carcinoid tumor

カルチノイド

WordNet

1. a small tumor (benign or malignant) arising from the mucosa of the gastrointestinal tract; usually associated with excessive secretion of serotonin
2. an abnormal new mass of tissue that serves no purpose (同)tumour, neoplasm

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/08/12 04:53:45」(JST)

wiki en

[Wiki en表示]

Carcinoid
Picture of a carcinoid tumor (center of image) that encroaches into the lumen of the small bowel (pathology specimen). The prominent folds are plicae circulares, a characteristic of the small bowel.
Classification and external resources
Specialty	Oncology
ICD-10	C75, E34.0
ICD-9-CM	209.60
ICD-O	M8240/3
OMIM	114900
DiseasesDB	2040
MedlinePlus	000347
eMedicine	med/271
MeSH	D002276

Primary site of a carcinoid cancer of gut

Carcinoid (also carcinoid tumor) is a slow-growing type of neuroendocrine tumor originating in the cells of the neuroendocrine system. In some cases, metastasis may occur. Carcinoid tumors of the midgut (jejunum, ileum, appendix, and cecum) are associated with carcinoid syndrome.

Carcinoid tumors are the most common malignant tumor of the appendix, but they are most commonly associated with the small intestine, and they can also be found in the rectum and stomach. They are known to grow in the liver, but this finding is usually a manifestation of metastatic disease from a primary carcinoid occurring elsewhere in the body. They have a very slow growth rate compared to most malignant tumors. The median age at diagnosis for all patients with neuroendocrine tumors is 63 years.

Signs and symptoms

While most carcinoids are asymptomatic through the natural lifetime and are discovered only upon surgery for unrelated reasons (so-called coincidental carcinoids), all carcinoids are considered to have malignant potential.

About 10% of carcinoids secrete excessive levels of a range of hormones, most notably serotonin (5-HT), causing:

• Flushing Flushing (serotonin itself does not cause flushing). Potential causes of flushing in carcinoid syndrome include bradykinins, prostaglandins, tachykinins, substance P, and/or histamine, diarrhea, and heart problems. Because of serotonin's growth-promoting effect on cardiac myocytes,[14] a serotonin-secreting carcinoid tumour may cause a tricuspid valve disease syndrome, due to the proliferation of myocytes onto the valve.
• Diarrhea
• Wheezing
• Abdominal cramping
• Peripheral edema

The outflow of serotonin can cause a depletion of tryptophan leading to niacin deficiency. Niacin deficiency, also known as pellagra, is associated with dermatitis, dementia, and diarrhea.

This constellation of symptoms is called carcinoid syndrome or (if acute) carcinoid crisis. Occasionally, haemorrhage or the effects of tumor bulk are the presenting symptoms. The most common originating sites of carcinoid is the small bowel, particularly the ileum; carcinoid tumors are the most common malignancy of the appendix. Carcinoid tumors may rarely arise from the ovary or thymus.

They are most commonly found in the midgut at the level of the ileum or in the appendix. The next most common affected area is the respiratory tract, with 28% of all cases — per PAN-SEER data (1973 – 1999). The rectum is also a common site.

Gastrointestinal

Carcinoid tumors are apudomas that arise from the enterochromaffin cells throughout the gut. Over two-thirds of carcinoid tumors are found in the gastrointestinal tract.

Lung

Main article: Typical lung carcinoid tumor

Carcinoid tumors are also found in the lungs.

Other sites / metastases

Metastasis of carcinoid can lead to carcinoid syndrome. This is due to the over-production of many substances, including serotonin, which are released into the systemic circulation, and which can lead to symptoms of cutaneous flushing, diarrhea, bronchoconstriction, and right-sided cardiac valve disease. It is estimated that less than 6% of carcinoid patients will develop carcinoid syndrome, and of these, 50% will have cardiac involvement.

Treatment

Surgery, if feasible, is the only curative therapy. If the tumor has metastasized (most commonly, to the liver) and is considered incurable, there are some promising treatment modalities, such as radiolabeled octreotide (e.g. Lutetium (¹⁷⁷Lu) DOTA-octreotate) or the radiopharmaceutical 131I-mIBG (meta iodo benzyl guanidine) for arresting the growth of the tumors and prolonging survival in patients with liver metastases, though these are currently experimental.

Chemotherapy is of little benefit and is generally not indicated. Octreotide or Lanreotide (somatostatin analogues) may decrease the secretory activity of the carcinoid, and may also have an anti-proliferative effect. Interferon treatment is also effective, and usually combined with somatostatin analogues.

