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Metastatic Glucagonoma
Presenting with Weight
Loss and Necrolytic
Migratory Erythema
胃腸科 林榮鈞
Glucagonoma
&
Glucagonoma
Syndrome
Surg Oncol Clin N Am. 2016 Apr;25(2):363-74
Glucagonoma
Rare; Incidence: 0.04 to 0.12 per million per year
Endocr Relat Cancer 2008; 15: pp. 409-427
typically diagnosed in the 5th-6th decade; age range of 16–88 years reported in the literature
equally distributed between the sexes
World Journal of Surgical Oncology, vol. 12, pp. 220–222, 2014
Med Oncol 2007; 24: pp. 330-337
most frequently in the tail; (≈85% are in body or tail)
Medicine (Baltimore) 1996; 75: pp. 53-63
usually large tumors (>5 cm); (0.4–25 cm)
> 60% malignant
50% ~ 80% metastatic liver disease; peripancreatic lymph nodes
Mayo Clin Proc 1996; 71: pp. 1030-1038
European Journal of Endocrinology, vol. 151, pp. 531–537, 2004
Glucagonoma syndrome
weight loss (60%–90%)
diabetes mellitus (40%–95%) 87、89、101年內科專科醫師考試筆試題
gastrointestinal symptoms: diarrhea (10%–25%), constipation, abdominal pain, and anorexia
neurologic symptoms: ataxia, dementia, depression, optic atrophy, and proximal muscle weakness.
necrolytic migratory erythema (NME): erythematous macules, papules, or plaques that
progressively enlarge, necrose, and then form pigmented scars; usually on the anterior lower
extremity or perianal genital regions 89年度內科專科醫師考試筆試題、94年第三次專門職業及技術人員檢覈筆試
97年第一次專門職業及技術人員高等暨普通考試
mucous membranes: painful glossitis, cheilitis, stomatitis, and blepharitis
Thromboembolism/DVT (up to 50%)
4Ds syndrome: dermatitis, diabetes, depression, and deep vein thrombosis.
may also be seen in MEN1 patients
Gastroenterology 2008; 135: pp. 1469-1492
Best Pract Res Clin Gastroenterol 2012; 26: pp. 737-753
Mayo Clin Proc 1996; 71: pp. 1030-1038
J Hepatobiliary Pancreat Sci 2015; 22: pp. 578-585
Nat Rev Gastroenterol Hepatol 2012; 9: pp. 199-208
Journal of Medical Case Reports, vol. 5, pp. 402–406, 2011
Laboratory studies
anemia (30%–90%)
hypoaminoacidemia (30%–100%)
Mayo Clin Proc 1996; 71: pp. 1030-1038
J Hepatobiliary Pancreat Sci 2015; 22: pp. 578-585
Diagnosis:
serum glucagon levels > 500 pg/mL (not specific) to glucagonoma
Normal fasting levels < 150 pg/mL
several conditions may cause mild elevations of glucagon: DM, acute burns and trauma, cirrhosis,
renal failure, Cushing syndrome, and bacteremia Gastroenterology 2008; 135: pp. 1469-1492
Best Pract Res Clin Gastroenterol 2012; 26: pp. 737-753
pancreatic polypeptide (PP) and insulin levels may also be elevated
serial CgA and pancreastatin levels may be helpful to monitor for progression
Novel advances in management of metastatic
pancreatic neuroendocrine tumors
complex liver resections & liver transplantation
percutaneous ablation of liver metastases
long-acting somatostatin analogues
targeted radiotherapy (peptide ligand receptor radionuclide therapy (PRRT)
Radioembolization with Selective Internal Radiation Microspheres
biologic therapy (Sunitinib and Everolimus)
Journal of the American Academy of Dermatology, vol. 54, no. 2, pp. 344–347, 2006
Cancer, vol. 113, no. 5, pp. 921–929, 2008
Post Tx skin lesion f/u
One month Later
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