Dermatologic Manifestations of Glucagonoma Syndrome Treatment & Management

Updated: Aug 24, 2021
  • Author: Ali Alikhan, MD; Chief Editor: William D James, MD  more...
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Medical Care

Most traditional chemotherapies are not effective on glucagonoma, probably due to the tumor's indolent nature. No cure by medical means has been reported, but some drug and hormone regimens may induce tumor regression or symptomatic remission of necrolytic migratory erythema (NME).

Streptozocin, a drug that causes selective islet cell damage, shows promise in combination with 5-fluorouracil. The combination can achieve a 60-70% response rate. Dacarbazine induced remission from NME by reducing tumor burden and glucagon levels in at least one instance. [17]

Transcatheter hepatic artery embolization (TAE) may induce selective necrosis of hepatic metastases by providing locally high levels of antineoplastic agents, thus sparing systemic adverse effects. TAE, in association with cisplatin-releasing gelatin microspheres, has been reported in one study for metastatic liver tumors. [18]

The somatostatin agonists octreotide and lanreotide are the drugs of choice in the treatment of patients with glucagonoma. These somatostatin analogs can be coupled to radioisotopes for both imaging and treatment applications. [19] Peptide receptor radioligand therapy (PRRT) with radiolabeled somatostatin analogue therapy has been tried with mixed results. [20]

Sunitinib, a small molecule inhibitor of multiple receptor tyrosine kinases critical for tumor growth and angiogenesis, including PDGF receptors, has been shown to be effective in treating numerous pancreatic neuroendocrine tumors but may be particularly efficacious in the treatment of glucagonomas. [21]

In pseudoglucagonoma syndrome, treating the underlying disease often resolves NME. One case report describes the successful use of octreotide for treating NME with pseudoglucagonoma. [22]


Surgical Care

Surgical resection is curative and, thus, is the treatment of choice if the tumor is localized. [23]   Often, the resection can be performed without adjunct therapy because the tumor is slow growing and tends to be encapsulated. The use of laparoscopy has been reported in a few cases.

When the tumor is disseminated, surgical management may be used for palliation while somatostatin or chemotherapeutic agents (see above) are administered. Since NME is the major source of morbidity in glucagonoma syndrome, palliative therapies are aimed at reducing the cutaneous eruption. Tumor debulking has been shown to decrease the intensity of the cutaneous symptoms by reducing the level of serum glucagon.

Multimodality therapy for liver metastases combining surgery, transarterial chemoembolization, percutaneous radiofrequency thermal ablation, and long-acting octreotide has been proven to be effective. [24]



Cases of NME secondary to celiac sprue can resolve with implementation of a gluten-free diet. Deficiencies in amino acids, essential fatty acids, and zinc have been linked to NME by observation that symptoms resolve with supplementation of the deficient nutrient. [25]