Glucagonoma syndrome: a review and update on treatment

@article{John2016GlucagonomaSA,
  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},
  year={2016},
  volume={30}
}
  • 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… 
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63 Citations
Highly Influential Citations
6
Background Citations
25
Methods Citations
2

63 Citations

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

TLDR
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.

MANIFESTATIONS OF GLUCAGONOMA SYNDROME.

TLDR
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.

TLDR
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

TLDR
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

TLDR
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

TLDR
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

TLDR
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

TLDR
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

TLDR
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.
...

References

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TLDR
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.

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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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  • R. Schwartz
  • Medicine
    International journal of dermatology
  • 1997
TLDR
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.

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TLDR
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.
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