Search

Abstract Background Pheochromocytomas and Paragangliomas (PCC/PGL) are rare endocrine tumors that are mostly benign, but often hormone producing, causing significant morbidity and mortality due to excess catecholamine secretion and cardiovascular crises. It is estimated that 30% of PCC/PGL are due to germline mutations, including Neurofibromatosis type 1 (NF1). There is little published data describing the phenotype of NF1-associated PCC/PGL and there are no established recommendations for PCC/PGL screening in NF1. Methods We conducted a retrospective chart review of 17 patients with NF1-associated PCC/PGL who received care at a large academic referral center between the years of 1992–2016. Results Average age of diagnosis was 42 years old. Both genders were equally affected. Average tumor size was 3.9 cm. Nine patients were hypertensive; one had orthostatic hypotension; three had tachycardia; the remaining two patients had normal BP and HR. Most tumors were benign, unilateral adrenal tumors that were hormonally active. Two had metastatic disease. Six patients experienced cardiovascular crises; three of which occurred during elective surgeries for neurofibroma removal, and a fourth occurred during labor and delivery. Conclusion These data highlight the importance of screening for PCC/PGL in NF1, especially prior to surgical procedures and pregnancy, labor and delivery as these events can trigger a cardiovascular crisis. Screening is easily accomplished with plasma or urine free fractionated metanephrine levels.

The infiltration of dilute epinephrine solution has been used for many years to provide hemostasis. However, epinephrine has adverse cardiovascular effects, such as arrhythmia, pulmonary edema, and even cardiac arrest. We have experienced epinephrine-induced cardiovascular crisis, with severe hypertension, tachycardia, and cardiac arrest after subcutaneous infiltration of a 2% lidocaine and 1 : 200,000 epinephrine solution in a patient with an asymptomatic subarachnoid hemorrhage. We provided successfully advanced cardiac life support in the operating room and cardioverted the patient back into a sinus rhythm with no untoward effects. The patient recovered without any apparent sequelae after intensive care.

The infiltration of dilute epinephrine solution has been used for many years to provide hemostasis. However, epinephrine has adverse cardiovascular effects, such as arrhythmia, pulmonary edema, and even cardiac arrest. We have experienced epinephrine-induced cardiovascular crisis, with severe hypertension, tachycardia, and cardiac arrest after subcutaneous infiltration of a 2% lidocaine and 1 : 200,000 epinephrine solution in a patient with an asymptomatic subarachnoid hemorrhage. We provided successfully advanced cardiac life support in the operating room and cardioverted the patient back into a sinus rhythm with no untoward effects. The patient recovered without any apparent sequelae after intensive care.