Adrenal Disorders
fnu marium, MD (she/her/hers)
endocrinology fellow
University of Missouri
columbia, Missouri, United States
A dopamine-secreting pheochromocytomas and paragangliomas (PPGL) is a very rare subtype that predominantly or exclusively secretes dopamine but not norepinephrine or epinephrine. It has been characterized by a lack of catecholamine excess symptoms, large tumor size, extra-adrenal location, and high malignant potential. We report the case of 46 yo patients with an organ of Zuckerkandl paraganglioma presented with acute excruciating abdominal pain. He was normotensive before the event and had no symptoms of catecholamine excess.
Case(s) Description :
46 years old male presented to hospital for acute onset of abdominal pain developed while playing golf. Imagine studies came back with a 1.4 cm tumor in the right retroperitoneal. On further evaluation he was found to have normal levels of metanephrines and normetanephrines (29 (15-100)mcg/24h, 29 (26-121) mcg/24h respectively). Dopamine was 1544 (52-480) mcg/24h. Dotatate scan came back with 1.8 x1.5 cm retroperitoneal nodule with avidly radiopharmaceutical uptake. Location and appearance suggestive of paraganglioma at the organ of Zuckerkandle. The patient was admitted to hospital for surgical treatment. Surgery was successful. Pathological and genetic evaluation is pending.
Discussion :
Organs of Zuckerkandl (O of Z) harbor the potential for deadly paragangliomas. Paragangliomas are one of the surgical causes of hypertension. Major treatises of medicine offer very little information on this topic. Dopamine secreting paragangliomas are more likely to present malignant features than classic catecholamine secreting type paragangliomas or pheochromocytomas. This rare tumor tends to be diagnosed in patients who are asymptomatic, and the tumor is detected later than tumors that secrete norepinephrine, epinephrine or both. Delayed diagnosis may be related to the higher incidence of malignant transformation and metastasis.
To minimize surgical mortality caused by hypertensive crisis during the manipulation of the pheochromocytoma or paraganglioma, preoperative administration of an α-blocker is essential for norepinephrine and epinephrine producing tumors. However, α-blocker treatment is contraindicated for exclusively dopamine secreting paraganglioma because it can cause profound cardiovascular collapse after surgery, and can even result in death following a hypotensive crisis.