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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/10/29 19:36:00」(JST)

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A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in up to a 1000-fold overproduction of the hormone glucagon. Alpha cell tumors are commonly associated with glucagonoma syndrome, though similar symptoms are present in cases of pseudoglucagonoma syndrome in the absence of a glucagon-secreting tumor.

History[edit]

Fewer than 251 cases of glucagonoma have been described in the literature since their first description by Becker in 1942. Because of its rarity (fewer than one in 20 million worldwide), long-term survival rates are as yet unknown.

Symptoms[edit]

The primary physiological effect of glucagonoma is an overproduction of the peptide hormone glucagon, which enhances blood glucose levels through the activation of anabolic and catabolic processes including gluconeogenesis and lipolysis respectively. Gluconeogenesis produces glucose from protein and amino acid materials. It also increases lipolysis, which is the breakdown of fat. The net result is hyperglucagonemia, decreased blood levels of amino acids (hypoaminoacidemia), anemia, diarrhea, and weight loss of 5–15 kg.

Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is the presenting problem in 70% of cases.^[1] Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower abdomen, buttocks, perineum, and groin.

Diabetes mellitus also frequently results from the insulin and glucagon imbalance that occurs in glucagonoma.^[2] Diabetes mellitus is present in 80–90% of cases of glucagonoma, and is exacerbated by preexisting insulin resistance.

Diagnosis[edit]

A blood serum glucagon concentration of 1000 pg/mL or greater is indicative of glucagonoma (the normal range is 50–200 pg/mL).

However, recent studies have shown that forty percent of patients have plasma glucagon levels ranging from 500 to 1000 pg/mL. Increased levels have been reported in cases of renal insufficiency, acute pancreatitis, hypercorticism, hepatic diseases, severe stress, extended fasting, and familial hyperglucagonemia. Rarely do these cases result in levels over 500 pg/mL, except in the case of patients with hepatic diseases.^[3]

Blood tests may also reveal abnormally low concentrations of amino acids, zinc, and essential fatty acids, which are thought to play a role in the development of NME. Skin biopsies may also be taken to confirm the presence of NME.

A CBC can uncover anemia, which is an abnormally low level of hemoglobin.

The tumor itself may be localized by any number of radiographic modalities, including angiography, CT, MRI, PET, and endoscopic ultrasound. Laparotomy is useful for obtaining histologic samples for analysis and confirmation of the glucagonoma.

Treatment[edit]

Heightened glucagon secretion can be treated with the administration of octreotide, a somatostatin analog, which inhibits the release of glucagon.^[4] Doxorubicin and streptozotocin have also been used successfully to selectively damage alpha cells of the pancreatic islets. These do not destroy the tumor, but help to minimize progression of symptoms.

The only curative therapy for glucagonoma is surgical resection, where the tumor is removed. Resection has been known to reverse symptoms in some patients.

References[edit]

^ van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR (November 2004). "The glucagonoma syndrome and necrolytic migratory erythema: a clinical review". Eur. J. Endocrinol. 151 (5): 531–7. doi:10.1530/eje.0.1510531. PMID 15538929.
^ Koike N, Hatori T, Imaizumi T, Harada, N, Fukuda, A, Takasaki, K, Iwamoto, Y (2003). "Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus". Journal of hepato-biliary-pancreatic surgery 10 (1): 101–5. PMID 12918465.
^ Goldman, Lee, and Ausiello Dennis. Cecil Textbook of Medicine. Philadelphia: Saunders, 2004.
^ Moattari AR, Cho K, Vinik AI (1990). "Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses". Surgery 108 (3): 581–7. PMID 2168587.

External links[edit]

The Johns Hopkins University Pancreatic Islet Cell Tumor / Pancreatic Endocrine Neoplasms Webpage

UpToDate Contents

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1. グルカゴン産生腫瘍およびグルカゴノーマ症候群 glucagonoma and the glucagonoma syndrome
2. 転移性の胃腸膵管系神経内分泌腫瘍：腫瘍増殖およびホルモン過剰分泌による症状を管理
するための全身治療の選択肢 metastatic gastroenteropancreatic neuroendocrine tumors systemic therapy options to control tumor growth and symptoms of hormone hypersecretion
3. 膵神経内分泌腫瘍（膵島細胞腫瘍）の分類、疫学、臨床症状、局在、および病期分類 classification epidemiology clinical presentation localization and staging of pancreatic neuroendocrine tumors islet cell tumors
4. 内臓悪性腫瘍の皮膚症状 cutaneous manifestations of internal malignancy
5. 消化器における神経内分泌腫瘍の発生の病理、分類、および重症度分類 pathology classification and grading of neuroendocrine tumors arising in the digestive system

English Journal

Fibroblast growth factor 21 mediates specific glucagon actions.

Habegger KM, Stemmer K, Cheng C, Müller TD, Heppner KM, Ottaway N, Holland J, Hembree JL, Smiley D, Gelfanov V, Krishna R, Arafat AM, Konkar A, Belli S, Kapps M, Woods SC, Hofmann SM, D'Alessio D, Pfluger PT, Perez-Tilve D, Seeley RJ, Konishi M, Itoh N, Kharitonenkov A, Spranger J, Dimarchi RD, Tschöp MH.SourceMetabolic Disease Institute, Division of Endocrinology, Diabetes, and Metabolism, Department of Medicine, University of Cincinnati, Cincinnati, Ohio.
Diabetes.Diabetes.2013 May;62(5):1453-63. doi: 10.2337/db12-1116. Epub 2013 Jan 10.
Glucagon, an essential regulator of glucose homeostasis, also modulates lipid metabolism and promotes weight loss, as reflected by the wasting observed in glucagonoma patients. Recently, coagonist peptides that include glucagon agonism have emerged as promising therapeutic candidates for the treatme
PMID 23305646

[Diagnosis and treatment of glucagonoma: report of one case].

