THREE CASES OF CATECHOLAMINESECRETING NEUROENDOCRINE TUMOR IN A TERTIARY HOSPITAL, WEST JAVA, INDONESIA

Abstract

CASE
Pheochromocytomas or paragangliomas (PPGL) are rare neuroendocrine chromaffin-derived tumors. The annual incidence of PPGL is approximately 0.66 per 100,000 person-years. Approximately 50% of patients present with paroxysmal hypertension, 15-25% have the classic triad, while 5–15% are asymptomatic. Patients with a high degree of catecholamine excess may present with PPGL crisis with target organ complications.

Three cases of catecholamine-secreting neuroendocrine tumors (NET) were entertained in our institution. Patient I presented with a nonclassical triad, and patient II presented with a classic triad. Patient III was diagnosed as having a pheochromocytoma crisis, manifesting as recurrent myocarditis and cardiogenic shock. The patients were diagnosed as PPGL based on elevated metanephrine, adrenal CT scan, and Iodine-131-metaiodobenzylguanidine scintigraphy (I131-MIBG) positivity. Two patients underwent tumor resection after being perioperatively stable for 14 days, and one patient was lost to follow-up. The final diagnosis for patient I was progressive malignant paraganglioma, Patient II had pheochromocytoma. The patients were followed up and showed no residual symptoms after 1 month.