Paraganglioma is an uncommon type of neuroendocrine tumor capable of secreting neuropeptides and catecholamines. Retroperitoneal paragangliomas are rare neoplasms that originate from chromaffin cells that secrete catecholamines in the sympathetic ganglia. Tumor reduction and management of excessive catecholamine secretion are the key treatment goals. Surgery is the choice of treatment modality when tumors are amenable to resection because of their malignant potential. Currently, high-dose metaiodobenzylguanidine (MIBG) radiation and chemotherapy are adjuvant therapy to surgery. This case is reported to demonstrate a good prognosis in a conservatively managed, huge, non-functional retroperitoneal paraganglioma for 14 years. This provides alternative options to the traditional surgical treatment in patients who refuse, are unfit for surgery, or have complex surgery, which carries a high mortality rate with analysis of follow-up modalities. Meanwhile, a review of the relevant literature was conducted in order to figure out the prognosis.
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http://dx.doi.org/10.7759/cureus.21072 | DOI Listing |
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 PDFUrol 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.
View Article and Find Full Text PDFJ Coll Physicians Surg Pak
October 2024
Department of Urology, The Affiliated Hospital of Hebei University, Heibei, China.
BMJ Case Rep
September 2024
Histopathology, PGIMER, Chandigarh, India.
Retroperitoneal neuroendocrine tumours are exceptionally rare. The excision of tumours located in the renal hilum near the renal vessels can be challenging. We report a case of a paraganglioma located at the renal hilum which was excised successfully in a child who presented with abdominal pain, breathlessness, left varicocele and hypertension.
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