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glucagonoma

Pathology: 1) tumor of pancreatic islet alpha cells 2) 50-80% malignant [3] Clinical manifestations: 1) rarely diabetes 2) rash (mean size of tumor with skin manifestations is 16 cm) a) secondary to branch-chain amino acid depletion in skin b) vulvovaginitis in women c) migrating necrotizing dermatitis - intertriginous erythema, scales & erosions d) erosions, crusting & peeling involving perineum & perioral areas e) lesions may be generalized 3) stomatitis 4) glossitis 5) angular cheilitis 6) anemia 7) diarrhea 8) weight loss 9) malabsorption 10) neuropsychiatric symptoms [5] Laboratory: 1) serum chemistries a) serum glucose: hyperglycemia b) glucagon: fasting levels > 1000 ng/L makes diagnosis c) low serum zinc 2) skin biopsy 3) see ARUP consult [4] Management: 1) prognosis depends on aggressiveness of tumor - hepatic metastases in 75% at time of diagnosis 2) response to chemotherapy is generally poor a) sunitinib & everolimus are active [3] b) capecitabine plus temozolomide is active [3] 3) surgical excision a) 25-30% success because peristent metastases b) reduces tumor mass & associated symptoms

Interactions

disease interactions

Related

glucagon; contains: glicentin; glicentin-related polypeptide; GRPP; oxyntomodulin; OXM; OXY; glucagon-like peptide 1; GLP-1; glucagon-like peptide 1(7-37); GLP-1(7-37); glucagon-like peptide 1(7-36); GLP-1(7-36); glucagon-like peptide 2; GLP-2 (GCG) migratory necrolytic erythema; glucagonoma syndrome; erythema necrolytica migrans

General

amine precursor uptake & decarboxylation tumor (APUDoma) islet cell tumor; pancreatic neuroendocrine neoplasm; nesidioblastoma

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 171
  2. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 512
  3. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17m 18. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018,
  4. ARUP Consult: Glucagonoma The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/glucagonoma
  5. NEJM Knowledge+ Endocrinology