A 27-year-old woman presented with an epigastric mass, accompanied by emesis and weight loss. An abdominal computed tomography (CT) scan showed a pancreatic head-body mass and liver metastasis. Biopsy revealed well-differentiated pancreatic neuroendocrine tumor, grade 3. Chromogranin and synaptophysin stains were positive, with a Ki-67 index of 33.6%. Increased frequency of episodic emesis with incomprehensible speech and right-sided weakness prompted admission. A cranial magnetic resonance imaging (MRI) scan showed a subacute cerebrovascular infarct. Serum glucagon was markedly elevated. On the third hospital day, neurologic symptoms progressed, with repeat cranial MRI demonstrating acute lacunar infarcts. Two-dimensional echocardiography was consistent with dilated cardiomyopathy. Because of her fragile condition, she was not a candidate for surgery and chemotherapy. She was treated with long-acting octreotide every 4 weeks for a total of 10 doses. Posttreatment, a 2-dimensional echocardiogram and an abdominal CT scan showed no significant change. We report a rare case of glucagonoma with associated prominently dilated cardiomyopathy and ischemic stroke.

Background Dilated cardiomyopathy has been reported in patients with glucagonoma. Since glucagon increases myocardial cAMP levels independent of beta adrenoreceptor blockade, thereby increasing heart rate and cardiac output, chronic excessive glucagon stimulation of the heart may lead to cardiomyopathy (Chang-Chretien, 2004). Clinical Case A 26 year old female presented with a 2-year history of palpable epigastric mass, vomiting and weight loss. Abdominal CT showed a heterogeneously enhancing, lobulated pancreatic head-body mass measuring 6.7×6.1×9.9 cm and a hypo-enhancing nodule in hepatic segment IVb, worrisome for metastasis. Endoscopy-guided FNAB of the pancreatic mass revealed a well-differentiated pNET, Grade 3. Chromogranin and synaptophysin stains were positive, Ki-67 index was 33.6%. She was lost to follow-up. After 4 months, she was admitted due to 1 week history of increased vomiting episodes up to 10x daily and abdominal discomfort with 2 day history of incomprehensible speech and right-sided weakness. On examination, she was weak-looking, tachycardic (114 bpm), underweight (BMI 17.45 kg/m2) and with palpable, hard, non-tender epigastric mass measuring 10×7 cm. She also had hyperpigmented patches on both legs initially manifesting as intermittent oozing excoriations over the past 2 years. Cranial MRI revealed subacute CVD infarct. Anticoagulation was given. On the second day, she started having diarrheal episodes. Supportive management was given. Serum glucagon was markedly elevated (>25,000 pg/mL, normal <80). Serum PP (6562 pg/mL, normal <228) and gastrin (621 pg/mL, normal 13-115) were elevated. Serum VIP (<50 pg/mL, normal <75) was normal. Further investigations showed prediabetes (FBS 112 mg/dL, HbA1c 5.7%) and normocytic, normochromic anemia. Screening for MEN1 was unremarkable. On the third day, she had progression of slurred speech and right-sided weakness. Cranial MRI revealed acute lacunar infarcts. As part of hypercoagulability workup, 2D-Echocardiogram showed depressed ejection fraction (20.9%), dilated LV with normal relative wall thickness, and global hypokinesia of the LV wall, confirming dilated cardiomyopathy. Carotid and Venous Duplex scans were normal. Anti-heart failure regimen was initiated. Due to her current neurologic, cardiac and metabolic conditions, she cannot proceed with surgery and chemotherapy. She was given octreotide injection 30 mg every 4 weeks for 3 doses. Post-treatment, repeat abdominal CT and 2D-Echocardiogram showed no significant change. In the meantime, she had improved appetite and no recurrence of vomiting nor diarrhea. She denies palpitations and can better tolerate walking and climbing stairs. Conclusion We reported a rare case of glucagonoma in a young female with documented constellation of symptoms and complications associated with the syndrome, prominently including dilated cardiomyopathy. It is therefore recommended that cardiovascular evaluation should be initiated at the time of glucagonoma diagnosis for timely and appropriate management. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.