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Gastrointestinal (GI) System

Glucagonoma

Extremely rare slowly growing, frequently malignant neuroendocrine tumor of the α-cells of the pancreas.

Extremely rare slowly growing, frequently malignant neuroendocrine tumor of the α-cells of the pancreas.

  • Type of functional pancreatic neuroendocrine neoplasm (pNEN)
  • Accounts for 2% of islet cell carcinomas

Glucagonomas are neuroendocrine tumors of the pancreatic islets that secrete glucagon.

History:

The first case was described in 1942 by Becker et al. in a 45-year-old woman presenting with widespread dermatitis, weight loss, glossitis, and abnormal glucose tolerance associated with an islet cell neoplasm of the pancreas on autopsy specimen. In 1966, McGavran and colleagues identified the classic symptoms of mild diabetes and dermatitis along with elevated glucagon levels related to a metastatic alpha cell tumor of the pancreatic islets.

Clinical features

Glucagonoma syndrome

Glucagonoma syndrome occurs due to the effects of elevated glucagon levels secreted by the tumor.

Classical description of glucagonoma syndrome includes the 4D syndrome, consisting of dermatosis (necrolytic migratory erythema), new onset of diabetes mellitus, deep vein thrombosis, and depression along with anemia, glossitis, weight loss in the presence of hyperglucagonemia

Necrolytic migratory erythema (NME) (~65–70% cases)

hallmark of glucagonoma syndrome and is characterized by an annular pattern of erythema with centrally formed fragile vesicles, bullae and crusts
Skin lesions of glucagonoma
Skin lesions of glucagonoma. (A) Necrolytic migratory erythema. (B) Cheilitis and glossitis. (C) Skin lesions with erosions, serous exudate and crusting. (D) Pathology of the skin biopsy, observed with hematoxylin and eosin staining (magnification, x100). | Wei, J., Lin, S., Wang, C., Wu, J., Qian, Z., Dai, C., Jiang, K., & Miao, Y. I. (2015). Glucagonoma syndrome: A case report. Oncology letters, 10(2), 1113–1116. https://doi.org/10.3892/ol.2015.3275

Diabetes mellitus (40% on presentation but 90% develop over time)

Clinical hallmark in which elevated glucagon levels promote the glucose output and antagonize the effect of insulin.

Neuropsychiatric manifestations:

  • Depression (50% cases)
  • Other features: Dementia, psychosis, agitation, hyperreflexia, ataxia, paranoid delusions, and proximal muscle weakness

Other features:

  • Weight loss (60% cases)
  • Anemia (50% cases)
  • Deep vein thrombosis (DVT) (50% cases)
  • Glossitis/stomatitis/cheilitis (40% cases)
  • Chronic diarrhea (30% cases)
Changes in skin lesions following treatment
Changes in skin lesions following treatment: (A) Skin lesion upon admission. (B) Changes in skin lesions following treatment of octreotide and intravenous amino acid infusion. (C) Relief of skin lesion one week after surgery. | Wei, J., Lin, S., Wang, C., Wu, J., Qian, Z., Dai, C., Jiang, K., & Miao, Y. I. (2015). Glucagonoma syndrome: A case report. Oncology letters, 10(2), 1113–1116. https://doi.org/10.3892/ol.2015.3275

Differential diagnosis:

  • Pseudoglucagonoma syndrome: Celiac disease, malabsorption, cirrhosis, malignancy and pancreatitis may also present NME-like skin lesions

Management

Surgical management:

Only method of treatment.
Tumor mass identified by computed tomography (CT) scan and surgical resection
Tumor mass identified by computed tomography (CT) scan and surgical resection. (A) CT scanning indicated the pancreatic body tumor. (B) Laparotomy confirmed the tumor in the pancreatic body. (C) Resected tumor. | Wei, J., Lin, S., Wang, C., Wu, J., Qian, Z., Dai, C., Jiang, K., & Miao, Y. I. (2015). Glucagonoma syndrome: A case report. Oncology letters, 10(2), 1113–1116. https://doi.org/10.3892/ol.2015.3275

Non-surgical mangement:

Temporary relief of symptoms
  • Somatostatin analogues (SSA)
  • Amino acid solution infusion
  • Transarterial chemoembolization
  • Radiation therapy
  • Peptide receptor radioligand therapy

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