MON-385 Malignant Pheochromocytoma/Paraganglioma Syndrome during Pregnancy: Clinical Case Report, a Therapeutic Challenge

Abstract

Background: Arterial hypertension due to endocrine disease is 3% of secondary causes, being more frequent in the young. Excess of mineralocorticoids, catecholamines and glucocorticoids is potentially curable with the appropriate treatment. Pheochromocytoma/Paraganglioma syndrome is 0.2 to 0.6% of the cause. Clinical Case: 27 year old woman with previously known left pheochromocytoma, treated with laparoscopic adrenalectomy in 2010, with biochemical tests, ruling out new lesions and being able to stop antihypertensive treatment. In 2017, a twenty week old pregnancy was stopped due to elevated arterial pressure. The patient consults in December 2017 with a 15th week old pregnancy, complaining of catecholaminergic symptoms and a hypertensive crisis in the emergency range. Initial treatment was labetalol with partial control of symptoms. Normetanephrines in plasma were elevated (2298 pg/mL, NR: < 180pg/mL). During follow up additional antihypertensive management was required with prazosin, clonidine and nifedipine achieving adequate control of hypertension, without complete control of symptoms. A Non contrasted MRI of the abdomen was performed where a retroperitoneal ganglionar conglomerate of 96mm around the aorta was documented. Additional finding were a cervical adenopathy in a computed tomography (CT) and a lytic bone in T7 and right ribcage, pulmonary nodule of 12mm in the lingula without other lesions in lung. Bone scintigraphy confirmed bone lesions, which made the diagnosis of malignant pheochromocytoma paranglioma syndrome (PPS). Cytoreductive surgery followed the management, for biochemical, clinical control and overall increase in survival. During the surgery dissection of the abdominal mass from the aorta to the iliac artery bifurcation, left nephrectomy and splenectomy were required. Obstetric ultrasonography found fetal suffering which led to an obstetric curettage. Pathology reports intermediate cells in an organoid pattern compatible with neuroendocrine neoplasia. Inmunohistochemistry with positivity for chromogranin, synaptophysin, inhibin and S100. AE1/AE3 showed focal positivity in neoplasic cells. Calretinin and MELAN-A were negative. These results confirmed the diagnostic suspicion of paraganglioma. Knowing the autosomal dominant inheritance pattern and variable expression, in the context of malignant PPS genetic studies were performed for the succinate dehydrogenase B and D, von hippler lindau and fumarate hidratase. Clinical evolution was satisfactory with decrease in the number and dosage of antihypertensives, control of arterial hypertension and a reduction in catecholaminergic symptoms. Conclusion: This is the first case of malignant PPS during pregnancy. Clinical control of the disease was obtained, with decrease in antihypertensive and control of symptoms. Unfortunately with a fetal loss during the treatment of the patient