setting of a Well-Differentiated Neuroendocrine Tumor of the Appendix: A Case Report.
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Surgical Oncology, Saint Luke's Hospital, Kansas City, USA.
Pheochromocytomas (PCCs) and paragangliomas (PGLs) represent tumors arising from chromaffin cells of the adrenal medulla and extra-adrenal sympathetic paraganglia, respectively. PCCs commonly produce one or more catecholamines (epinephrine, norepinephrine, and dopamine), but rarely are they biochemically silent. PGLs on the other hand, generally do not produce catecholamines.
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Standard methods of variant assessment in hereditary cancer susceptibility genes are limited by the lack of availability of key supporting evidence. In cancer, information derived from tumors can serve as a useful source in delineating the tumor behavior and the role of germline variants in tumor progression. We have previously demonstrated the value of integrating tumor and germline findings to comprehensively assess germline variants in hereditary cancer syndromes.
View Article and Find Full Text PDF[Glomuvenous malformation: a clinicopathological analysis of 31 cases].
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