As the metastatic potential of a coincidental carcinoid is probably low, the current recommendation is for follow up in 3 months with CT or MRI, labs for tumor markers such as serotonin, and a history and physical, with annual physicals thereafter.

Goblet cell carcinoid

Main article: Goblet cell carcinoid

This is considered to be a hybrid between an exocrine and endocrine tumor derived from crypt cells of the appendix. Histologically, it forms clusters of goblet cells containing mucin with a minor admixture of Paneth cells and endocrine cells. The growth pattern is distinctive: typically producing a concentric band of tumor nests interspersed among the muscle and stroma of the appendiceal wall extending up the shaft of the appendix. This makes the lesion difficult to suspect grossly and difficult to measure. Small tumor nests may be camouflaged amongst the muscle or in periappendiceal fat; cytokeratin preparations best demonstrate the tumor cells; mucin stains are also helpful in identifying them. They behave in a more aggressive manner than do classical appendiceal carcinoids. Spread is usually to regional lymph nodes, peritoneum, and particularly the ovary. They do not produce sufficient hormonal substances to cause the carcinoid or other endocrine syndromes. In fact, they more closely resemble exocrine than endocrine tumors. The term 'crypt cell carcinoma' has been used for them, and though perhaps more accurate than considering them carcinoids, has not been a successful competitor.

History

They were first characterized in 1907 by Siegfried Oberndorfer, a German pathologist at the University of Munich, who coined the term karzinoide, or "carcinoma-like", to describe the unique feature of behaving like a benign tumor despite having a malignant appearance microscopically. The recognition of their endocrine-related properties were later described by Gosset and Masson in 1914, and these tumors are now known to arise from the enterochromaffin (EC) and enterochromaffin-like (ECL) cells of the gut. Some sources credit Otto Lubarsch with the discovery.

In 2000, the World Health Organization redefined "carcinoid", but this new definition has not been accepted by all practitioners. This has led to some complexity in distinguishing between carcinoid and other neuroendocrine tumors in the literature. According to the American Cancer Society, the 2000 WHO definition states:

The WHO now divides these growths into neuroendocrine tumors and neuroendocrine cancers. Neuroendocrine tumors are growths that look benign but that might possibly be able to spread to other parts of the body. Neuroendocrine cancers are abnormal growths of neuroendocrine cells which can spread to other parts of the body.

See also

• Carcinoid syndrome
• Don Meyer, head coach emeritus of the Northern State University men's basketball team. Professor Meyer was found to have carcinoid cancer following an automobile accident in September 2009.
• Derrick Bell, Professor and Legal Scholar, died of carcinoid cancer October 5, 2011.

References

Cunningham JL, Janson ET (2011). "The Hallmarks of Ileal Carcinoids". Eur J Clin Invest. 41 (12): 1353–60. PMID 21605115. doi:10.1111/j.1365-2362.2011.02537.x. 

External links

• The Neuroendocrine Cancer Awareness Network (NCAN)
• Carcinoid Cancer Foundation
• European Neuroendocrine Tumor Society

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. カルチノイド腫瘍の臨床的特徴 clinical characteristics of carcinoid tumors
• 2. Staging, treatment, and posttreatment surveillance of non-metastatic carcinoid tumors
• 3. カルチノイド症候群の臨床的特徴 clinical features of the carcinoid syndrome
• 4. Metastatic well-differentiated gastrointestinal neuroendocrine (carcinoid) tumors: Systemic therapy options to control tumor growth and symptoms of hormone hypersecretion
• 5. カルチノイド症候群および腫瘍局在の診断 diagnosis of the carcinoid syndrome and tumor localization

English Journal

• Three synchronous gastrointestinal tumours.

• Boland BM, Collins CG, Christiansen E, O'Brien A, Duignan J.SourceDepartment of Surgery, St Michaels Hospital, Dun Laoghaire, Co Dublin, Ireland, bredanmboland@gmail.com.
• Irish journal of medical science.Ir J Med Sci.2011 Dec;180(4):897-900. Epub 2009 Mar 17.
• Mucinous cystadenomas of the appendix are rare, with significant malignant potential [Lo and Sarr in Hepatogastroenterology 50(50):432-437, 2003]. Carcinoid tumours are similarly uncommon, rarely occurring in Meckel's diverticula but are still the commonest tumour of Meckel's diverticulae [Nies et a
• PMID 19291353

• Ghrelin immunoexpression in pituitary adenomas.

