Abstract 17116: Recurrent Atrial Myxomas As A Presenting Feature Of Carney Complex

Abstract

Case Presentation: A 24-year-old man with a history of adrenal insufficiency on hydrocortisone presented to the hospital after losing consciousness in a swimming pool and was found to have an acute MCA stroke. PMHx was significant for left atrial myxoma status post resection 3 years ago, known L frontal brain, L adrenal, and testicular masses status post-resection. His father had a history of resected left atrial mass. Cardiology was consulted for a L atrial mass on the bedside echocardiogram upon initial emergency evaluation, during which the patient was placed on mechanical ventilation with stable vitals. The cardiovascular exam revealed a midsternal scar but was otherwise normal. Pulses were 2+ bilaterally on the upper and lower extremities. The skin was noted to have multiple hyperpigmented macules resembling cafe-au lait spots. The patient underwent mechanical thrombectomy with thrombolysis for his large MCA occlusion. His postoperative course was complicated by a worsening neuro exam in the setting of mid-line shift for which he had undergone decompressive hemicraniectomy. His adrenal insufficiency was managed by endocrinology. A TTE was significant for a L atrial mass measuring 4.8 cm attached to the interatrial septum with a stalk. A CT scan identified 2 discrete hypodensities of 8 mm and 3.8 cm which were thought to be the cardioembolic source for the stroke. An abdominal CT revealed a recurrent L adrenal neoplasm with absent R adrenal gland post resection of neoplasm. Conservative cardiac management was elected in the setting of a worsening neuroprognosis with follow-up post neurologic insult. Discussion: Considering the constellation of neoplastic and cutaneous findings, this was diagnosed as a case of Carney complex. This case identifies recurrent myxoma as a unique feature of Carney complex, ruling out other differentials such as neurofibromatosis and MEN syndrome. It highlights the importance of screening to prevent embolic stroke from recurrent myxomas.