Vasoactive intestinal peptide producing pheochromocytoma and intracardiac thrombosis

Abstract

A case of pheochromocytoma producing vasoactive intestinal peptide (VIP) and left ventricular thrombus in the absence of cardiomyopathy or wall motion abnormalities on echocardiogram is presented along with a review of the relevant literature. A 30-year-old female of Afghani descent with past medical history of panic attacks presented with fever, cough, sore throat, vomiting, and was found to have an 11 cm adrenal mass consistent with primary adrenocortical adenoma versus carcinoma. Her tumor elicited catechols and vasoactive intestinal peptide. Her hospitalization was complicated by left ventricular thrombosis leading to an embolic injury to her right kidney, respiratory failure, need for transient dialysis and urinary tract infections. She developed a profuse secretory diarrhea and decision was made to treat with empiric octreotide infusion and imodium with improvement in symptoms. She underwent coil and particle embolization followed by resection. Followup PET gallium scan showed no evidence of residual disease or metastasis. VIP producing pheochromocytoma associated with intracardiac thrombosis is rare. Outcomes depend on prompt diagnosis of the pheochromocytoma and multidisciplinary approach to management.

Vasoactive Intestinal Peptide-Secreting Pheochromocytoma: A Case Report and Review of Literature

“…It is possible that the vasodilatory effects of VIP “masked” the hypertensive effects of catecholamines, potentially delaying diagnosis. There were no reports of associations with other endocrinopathies, and only 1 case (in the study by Hermel et al 5 ) reported a family history positive for an aunt with thyroid disease. Genetic testing was reported in 4 cases (in the studies by Negro et al 6 , Jiang et al 8 , Leibowitz-Amit et al 9 , and Ozbay et al 11 ), of which only 1 (in the study by Negro et al 6 ) reported a “synonymous single nucleotide variant of the SDHA gene” associated with “probably benign pheochromocytoma.” In localized disease, surgical resection is generally definitive with good prognosis.…”

“…The rare case of a VIP-secreting pheochromocytoma was first reported in 1975 by Loehry et al 4 , and since this time, only approximately 25 cases have been described. 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 We describe a case of a man found to have a pheochromocytoma who presented with hypertensive emergency and WDHA syndrome, resolved with adrenalectomy.…”