A Glucagon-Secreting Alpha-Cell Carcinoma of the Pancreas

McGavran, Malcolm H.; Unger, Roger H; Recant, Lillian; Polk, Hiram C.; Kilo, Charles; Levin, Marvin E.

doi:10.1056/nejm196606232742503

Cited by 324 publications

(83 citation statements)

References 5 publications

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“…Originating from the histamine-containing entero chromaffinlike (ECL) cells of the embryologic foregut, gastric carcinoid tumors represent appro xi mately 1.8% of all gastric neoplasms and approximately 7% of all carcinoids (44,45). Because they are most often discovered incidentally during endoscopy, the incidence of gastric carcinoid tumors has increased in recent years as endoscopic technology continues to improve both technologically and diagnostically.…”

Section: Gastric Carcinoidmentioning

confidence: 99%

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Neuroendocrine Tumors — Laboratory Diagnosis

Tzontcheva¹

2010

Journal of Medical Biochemistry

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Kratak sadr`aj: Neuroendokrini tumori (NETs) jesu heterogena grupa neoplazmi poreklom iz endokrinih }elija, koje odlikuju prisustvo sekretornih granula i sposobnost produkcije biogenih amina i polipeptidnih hormona. Ovi tumori poti ~u od endokrinih `lezda kao {to su adrenalna medula, hipofiza i paratiroide, kao i endokrinih insula u okviru tiroide ili pankreasa i raspr{enih endokrinih }elija u respiratornom ili gastrointestinalnom traktu. Klini~ko pona{anje NETs varira u znatnoj meri. Oni mogu biti funkcionalni ili nefunkcionalni, kao i spororastu}i (uspe{no diferentovani NETs), koji ~ine ve}inu, ili veoma agresivni i vrlo zlo}udni tumori (nedovoljno diferentovani NETs). NETs gastrointestinalnog trakta obi~no se dele na dve glavne grupe: 1) karcinoide, i 2) endokrine tumore pankreasa (EPTs). Ve}ina neuroendokrinih tumora proizvodi i sekretuje peptidne hormone i amine. Neke od tih supstanci izazivaju specifi~ne klini~ke sindrome: karcinoidni, Zolinger-Elisonov, hiperglikemijski, glukagonom i VernerMo risonov. Specifi~ni markeri za te sindrome su bazalni i/ili stimulisani nivoi 5-HIAA u urinu, gastrina, insulina, glukagona i vazoaktivnog intestinalnog polipeptida u serumu ili plazmi. Neki karcinoidni tumori kao i otprilike tre}ina endokrinih tumora pankreasa ne daju nikakve klini~ke simptome i nazivaju se »nefunkcionalnim« tumorima. Stoga se uobi ~a -jeni tumorski markeri, poput hromogranina A, pankreasnog polipeptida, neuron-specifi~ne enolaze u serumu i podjedinica glikoproteinskih hormona, koriste za skrining kod pa cijenata bez jasnih klini~kih simptoma vezanih za hormo ne. Me u uobi~ajenim tumorskim markerima kao veoma osetljiv i specifi~an serumski marker za razli~ite tipove neuro endokrinih tumora pokazao se pankreasni polipeptid hro mogranin A, iako njegova funkcija jo{ nije ustanovljena. Razlog tome je {to mo`e biti povi{en i u mnogim slu~ajevima nedovoljno diferentovanih tumora neuroendokrinog porekla koji ne lu~e poznate hormone. Hromogranin A se u ovom trenutku smatra najboljim neuroendokrinim markerom u serumu ili plazmi koji je dostupan za dijagnozu i terapeutsku evaluaciju.

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Section: Gastric Carcinoidmentioning

confidence: 99%

“…The mean somatostatin-like immunoreactivity (SLI) concentration in patients with pancreatic somatostatinoma was 50 times higher than normal (range, 1-250 times). Intestinal somatostatinomas, however, present differently and have only slightly elevated or normal SLI concentrations (31,45).…”

Section: Somatostatinomamentioning

confidence: 99%

Neuroendocrine Tumors — Laboratory Diagnosis

Tzontcheva¹

2010

Journal of Medical Biochemistry

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“…McGavran et al (9) identi-9) identified elevated glucagon levels in the blood and tumor tissue of a patient with diabetes mellitus, NME and a pancreatic tumor. Wilkinson (10) first hypothesised that there may be an association between NME and glucagonoma in his report of a patient with pancreatic carcinoma.…”

Section: Introductionmentioning

confidence: 99%

Glucagonoma and the glucagonoma syndrome (Review)

Song

Zheng

et al. 2017

Oncol Lett

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Abstract. Glucagonoma is an extremely rare pancreatic α-islet cell tumor and is often accompanied by certain clinical symptoms including necrotizing migratory erythema (NME), diabetes, weight loss and anemia. The objectives of the current review were to discern the clinical features, diagnosis, treatment and prognosis of glucagonoma by evaluating 623 reported cases. A 1998 study reviewed 407 cases and 216 cases were reported in studies published after 1998. The current review consisted of 268 males and 339 females, with an average age of 52.4 years. The male-to-female ratio was 0.79. The incidence of typical clinical findings were as follows: NME, 82.4% (350/425); diabetes, 68.5% (291/425); weight loss, 60.2% (256/425); anemia, 49.6% (211/425); and glossitis or stomatitis or cheilitis, 41.2% (175/425). A total of 499 cases reported the location of the tumor as the pancreas and 64.1% (320/499) involved the pancreatic tail. Tumor size was recorded in 58.3% (126/216) cases reported after 1998 and average tumor size was 5.0 cm. Metastasis was detected in 49.2% of patients (293/595 for whom metastasis or no metastasis were recorded) upon diagnosis. These patients were older than those without metastasis (average age, 54.0 years old vs. 50.8 years old).

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“…More than 20 years later, McGavran et al (2) documented hyperglucagonemia in association with the cutaneous eruptions. The combination of symptoms was called the glucagonoma syndrome.…”

Section: Introductionmentioning

confidence: 99%

The glucagonoma syndrome and necrolytic migratory erythema: a clinical review

Beek

Haas

Vloten

et al. 2004

European Journal of Endocrinology

177

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The glucagonoma syndrome is a rare disease in which a typical skin disorder, necrolytic migratory erythema, is often one of the first presenting symptoms. Weight loss and diabetes mellitus are two other prevalent characteristics of this syndrome. Necrolytic migratory erythema belongs to the recently recognized family of deficiency dermatoses of which zinc deficiency, necrolytic acral erythema and pellagra are also members. It is typically characterized on skin biopsies by necrolysis of the upper epidermis with vacuolated keratinocytes. In persistent hyperglucagonemia, excessive stimulation of basic metabolic pathways results in diabetes mellitus at the expense of tissue glycogen stores, and muscle and fat mass. Multiple (essential) nutrient and vitamin B deficiencies develop, which contribute to the dermatosis. In addition, glucagonomas may produce various other products, like pancreatic polypeptide, that add to the catabolic effects of glucagon.European Journal of Endocrinology 151 531-537

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A Glucagon-Secreting Alpha-Cell Carcinoma of the Pancreas

Cited by 324 publications

References 5 publications

Neuroendocrine Tumors — Laboratory Diagnosis

Neuroendocrine Tumors — Laboratory Diagnosis

Glucagonoma and the glucagonoma syndrome (Review)

The glucagonoma syndrome and necrolytic migratory erythema: a clinical review

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