Category: Other - Neuroendocrinology
Objective : Paragangliomas are rare neoplasms; the combined coincidence with pheochromocytmas is about 0.8 for every 100,000 person years. This would imply that the incidence for paragangliomas is much less, but may also imply that many paragangliomas are actually undiagnosed. Here we present a patient with a paraganglioma which, despite the patient having classic symptomatology, was undiagnosed until incidentally when the patient underwent a left nephrectomy to remove what was thought to be renal cell carcinoma.
Methods : N/A
This is a 65 year old male with a PMH of uncontrolled HTN, headaches, and anxiety, who was seen by his primary doctor for an initial encounter; he complained of having more anxiety associated with sweating and palpitations. His blood pressure at that time was also noted to be 152/88 (heart rate of 79, respiratory rate of 16, and temperature of 98F), and he was started metoprolol in addition to his amlodipine and benazepril that he had already been taking. Despite the visit, he continued to have increased blood pressures ranging in the 190’s systolic for several days, and is repeat blood pressure in the office was 160/80 (heart rate 69, resp 14, and temperature 98.1F). His creatinine was also elevated. A CT abdomen was ordered which revealed a left upper pole renal cyst; MRI abdomen showed 11.9 cm complex cyst with enhancing peripheral wall thickening with internal nodular component, suggestive of malignancy. He underwent an open nephrectomy. The gross specimen was shown to be adherent to the kidney and the adrenal gland, however the surgical pathology showed no involvement in any of the slides. In addition, the neoplastic cells stained strongly positive for chromogranin A and synaptophysin. Further tests showed lack of expression for epithelial markers for renal or adrenal origin. The tumor was believed to be a paraganglioma from an extra-adrenal site with adhesions to the kidney and adrenal gland. Urine metanephrines measured a month after the surgery was 687 mcg/24hrs (they were not measured preoperatively or immediately postoperatively until the results of the pathology were made known). After the surgery, his symptoms resolved; his antihypertensive medications were weaned and his blood pressures remained stable.
Discussion : There are approximately 500 to 1600 cases of paragangliomas/pheochromocytomas diagnosed each year, but studies involving autopsies revealed a greater prevalence, suggesting that many tumors go undiagnosed.
Conclusion : This case illustrates how paragangliomas can remain hidden and highlights the need for better diagnostic studies to further identify paragangliomas early.
Overlook Medical Center, New Jersey
Born in Somerset, NJ. Attended New Jersey Institute of Technology for undergraduate studies. Attended St. George's University for Medical School. Currently a PGY-2 internal medicine resident at Overlook Medical Center.
Summit, New Jersey
Graduated from Ss. Cyril and Methodus University Medical School in Macedonia. Completed internal medicine residency program at Overlook Hospital. Served as chief resident for the residency program in academic to 2017-2018. Currently serving as one of the teaching hospitalists at Overlook hospital.