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Pediatric Retroperitoneal Paraganglioma Invading the Inferior Vena Cava: A Case Report and Literature Review.
J Nippon Med Sch
January 2025
Department of Surgery, Nippon Medical School.
Paraganglioma, a catecholamine-producing tumor originating in extra-adrenal paraganglion cells, is rare in children. Although diagnosis of paraganglioma is based on the presence of catecholamine symptoms, some patients lack such symptoms. Delayed diagnosis is associated with tumor growth and invasion of surrounding vessels.
View Article and Find Full Text PDFDurable and deep response to CVD chemotherapy in SDHB-mutated metastatic paraganglioma: case report.
Front Endocrinol (Lausanne)
January 2025
Division of Abdominal Tumor, Department of Medical Oncology, Cancer Center and State Key Laboratory of Biological Therapy, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Introduction: Succinate dehydrogenase subunit B (SDHB)-mutated paragangliomas (PGLs) are rare neuroendocrine tumors characterized by increased malignancy, readily metastasizing, and poorer prognosis. Here we report a case of SDHB-mutated metastatic PGL, wherein the patient showed significant tumor shrinkage and complete symptom remission following chemotherapy. We aim to contribute additional evidence to the existing knowledge associated with SDHB-mutated PGLs.
View Article and Find Full Text PDFCase report: Paraganglioma masquerading as angiosarcoma: diagnostic-dilemma in vascular tumors.
Front Oncol
December 2024
Vascular Unit, Department of Surgery, Universiti Kebangsaan Malaysia, Cheras, Malaysia.
Paragangliomas originating from blood vessels are exceptionally rare, presenting diagnostic challenges due to their histological resemblance to other vascular neoplasms. We present a case study of a 60-year-old woman with underlying hypertension and dyslipidemia with obesity, initially diagnosed with angiosarcoma based on imaging and histological characteristics viewed via CT-guided biopsy. Intraoperative exploration revealed a lobulated tumor located between the inferior vena cava (IVC) and aorta measuring 7 cm × 8 cm, during which the patient developed transient hemodynamic instabilities.
View Article and Find Full Text PDFMultidisciplinary management of chylous ascites following resection of a retroperitoneal, functional Paraganglioma.
Urol Case Rep
November 2024
Department of Urology, Emory University School of Medicine, Atlanta, GA, USA.
Extra-adrenal, functional paraganglioma, if not medically controlled, requires early surgical resection. This can often be challenging when found in a retroperitoneal location, where resections are prone to lymphatic disruption and chyle leaks. Chylous ascites carry a significant risk of protein and caloric malnutrition, dehydration, and even mortality given the nutrient-rich characteristic of lost lymphatic fluid.
View Article and Find Full Text PDFPheochromocytoma and paraganglioma are often associated with hereditary syndromes, particularly those involving genes such as , 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.
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