• Rotondo F, Cusimano M, Scheithauer BW, Rotondo A, Syro LV, Kovacs K.SourceDivision of Pathology, Department of Laboratory Medicine, St. Michael's Hospital, University of Toronto, 30 Bond Street, Toronto, ON, M5B 1W8, Canada, rotondof@smh.ca.
• Pituitary.Pituitary.2011 Dec;14(4):318-22.
• Ghrelin, an orexigenic hormone, is normally produced mainly in stomach. In addition, it has been demonstrated in gastric carcinoid tumors and less often in other neuroendocrine tumors. We investigated ghrelin expression by immunohistochemistry (streptavidin-biotin-peroxidase complex method) in the f
• PMID 21340501

Japanese Journal

• 当院にて経験した気管支肺カルチノイドの6例

• 緒方 彩子,出水 みいる,高山 浩一,井上 博雅,中西 洋一
• 気管支学 : 日本気管支研究会雑誌 33(1), 16-20, 2011-01-25
• 背景.肺カルチノイドは肺悪性腫瘍全体の1?2%程度を占める稀な疾患である. 対象.2004年6月から2009年6月までの過去5年間に当科において診断,治療を施行したカルチノイドの6例を検討した. 結果.当科における過去5年間の肺悪性腫瘍727例中,カルチノイドは0.8%にあたる6例に認めた.平均年齢68.7歳,女性,定型的カルチノイド,右肺が多い傾向が見られ,従来の報告どおりであった.転帰はリンパ …
• NAID 110008460777

• 肺カルチノイド切除例の臨床的検討

• 馬場 哲郎,浦本 秀隆,山田 壮亮,桑田 泰治,永田 好香,重松 義紀,下川 秀彦,小野 憲司,竹之山 光広,花桐 武志
• 日本呼吸器外科学会雑誌 = The journal of the Japanese Association for Chest Surgery 25(1), 2-6, 2011-01-15
• 肺カルチノイド切除10例について臨床病理学的因子と治療成績に関して検討を行った．定型的カルチノイド（TC）が7例で非定型的カルチノイド（AC）が3例．平均年齢は49.5歳で，男性6例，女性4例（全例TC）であった．術前にカルチノイドの診断がついたのは4例であり，他組織型の肺癌と診断されたのが4例．術式は肺摘除が2例，二葉切除が1例，肺葉切除4例，区域切除1例，部分切除1例，気管支形成術1例であった …
• NAID 10027421553

Related Links

• Carcinoid Tumor

4 May 2010 ... Carcinoid tumors are derived from primitive stem cells in the gut wall but can be seen in other organs,[1] including the lungs,[2] mediastinum, thymus,[3] liver, pancreas, bronchus, ovaries,[4] prostate,[5] and kidneys.

Related Pictures

---

★リンクテーブル★

リンク元	「ソマトスタチン」「カルチノイド」
拡張検索	「carcinoid tumor of lung」
関連記事	「carcinoid」「tumor」

「ソマトスタチン」

　　[★]

英

somatostatin (Z)

同

膵ソマトスタチン pancreatic somatostatin、成長ホルモン分泌抑制因子 growth hormone release inhibiting hormone(GH-RIH)、somatotropin release inhibiting factor(SRIF)

関

消化管ホルモン

• 成長ホルモンに拮抗する作用を有する
• 成長に必要な栄養の消化吸収を抑制

分類

• 消化管ホルモン？・中枢神経系のホルモン(様々なところで発現しており、多くの生理活性により神経の調節を行っている(参考1))？

性状

• ペプチド

• 環状構造を取っており、ジスルフィド結合によりこの構造が保たれている。
• 二つの型があり、共通のpreprohormonが転写後プロセッシングを受けてsomatostatin-14(14 a.a.)とsomatostatin-28(28 a.a.)ができる。somatostatin-14はsomatostatin-28のC末の配列と同一である。

産生組織

• 消化器

• 膵臓：膵島D細胞
• 消化管の粘液細胞(胃の噴門、小腸)

• 視床

参考1

• 神経系：大脳皮質、視床下部、脳幹、脊髄の皮質に豊富。心臓、甲状腺、皮膚、眼、胸腺の神経に局在している。
• 消化器系：消化管と膵臓に豊富。そこで傍分泌あるいは内分泌のような形でD細胞により、そして腸神経によって産生されている。