Cheng H, Chen MS, Yang GL.SourceDepartment of General Surgery, First Affiliated Hospital of Chongqing Medical University, Chongqing 400016, China. E-mail: eschenghong@126.com.
Nan fang yi ke da xue xue bao = Journal of Southern Medical University.Nan Fang Yi Ke Da Xue Xue Bao.2013 Apr 20;33(4):618-insidebackcover.
OBJECTIVE: lucagonoma is a rare islet alpha-cell tumor. We report a case of glucagonoma in a 55-year-old male patient with such clinical findings of necrolytic migratory erythema, diabetes mellitus, body weight loss, and anemia. CT examination found a space-occupying lesion in the pancreas, and an e
PMID 23644134

Pancreatic insulinoma combined with glucagon positive cell: A case report.

Yamashita S, Tanaka N, Takahashi M, Nagai M, Furuya T, Suzuki Y, Nomura Y.SourceSuguru Yamashita, Nobutaka Tanaka, Michiro Takahashi, Motoki Nagai, Takatoshi Furuya, Yukihiro Nomura, Department of Surgery, Asahi General Hospital, Chiba 289-2511, Japan.
World journal of gastrointestinal surgery.World J Gastrointest Surg.2013 Mar 27;5(3):68-72. doi: 10.4240/wjgs.v5.i3.68.
We present a 70-year-old man who was referred for surgery with uncontrollable hypoglycemia. Ultrasonography and abdominal contrast computed tomography revealed a hypervascular tumor of 1 cm in diameter in the pancreatic tail. With a diagnosis of insulinoma, we performed a distal pancreatectomy. The
PMID 23556064

Japanese Journal

外科的切除によって耐糖能異常の改善が得られたグルカゴノーマ合併インスリノーマの1例

糖尿病 60(1), 30-36, 2017
NAID 130005310328

腹腔鏡下膵体尾部切除を施行したグルカゴン産生成分を伴う膵インスリノーマの1例

膵臓 31(2), 145-149, 2016
NAID 130005149301

主膵管内進展を伴う膵グルカゴノーマの1切除例

日本消化器外科学会雑誌 49(3), 216-224, 2016
NAID 130005138114

Related Pictures

★リンクテーブル★

リンク元	「グルカゴノーマ」「膵内分泌腫瘍」
拡張検索	「glucagonoma syndrome」

「グルカゴノーマ」

Glucagonoma
	Classification and external resources
ICD-10	C25.4
ICD-9	157.4, 211.7
ICD-O:	M8152/1
DiseasesDB	5257
MedlinePlus	000326
eMedicine	med/896
MeSH	D005935

　　[★]

英: glucagonoma
同: グルカゴン産生腫瘍、A細胞腫 A cell tumor、グルカゴノーマ症候群 glucagonoma syndrome
関: 膵内分泌腫瘍、膵島細胞腫瘍、壊死性遊走性紅斑。多発性内分泌腫瘍症1型(ウェルマー症候群)

[show details]

グルカゴノーマ : 約 8,640 件
グルカゴン産生腫瘍 : 約 20,000 件

概念

膵臓のα細胞に由来するグルカゴン産生腫瘍。
膵臓原発がほとんどで、ごく稀に腸管に原発する。
悪性が多い。

疫学

中年以降に好発

症候

三徴：高血糖、口内炎・舌炎、壊死性遊走性紅斑

口腔　：舌炎、口内炎
内分泌：高血糖　→　糖尿病
血液　：低アミノ酸血症
皮膚　：致死性遊走性紅斑

検査

画像検査：膵管が突然途絶するような像はみられない？　⇔　膵癌

診断

血中グルカゴン値、皮疹、画像診断で診断

参考

1. [charged] グルカゴン産生腫瘍およびグルカゴノーマ症候群 - uptodate [1]

「膵内分泌腫瘍」

　　[★]

英: pancreatic endocrine tumor, endocrine tumor of pancreas
同: 膵内分泌性膿瘍、膵内分泌系腫瘍

インスリノーマ(insulinoma)：インスリン分泌腫瘍：インスリン：膵内分泌腫瘍の中で最も多い
グルカゴノーマ(glucagonoma)：グルカゴン分泌腫瘍：グルカゴン：膵内分泌腫瘍の15%
ガストリノーマ(gastrinoma)：ガストリン分泌腫瘍：ガストリン：

「glucagonoma syndrome」

　　[★] グルカゴノーマ症候群

[pmid15538929-1] van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR (November 2004). "The glucagonoma syndrome and necrolytic migratory erythema: a clinical review". Eur. J. Endocrinol. 151 (5): 531–7. doi:10.1530/eje.0.1510531. PMID 15538929.

[2] Koike N, Hatori T, Imaizumi T, Harada, N, Fukuda, A, Takasaki, K, Iwamoto, Y (2003). "Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus". Journal of hepato-biliary-pancreatic surgery 10 (1): 101–5. PMID 12918465.

[3] Goldman, Lee, and Ausiello Dennis. Cecil Textbook of Medicine. Philadelphia: Saunders, 2004.

[4] Moattari AR, Cho K, Vinik AI (1990). "Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses". Surgery 108 (3): 581–7. PMID 2168587.

匿名

検索

案内

案内

glucagonoma