Glucagonoma syndrome: a review and update on treatment

  title={Glucagonoma syndrome: a review and update on treatment},
  author={A John and Robert A. Schwartz},
  journal={Journal of the European Academy of Dermatology and Venereology},
  • A. John, R. Schwartz
  • Published 1 December 2016
  • Medicine
  • Journal of the European Academy of Dermatology and Venereology
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… 

Glucagonoma: From skin lesions to the neuroendocrine component (Review).

Increased awareness of the condition involves multidisciplinary practitioners because NME as paraneoplastic syndrome may be present for months and even years until adequate recognition and therapy; it is remitted after successful pancreatic surgery.


In this case, pancreatectomy and hepatic lobectomy followed by somatostatin analogue therapy was the chosen treatment strategy and adds new knowledge about glucagonoma which is important due to the low incidence of the disease and the particular characteristics of the syndrome.

Necrolytic Migratory Erythema: Complete Healing after Surgical Removal of Pancreatic Carcinoma.

A 55-year-old female patient with a 5-year history of unrecognized cutaneous and systemic manifestations of glucagonoma syndrome without metastases is presented, and the diagnosis of glucagonsoma syndromewithout metastases was established.

Case report of a recurrent resected glucagonoma

Glucagonoma syndrome with severe erythematous rash

Clinicians should consider the diagnosis of glucagonoma according to the typical initial symptoms, and a multidisciplinary approach is effective in patients with unresectable metastatic tumors.

Necrolytic Migratory Erythema: A Forgotten Paraneoplastic Condition

A case of a middle-aged woman seen in the authors' dermatology clinic with longstanding skin findings suggestive of NME revealing a glucagonoma, which is often the first clinical finding of an occult neuroendocrine pancreatic neoplasia is reported.

Normoglycemic glucagonoma syndrome associated with necrolytic migratory erythema

A 44-year-old male patient presented with a 4-year history of unexplained exanthema and unintentional weight loss with a mildly pruritic rash, and two low-grade neuroendocrine neoplasms consistent with glucagonoma and NME were confirmed.

Spleen-preserving distal pancreatectomy and lymphadenectomy for glucagonoma syndrome

The patient recovered uneventfully with the glucagonoma syndrome disappeared soon after surgery, and the postoperative plasma glucagon decreased to a normal level, and follow-up showed no recurrence for 5 years since the surgery.

Necrolytic migratory erythema associated with glucagonoma treated successfully with cyclosporine

Additional knowledge regarding effective drugs for treating persistent NME is needed because NME significantly affects patients’ quality of life and is characteristically resistant to treatment.

Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma

The data suggest that if persistent NME is associated with DM and high glucagon levels, timely abdominal imaging should be performed to confirm glucagonoma and surgery and somatostatin analogs are effective for symptom relief and tumor control.



Glucagonoma syndrome: A case report.

A case of NME with a typical skin rash, which was misdiagnosed and treated with corticosteroids for two years, and removal of the tumor in the pancreatic body led to the rapid relief of the symptoms.

Glucagonoma and pseudoglucagonoma syndrome.

A retrospective review of glucagonoma and pseudoglucagonoma cases observed between January 1998 and December 2003 in three hospitals suggests a real prevalence of glucagonaloma syndrome could be greater than currently estimated.

Glucagonoma syndrome associated with necrolytic migratory erythema.

The necrolytic migratory erythema is important for the clinical recognition of glucagonoma, and its early diagnosis is essential for a successful curative therapy.

The glucagonoma syndrome: clinical features, diagnosis, and treatment.

For many years thereafter, pancreatic endocrine tumors were characterized as either insulin-producing or “nonfunctional,” the latter being derived from cells apparently devoid of clinically significant hormonal activity, but with time, other islet cell secretory products were discovered and their tumors identified with various clinical syndromes.

Glucagonoma syndrome: a case report with focus on skin disorders

This report reports a case of a 55-year-old man with a 2-year history of recalcitrant eruption, who had abdominal computer tomography performed, which revealed a tumor in the tail of the pancreas, and cutaneous lesions vanished in a few days.

Clinical and Metabolic Aspects of Glucagonoma

Study of patients with glucagonoma does suggest that glucagon has some role in the etiology of hyperglycemia in diabetic states; however, as in studies on diabetes, investigations on glucoseagonoma do not demonstrate that glucagons has a primary role in producing severe glucose intolerance.

Metastatic glucagonoma.

A 53-year-old female who has necrotizing and crusty maculopapular lesions on the upper and lower extremities and was diagnosed with bullous pemphigoid who died on postoperative day 12 due to liver failure has a case of a very rare endocrine tumor of the pancreas.

Imaging features of glucagonoma syndrome: A case report and review of the literature

In conclusion, imaging examinations are useful for determining the location and size of a glucagonoma, and MRI is able to identify the distinctive morphological features of the lesion.

Glucagonoma and pseudoglucagonoma syndromes

  • R. Schwartz
  • Medicine
    International journal of dermatology
  • 1997
The hallmark of both the glucagonoma and the pseudoglucagonoma syndromes is a specific cutaneous eruption, necrolytic migratory erythema, all attributable to a glucagon-secreting alpha-cell tumor of the pancreas.

Etiology and pathogenesis of necrolytic migratory erythema: review of the literature.

The exact pathogenesis and treatment of NME remain ill-defined despite many case reports and studies on NME in the literature, and a PubMed review of the literature on the etiology and pathogenesis found no clear consensus among the investigators of a universally accepted pathogenesis.