受容体

• G蛋白共役型受容体(5つのサブタイプがある。どのサブタイプもGiαサブユニットを介する)

作用

First Aid step 1 p.269

総じて、消化活動を止めさせる方向に作用

• 分泌抑制：胃：胃酸・ガストリンの分泌
• 分泌抑制：十二指腸：セクレチンの分泌 (QB CBT vol2 p.295)
• 分泌抑制：膵臓と小腸の分泌
• 分泌抑制：胆嚢の収縮
• 分泌抑制：インスリン、グルカゴン放出

(下垂体で？)

• 成長ホルモン分泌抑制
• 甲状腺刺激ホルモン分泌抑制

参考1より

• ソマトスタチンにより抑制されるホルモン　　(↓ホルモンとは思えない物もまじっていいる気が)

• 中枢神経系：アセチルコリン、バソプレシン、成長ホルモン、ニューロテンシン、セロトニン、甲状腺刺激ホルモン(TSH)、サブスタンスP
• 消化管ホルモン：コレシストキニン、グルカゴン、ガストリン、インスリン、モチリン、pancreatic polypeptide、セクレチン、セロトニン、サブスタンスP、VIP
• その他：表皮成長ホルモン(epidermal growth hormone)

分泌の調整

分泌亢進

• 酸

分泌抑制

• 迷走神経刺激

臨床関連

• VIPomaとcarcinoid tumorの治療に使われる
• ソマトスタチノーマ

参考

• 1.[charged]Physiology of somatostatin and its analogues - uptodate [1]

「カルチノイド」

　　[★]

英

carcinoid

同

カルチノイド腫瘍 carcinoid tumor

好銀性細胞腫 argyrophil cell tumor

関

カルチノイド症候群、消化管カルチノイド

概念

• 気管支や消化管に発生し、原腸系臓器に広く分布する内分泌系細胞腫瘍の低異型度腫瘍。消化管に発生したものは特に消化管カルチノイドという(YN.A-75)

• 腫瘍。神経内胚葉系の腸クロム親和細胞に由来。形態的に癌に類似するが、悪性度は低い。発育は緩慢。転移はしうる。
• chromogranin A, synaptophysin, neuron-specific enolaseを含む
• 生理活性物質を産生しうる　→　カルチノイド症候群

• ヒスタミン、セロトニン、カテコールアミン、プロスタグランジン、カリクレイン、ACTH

疫学

• 欧米では小腸腫瘍の中で悪性腫瘍に次いで2番目に多いが、日本では非常にまれ。

分類

部位

• 甲状腺腫性カルチノイド
• 胸腺カルチノイド
• 気管支カルチノイド
• 肺カルチノイド

• 消化管カルチノイド

• 胃カルチノイド
• 小腸カルチノイド
• 大腸カルチノイド
• 虫垂カルチノイド
• 直腸カルチノイド

• 副腎カルチノイド

発生部位

• 発生部位：消化管(虫垂、大腸、小腸、直腸、胃)および肺、気管支

• 直腸が最多 (YN.A-75)
• 肺：発生母地(気管支腺、細気管支上皮内に存在するKultchitzky細胞)。神経内分泌顆粒を有する。(NSU.344)

病理

• 粘膜下腫瘍と同様の所見を呈することが多い。　　ちなみにGISTは粘膜下腫瘍
• つまり辺縁はなだらかである。

• 細胞の配列：索状、リボン状。時にロゼット様
• 核：小型、円形で中心に存在し、分裂像は少ない

予後

• 予後(5年生存率)：虫垂のカルチノイドを除いて90%。小腸原発のカルチノイドで肝臓に転移があっても50% (BPT.627)

消化管 (BPT.626)

疫学

• The peak incidence of these neoplasms is in the sixth decade.(BPT.626)
• 直腸結腸癌の2%以下。小腸の悪性腫瘍の半分を占める
• 転移：虫垂と直腸のカルチノイドは転移しにくい。90%の胃、空腸、結腸のカルチノイドは

直腸

疫学(NSU.590)

• 消化器系のカルチノイドのうち最多(36%)。
• 40-50歳代に多い。
• 男：女=1.4:1

国試

• 101G027
• 097D026

「carcinoid tumor of lung」

　　[★] 肺カルチノイド、肺カルチノイド腫瘍

「carcinoid」

　　[★] カルチノイド

同

argentaffinoma

　

「tumor」

　　[★]

• n.

• 腫瘍、腫瘤、腫脹