S3004 Giant Hepatic Hemangioma: Dulling the Blade of Ockham’s Razor

Abstract

Introduction: Ockham’s razor suggests the simplest diagnosis is likely correct. Hickman’s dictum says multiple diagnoses may occur in one patient. We present a patient with abdominal pain due to giant hepatic hemangioma, as well as secondary hypertension due to adrenal mass masked by giant hepatic hemangioma. Case Description/Methods: A 38-year-old female with abnormal uterine bleeding due to cervical ectropion developed right-sided abdominal pain and hypertension two years prior to presentation. She reported increased facial hair and acne which improved on spironolactone. Other medications included amlodipine, irbesartan, and progestin intrauterine device. Physical exam showed normal vital signs, facial hirsutism and acne, and abdominal distension with tenderness to palpation in the right upper and lower quadrants. Laboratory studies including serum adrenocorticotropic hormone, cortisol, aldosterone, and renin, and plasma and urine norepinephrine, epinephrine, and dopamine levels were unremarkable (Table 1). Magnetic resonance imaging with intravenous gadobutrol showed a 13.9 x 14.1 x 20.2 centimeter (cm) mass replacing the right hepatic lobe, compatible with hemangioma (Figure 1A), normal left adrenal gland, and patent renal vessels. The right adrenal gland was not visualized. She underwent right hepatic resection of hemangioma with enucleation (Fig. 1B). This allowed visualization and palpation of the right adrenal gland, revealing a 3 cm mass on the inferior portion (Fig. 1C). Right adrenalectomy was performed. Pathology revealed a well-demarcated vascular neoplasm consistent with liver hemangioma. Right adrenalectomy specimen revealed nodular cortical hyperplasia (Fig. 1D), correlating with aldosterone hypersecretion. Following surgery, she was normotensive without medication, and hirsutism resolved. Hepatic hemangioma alone is not known to cause hypertension. Her hypertension was attributed to adrenal hyperplasia, with potential elevation of serum aldosterone masked by spironolactone. Discussion: Our patient presented with concurrent onset of abdominal pain and hypertension, secondary to two distinct, unrelated entities – hepatic hemangioma and unilateral adrenal hyperplasia. This unique case of a giant hepatic hemangioma obscuring radiographic views of an adrenal mass reminds us that patients can have as many diseases as they please.Figure 1.: A, MRI of the abdomen with intravenous gadobutrol shows 13.9 x 14.1 x 20.2 cm hemangioma replacing the right lobe. B, Giant liver hemangioma specimen. C, Intraoperative photo of right adrenal mass. D, Adrenal cortical hyperplasia with enlarged lipid-rich cells (black open arrow) and lipid-depleted cells (black solid arrow. Hematoxylin and Eosin stain, magnification 200X. Table 1. - Laboratory Data Serologies Patient Values Reference Range White blood cell count (K/μL) 4.38 3.98-10.04 Hematocrit (%) 10.9 11.2-15.7 Hemoglobin (g/dL) 33.3 34.1-44.9 Platelet count (K/μL) 185 173-369 Sodium (mmol/L) 139 136-145 Potassium (mmol/L) 3.7 3.4-5.1 Chloride (mmol/L) 101 98-107 Total CO2 (mmol/L) 26 22-29 Urea nitrogen (mg/dL) 14 6-20 Creatinine (mg/dL) 0.60 0.51-0.95 Glucose (mg/dL) 88 74-99 Alkaline phosphatase (U/L) 66 35-105 Alanine aminotransferase (U/L) 19 0-33 Aspartate aminotransferase (U/L) 16 0-32 Bilirubin, total (mg/dL) 0.5 0.0-1.2 Bilirubin, direct (mg/dL) < 0.2 0.0-0.3 Activated partial thromboplastin time (sec) 31.2 25.3-37.3 Prothrombin time (sec) 14.9 11.6-15.2 Hemoglobin A1c (%) 5.6 4.0-6.0 Albumin (g/dL) 4.2 3.5-5.2 Calcium (mmol/L) 2.30 2.15-2.55 Magnesium (mmol/L) 0.81 0.66-1.07 Phosphorus (mg/dL) 3.1 2.5-4.5 Thyroid stimulating hormone (mcIU/mL) 1.24 0.27-4.2 Cortisol, serum 5.9 5.0-25.0 Follicle stimulating hormone (U/L) 3.5 < 21 Luteinizing hormone (U/L) 3.9 < 77 Estradiol, serum (pg/mL) 212.1 15-350 Dehydroepiandrosterone sulfate (mcg/mL) 0.85 0.35-4.30 Norepinephrine, plasma (pg/mL) 286 112-750 Epinephrine, plasma (pg/mL) 21 0-50 Dopamine, plasma (pg/mL) < 25 0-29 Fractionated normetanephrine (pg/mL) 70 18-112 Fractionated metanephrine (pg/mL) 14 12-61 Aldosterone, serum (ng/dL) 6.6 < 44.5 Renin, plasma (ng/mL/h) 0.8 0.6-4.3 Adrenocorticotropic hormone (pg/mL) 8.2 5.0-46.0 Alpha fetoprotein (ng/mL) 3.7 0.6-6.6 Sex hormone binding globulin (nmol/L) 100 18-114 Testosterone, total (ng/dL) < 20 < 81 Vitamin D, 1,25-dihydroxy (pg/mL) 64 20-79 Parathyroid hormone, intact (pg/mL) 48.1 15-65 Serotonin, whole blood (ng/mL) 87 < 330 Chromogranin A (ng/mL) 38 < 93 Human chorionic gonadotropin (IU/L) < 1 0-5 Epinephrine, urine (mcg/24h) 8.3 < 21 Norepinephrine, urine (mcg/24h) 61 15-80 Dopamine, urine (mcg/24h) 250 65-400