Surgical management of pheochromocytoma in a 13-week pregnant woman

Abstract

SUMMARYA 34-year-old 13-week pregnant woman presented with hypertension refractory to medical therapy and on workup was found to have a right adrenal mass. Due to her persistent increased blood pressure she was advised urinary vanillylmandelic acid (VMA) and its level was raised. MRI of the abdomen showed a well-circumcised lesion in the right adrenal of 3.0×2.5 cm suggestive of pheochromocytoma. The patient was started on antihypertensives including α-blockers and β-blockers and planned for right open adrenalectomy. Intraoperatively, blood pressure was raised up to 180/110 mm Hg on slight manipulation of adrenal gland which was controlled with glyceryl-trinitrate and volatile agents. Postoperatively urinary VMA decreased to normal range and all antihypertensives were gradually stopped. She had uneventful pregnancy and delivered vaginally. This case report highlights the importance of surgical management of pheochromocytoma in second trimester of pregnancy to avoid catastrophic complications later in pregnancy. BACKGROUND

Laparoscopic approach to pheochromocytoma in pregnancy: case report

“…PCC in pregnancy can cause fatal hypertensive crisis that can be triggered by vaginal delivery, general anesthesia, the physical effects of the enlarging uterus, uterine contractions, or fetal movements ( 2 ), and may impose a serious risk to the fetus because extreme vasoconstriction in the uteroplacental circulation may result in intrauterine hypoxia and premature placental abruption ( 5 ). Undiagnosed and/or untreated PCC carry a risk of mortality for both mother and fetus as high as 58% ( 4 , 6 ).…”

“…Symptoms may occur for the first time in pregnancy due to increased vascularity of the tumor and/or mechanical factors such as pressure from the enlarging uterus or fetal movements, which can stimulate catecholamine secretion ( 7 , 8 ). These signs and symptoms include hypertension (98% of cases), orthostatic hypotension, palpitations, headaches, sweating, anxiety attacks, facial flushing, and chest pain ( 5 , 6 ). However, in pregnant women, the disease can be mistaken for other causes of hypertension, especially specific hypertensive disorders of pregnancy such as pre-eclampsia ( 3 ).…”

“…A collaborative multidisciplinary team, including surgeons, anesthesiologist, obstetrician, and endocrinologist, is decisive for case management ( 10 ). Surgical resection of the tumor is the definitive treatment for PCC, which can be achieved either by open, laparoscopic, or robotic approaches ( 1 , 6 , 8 ). Laparoscopic adrenalectomy is the surgical approach of first choice if tumor size is <7 cm ( 4 , 10 , 11 ); this is a safe procedure with a complication rate <8% ( 5 , 9 ).…”

“…The diagnosis can be established by detecting elevated levels of catecholamines and their metabolites in plasma or urine. Ultrasound and magnetic resonance imaging (MRI) are the preferred imaging modalities for tumor localization during pregnancy ( 6 ). Surgery is the definitive treatment for PCC after management of hypertension with α blockade, but the timing of tumor removal depends on the gestational age, the severity of maternal symptoms, and the risks associated with pregnancy termination ( 4 ).…”

Laparoscopic management of paraganglioma in a pregnant woman: a case report

“…Pregnancy does not change plasma or urine level of metanephrines (normetanephrine and metanephrine). Measurement of metanephrines, either in blood or in urine, is the preferred test to rule out or to confirm the diagnosis of pheochromocytoma and paraganglioma ( 7 ).…”