Glucagonoma syndrome: a review and update on treatment

Abstract

Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumour of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema (NME). NME is usually a specific and often initial finding of glucagonoma syndrome, but it may occur in other settings unassociated with an alpha-cell pancreatic tumour (pseudoglucagonoma syndrome). Glucagonoma syndrome must be distinguished from pseudoglucagonoma syndrome. Prompt recognition of NME and subsequent workup for a glucagonoma can allow for an earlier diagnosis and enhance the chances of a favourable outcome. In particular, metastases occur late, so early recognition of glucagonoma syndrome before liver metastases can be life-saving. Surgical resection is the definitive treatment for glucagonoma syndrome, although chemotherapeutic agents, somatostatin analogues and radionuclide therapy are also employed. Herein, we offer an approach to workup after identifying NME and an update on its current treatment modalities.

Normoglycemic glucagonoma syndrome associated with necrolytic migratory erythema

“…Glucagonoma is associated with striking clinical manifestations, referred to as the 'glucagonoma syndrome'. 1 The glucagonoma syndrome includes NME, glossitis, cheilitis, venous thrombosis, hyperglucagonaemia, diabetes mellitus, Letters to the Editor anaemia, weight loss and neuropsychiatric disorders. Apart from NME, the manifestations are non-specific, accounting for a frequent diagnostic delay.…”

“…It has a worldwide prevalence of 0.5-2%. 1 Vitiligo can have a major negative impact on patients' quality of life. 2 Current treatments include topical corticosteroids and immunomodulators, systemic immunosuppressants, cosmetic camouflage, ultraviolet radiation and surgical procedures, e.g.…”

Necrolytic migratory erythema associated with glucagonoma treated successfully with cyclosporine

“…Glucagonoma syndrome is a rare paraneoplastic syndrome characterized by elevated serum levels of glucagon, the presence of glucagonoma, and dermatological clinical findings such as necrolytic migratory erythema (NME) (John & Schwartz, ; Luber, Ackerman, Culpepper, Buschmann, & Koep, ). The main challenge associated with the diagnosis of glucagonoma syndrome is that at diagnosis, metastasis has already occurred in more than 50% of cases.…”

“…The main challenge associated with the diagnosis of glucagonoma syndrome is that at diagnosis, metastasis has already occurred in more than 50% of cases. However, this tumor tends to exhibit slow growth; median survival is 3–7 years and patients have survived for up to 24 years (John & Schwartz, ; Mendoza‐Guil, Hernández‐Jurado, Burkhardt, Linares, & Naranjo, ). Furthermore, NME significantly affects patients' quality of life and is characteristically resistant to treatment (Thomaidou, Nahmias, Gilead, Zlotogorski, & Ramot, ).…”

Erythema as a Visual Surrogate Marker of Glucagonoma

“…We thought that the successful treatment of glucagonoma by sunitinib improved the necrolytic migratory erythema. Because necrolytic migratory erythema is an important paraneoplastic syndrome associated with glucagonoma (2), physicians should be aware of this symptom.…”