Glucagonoma syndrome

     

     

     

    Introduction:

     

    Glucagonoma syndrome:

    • Paraneoplastic syndrome associated with glucagon-producing (α-cell) pancreatic islet tumor
    • In 3% part of MEN1
    • In 70% presenting syndrome
    • 5 year survival 50%
    • Hypercatabolic state induced by excessive levels of glucagon (and pancreatic polypeptide) leads to relative deficiency of Zn, free fatty acids, amino acids, vitamins B and perhaps other essential nutrient

    Clinical Presentation:

    • Necrolytic migratory erythema (NME) (erythema, epidermal necrosis, bulla formation, shedding and hyperpigmentation) (B3, free fatty acids, B12)
    • Diabetes mellitus
    • Weight loss
    • Anemia (B12)
    • Stomatitis, cheilitis, glossitis (B2,B3)
    • Diarrhoea (B3)

    Important considerations:

    Glucagonoma syndrome is not the only syndrome associated with clinical features of migratory necrolytic erythema. Additional syndromes include:

    • Paraglucagonoma
    • Necrolytic aral erythema

    Paraglucagonoma is a multifactorial nutritional deficiency syndrome associated with migratory necrolytic erythema in the context of:

    • Other pancreatic and non pancreatic cancers
    • Chronic pancreatitis
    • Celiac disease
    • Jejunal adenocarcinoma
    • Crohn’s disease
    • Hepatic cirrhosis

    Clinical differential diagnosis:

    Necrolytic acral erythema is a multifactorial nutritional deficiency syndrome associated with hyperkeratotic necrolytic erythema. citation Most cases were associated with Hepatitis C infection. citation

     

    Histological features citation:

     

    • Acanthosis
    • Parakeratosis
    • Epidermal pallor
    • Epidermal cell vacuolization
    • Later stages: superficial and intraepidermal mixed infiltrate with lymphocytes, eosinophils and neutrophils
    • Residual: post-inflammatory hyperpigmentation

    Histological pearls:

    • lorem
    • ipsum
    • dolor

    External Links:

    Cases associated with this book:

  • Scottsdale 2012 Didactic Session VI Case Vignette 26
    Author: Artur Zembowicz M.D. Ph.D.

    Conference: Dr. Z's Consultations
  • Glucagonoma syndrome
    Author: Artur Zembowicz M.D. Ph.D.

    Conference: DermatopathologyConsultations.com Teaching Collection
  • Early histological changes in glucagonoma
    Author: Artur Zembowicz M.D. Ph.D.

    Conference: Dr. Z's Consultations