A shining heart at Valentine's Day

A 60-year-old men was admitted mid-January for acute left abdominal pain. He had no medical history and didn’t smoke or take any medication. Abdominal CT-scan showed a voluminous (61 × 57 × 47 mm), heterogeneous, hemorrhagic mass in left adrenal gland with a high spontaneous density (45UH). Pheochromocytoma diagnosis was first suspected although 24-hours urine fractionated metanephrines and catecholamines measurements were only slightly increased (NA:1.1xULN and NMN:1.2xULN). Hormonal evaluation did not demonstrate excessive cortisol or sexual steroids secretion. 18F-FDG PET-CT imaging revealed intense FDG-uptake in the left adrenal gland as well as in the right adrenal gland and in the heart. With this new information, the differential diagnosis includes carcinoid cardiac tumor, malignant cardiac myxoma, sarcoma and metastases. All others tumor markers were negative (5HIAA, NSE, CA 19-9, CA 125, CEA, PSA, β-hCG). For Valentine's Day, cardiac MRI confirmed a pathological thickening of right inter-atrioventricular groove with marked late enhancement at this level, corresponding to pathological areas in isotopic imaging. Next day, patient presented acute right flank pain similar to the one he had on left side one month earlier. New abdominal CT-scan showed a large hemorrhage in right adrenal gland that was not present on 18F-FDG PET-CT performed 3 days before. Latest Ga-DOTATATE-PET imaging didn’t show anywhere STT-receptors presence. Morning cortisol level (411 nmol/L) remained non-suggestive of adrenal insufficiency. This case was discussed by web-conference with the French experts of the national COMETE multidisciplinary committee: it was decided to obtain histology from left adrenal tumor by percutaneous biopsy rather than by unilateral adrenalectomy because the radicality of surgery might change depending on histological diagnosis. So far, diagnosis has not yet been established. This case underlines importance of discussing complex situations with experts as allowed by COMETE or ENDO-ERN networks.