Glucagonoma syndrome associated with necrolytic migratory erythema

Filho, Florentino de Araújo Cardoso; Feitosa, Roney Gonçalves Fechine; Fechine, Carolina Oliveira Costa; Matos, Carlos; Cardoso, Amanda Linhares; Cardoso, Daniel

doi:10.1590/1806-9282.61.03.203

Cited by 10 publications

(10 citation statements)

References 16 publications

(19 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Glucagonoma syndrome is rare, with an estimated global incidence of one in 20 million people . A recent report found an incidence of glucagonoma of 2% in 1310 cases of pancreatic neuroendocrine tumours in the US over a 28‐year period .…”

Section: Epidemiology/aetiologymentioning

confidence: 99%

“…There are several theories for the pathogenesis of NME. First, hyperglucagonemia seems to play a role, since surgical removal of glucagonomas or stabilizing glucagon levels with medication results in rash resolution . Likely, the increased hepatocyte gluconeogenesis and lipolysis leads to hypoaminoacidemia.…”

Section: Pathogenesismentioning

confidence: 99%

“…Its distribution may be widespread or isolated to the perioral region, trunk, groin, intergluteal region, genital area, lower extremities and fingers (Fig. ) . It preferentially forms in intertriginous areas and pressure points; it can also form in areas of trauma, a response pattern known as koebnerization …”

Section: Clinical Featuresmentioning

confidence: 99%

See 2 more Smart Citations

Glucagonoma syndrome: a review and update on treatment

John

Schwartz

2016

Acad Dermatol Venereol

View full text Add to dashboard Cite

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.

show abstract

Section: Epidemiology/aetiologymentioning

confidence: 99%

Section: Pathogenesismentioning

confidence: 99%

See 1 more Smart Citation

Glucagonoma syndrome: a review and update on treatment

John

Schwartz

2016

Acad Dermatol Venereol

View full text Add to dashboard Cite

show abstract

“…The NME rash starts as annular pattern of erythema and progresses to bullae, ulcers and crusts [7,8]. It has a characteristic distributionthe lesions are most often seen in the perigenital regions, although they can also be found on the trunk, lower limbs, perioral region and sites of minor trauma [2,10]. The differential diagnosis includes such dermatological diseases as contact dermatitis, inertrigo, acrodermatitisenteropathica, pellagra, psoriasis, eczema, pemphigus and seborrheic dermatitis [9,10].…”

Section: Discussionmentioning

confidence: 99%

“…In 1942, Becker, Kahn and Rothman first noticed an association between characteristic skin disorder and pancreatic tumor [1]. Then in 1966 McGavran et al [1] discovered the origin of this disorder thanks to radioimmunoassay technique for glucagon and named this combination of symptoms as a glucagonoma syndrome [2]. Since its first description [1], around 300 cases of glucagonoma and glucagonoma syndrome have been described.…”

Section: Introductionmentioning

confidence: 99%

PRRT as an alternative method of treatment in patient with glucagonoma syndrome: A case report

Popławska-Kita

Szyszkowska

Brelska³

et al. 2017

Prog Health Sci

View full text Add to dashboard Cite

Introduction: Glucagonoma is a rare pancreatic neuroendocrine tumor derived from alpha-cells of the islet of Langerhans. Due to oversecretion of glucagon it is associated with a characteristic paraneoplastic phenomenon, called glucagonoma syndrome, which consists of necrolytic migratory erythema (NME), weight loss, diabetes mellitus, diarrhea, normochromic normocytic anemia, deep vein thrombosis or pulmonary embolism and neuropsychiatric disturbances. Treatment modalities include surgical removal of tumor, somatostatin analogs and peptide receptor radionuclide therapy (PRRT). Case report: We present a case of 61-year-old woman diagnosed with glucagonoma in April 2012. Initially, body-caudal pancreatomy and resection of regional lymph nodes were performed. Five months after surgery, a PET-CT scan detected pathological mass with expression of somatostatin receptors in pancreatic body and metastases to regional lymph nodes. What is more, since April 2014 the patient had complained about persistent pruritus of the entire body. At present, due to the nonsurgical pancreatic mass and metastases she is treated with somatostatin analogs and PRRT. During this therapy the pruritus had decreased and currently there is no sign of cutaneous disease. Moreover, reduction of tumor size was obtained. Conclusions: PRRT may reduce tumor size and by reducing bothersome symptoms substantially improve the quality of life in patients with SSTR-positive tumors

show abstract

Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma

Yang

Deng

et al. 2022

Sci Rep

View full text Add to dashboard Cite

Glucagonoma is an extremely rare neuroendocrine tumor that arises from pancreatic islet alpha cells. Although glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. In this study, we present the cumulative experiences, clinical characteristics and treatments of seven patients diagnosed with glucagonoma during the past 10 years at the First Affiliated Hospital of Xi’an Jiaotong University. The seven patients in our cohort consisted of six females and one male with an average diagnosis age of 40.1 years (range 23–51). The average time from onset of symptoms to diagnosis of glucagonoma was 14 months (range 2–36 months). All the patients visited dermatology first for necrolytic migratory erythema (NME) 7/7 (100%), and other presenting symptoms included diabetes mellitus (DM) 4/7 (57%), stomatitis 2/7 (28%), weight loss 4/7 (57%), anemia 4/7 (57%), diarrhea 1/7 (14%), and DVT1/7 (14%). Plasma glucagon levels were increased in all patients (range 216.92–3155 pg/mL) and declined after surgery. Imaging studies revealed that four of seven patients had liver metastasis. Six of seven patients received surgical resection, and all of them received somatostatin analog therapy. Symptoms improved significantly in 6 out of 7 patients. Three of seven patients died of this disease by the time of follow-up. Our data suggest that if persistent NME is associated with DM and high glucagon levels, timely abdominal imaging should be performed to confirm glucagonoma. Once diagnosed, surgery and somatostatin analogs are effective for symptom relief and tumor control.

show abstract

Glucagonoma syndrome associated with necrolytic migratory erythema

Cited by 10 publications

References 16 publications

Glucagonoma syndrome: a review and update on treatment

Glucagonoma syndrome: a review and update on treatment

PRRT as an alternative method of treatment in patient with glucagonoma syndrome: A case report

Necrolytic migratory erythema is an important visual cutaneous clue of glucagonoma

Contact Info

Product

Resources

About