carcinoid

カルチノイド

同

argentaffinoma

　

WordNet

1. a small tumor (benign or malignant) arising from the mucosa of the gastrointestinal tract; usually associated with excessive secretion of serotonin

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/11/21 01:45:27」(JST)

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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.

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
• Diarrhea
• Wheezing
• Abdominal cramping
• Peripheral edema

The outflow of serotonin can cause a depletion of tryptophan leading to niacin deficiency. Niacin deficiency, aka 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

Carcinoid metastasis can lead to carcinoid syndrome. This is due to the over-production of many substances, including serotonin, which is 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% 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.

While 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, like serotonin, and a history and physical, annual physicals thereafter.

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.

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. doi:10.1111/j.1365-2362.2011.02537.x. 

External links

• Caring for Carcinoid Foundation
• Carcinoid Cancer Foundation
• European Neuroendocrine Tumor Society

UpToDate Contents

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• 1. カルチノイド症候群の臨床的特徴 clinical features of the carcinoid syndrome
• 2. 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
• 3. 非転移性カルチノイド腫瘍の病期分類、治療、およびサーベイランス staging treatment and surveillance of non metastatic carcinoid tumors
• 4. 気管支神経内分泌（カルチノイド）腫瘍：疫学、危険因子、分類、組織学的検査、診断、および病期分類 bronchial neuroendocrine carcinoid tumors epidemiology risk factors classification histology diagnosis and staging
• 5. カルチノイド腫瘍の臨床的特徴 clinical characteristics of carcinoid tumors

English Journal

• Outcome after resection and radiofrequency ablation of liver metastases from small intestinal neuroendocrine tumours.

• Norlén O, Stålberg P, Zedenius J, Hellman P.SourceDepartment of Surgical Sciences, Uppsala University, Uppsala, Sweden.
• The British journal of surgery.Br J Surg.2013 Oct;100(11):1505-14. doi: 10.1002/bjs.9262.
• BACKGROUND: In patients with small intestinal neuroendocrine tumour (SI-NET), liver resection or radiofrequency ablation (RFA) of liver metastases is performed for palliation of carcinoid syndrome, and in an effort to improve survival. Data are generally reported from case series, and no randomized
• PMID 24037573

• Initial experience with video-assisted thoracoscopic bronchoplasty.

• Agasthian T.SourceNational University Hospital, National University of Singapore, Singapore.
• European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery.Eur J Cardiothorac Surg.2013 Oct;44(4):616-23. doi: 10.1093/ejcts/ezt166. Epub 2013 May 14.
• OBJECTIVES: Bronchial-origin involvement by endobronchial tumours or direct invasion by tumour or metastatic lymph nodes is a relative contraindication for video-assisted thoracoscopic (VATS) lobectomy. However, selected cases can be resected by VATS bronchoplasty.METHODS: Between 2006 and 2009, 21
• PMID 23674660

• Small bowel tumors discovered during double-balloon enteroscopy: analysis of a large prospectively collected single-center database.

• Cangemi DJ, Patel MK, Gomez V, Cangemi JR, Stark ME, Lukens FJ.SourceDepartments of *Internal Medicine †Gastroenterology & Hepatology, Mayo Clinic, Jacksonville, FL.
• Journal of clinical gastroenterology.J Clin Gastroenterol.2013 Oct;47(9):769-72. doi: 10.1097/MCG.0b013e318281a44e.
• BACKGROUND: The emergence of capsule endoscopy and double-balloon enteroscopy (DBE) has greatly enhanced the management of small bowel tumors (SBTs). DBE is particularly useful as a diagnostic modality because it allows for direct investigation of the gastrointestinal lumen, yet little data exist re
• PMID 23426457

Japanese Journal

• 症例 十二指腸カルチノイドを合併した胃癌の2例

• 寒河江 三太郎,永瀬 剛司,金井 歳雄 [他]
• 外科 74(10), 1114-1118, 2012-10
• NAID 40019441903

• 症例報告 腎カルチノイドに対して腎部分切除4年後,腎局所再発および多発リンパ節転移を認めた1例

• 米虫 良允,永田 政義,西松 寛明 [他]
• 日本泌尿器科学会雑誌 103(5), 665-670, 2012-09
• NAID 40019418767

Related Links

• カルチノイド - Wikipedia

カルチノイド（英: carcinoid）とは、神経内分泌細胞への分化を示す腫瘍のひとつである 。小腸や大腸などの消化管や肺の気管支などの神経内分泌細胞が常在している粘膜 内に発生することが多いが、膵臓、肝臓、腎臓、精巣、卵巣、胸腺、乳腺、前立腺など ...

Related Pictures

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★リンクテーブル★

リンク元	「カルチノイド」「癌様体」
拡張検索	「malignant carcinoid syndrome」「bronchial carcinoid」

「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

「癌様体」

　　[★]

英

carcinoid

関

カルチノイド

「malignant carcinoid syndrome」

　　[★]

悪性カルチノイド症候群

「bronchial carcinoid」

　　[★] 気管支カルチノイド