WordNet

of or relating to or resembling a cyst
of or relating to a normal cyst (as the gallbladder or urinary bladder)
a closed sac that develops abnormally in some body structure
relating to or containing mucin

PrepTutorEJDIC

胞嚢(ほうのう)性の
(体内にできる)新生物;腫瘍(しゅよう)
胞嚢(ほうのう)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/06/02 23:49:10」(JST)

wiki en

Pancreatic mucinous cystic neoplasm, also mucinous cystic neoplasm of the pancreas and mucinous cystic tumour, is a grouping of cystic neoplasms that arise from the pancreas. They may be benign, malignant or in between.

The prognosis of this set of tumours is highly variable and dependent on the specific sub-classification (benign, borderline, malignant).

Definition

Mucinous cystic neoplasms of the pancreas are defined by the World Health Organization (WHO) as cystic epithelial neoplasms that occur almost exclusively in women; do not communicate with the pancreatic ductal system and which are composed of columnar, mucin-producing epithelium, supported by ovarian-type stroma. This means that these tumors make mucin (a thick sticky fluid), they do not arise in the larger pancreatic ducts, and they have a peculiar supporting stroma (or support structure).

Classification

Mucinous cystic neoplasms of the pancreas classified as benign, borderline, or malignant, based on histomorphologic features which predict their behaviour.^[1]

Symptoms

Most mucinous cystic neoplasms arise in women (95%) and these women are usually in their 40s or 50s. Clinically many mucinous cystic neoplasms are discovered as incidental findings during imaging for another indication. Larger mucinous cystic neoplasms can compress adjacent organs and lead to weight loss, or a sense of fullness. Really large mucinous cystic neoplasms can even be felt on physical examination. A multidisciplinary approach including good clinical history, state of the art imaging, and careful pathology is often needed to establish the correct diagnosis.^[2]

Pathology

Resected mucinous cystic neoplasms have a characteristic appearance- they tend to be between 6 and 10 centimeters in size (2 to 5 inches), they contain numerous cysts (small cavities), and the cysts are filled with thick mucin (see the photograph). Under the microscope, the cysts are lined by tall mucin-producing cells. These are the tumor cells. The tumor cells “sit” on ovarian-type stroma. This “ovarian-type stroma” is connective tissue that resembles some of the tissue normally found in the female ovary.^[3]

Some mucinous cystic neoplasms are confined to the tumor. These are designated using various names including “mucinous cystic neoplasm with low-grade dysplasia,” “mucinous cystic neoplasm with moderate dysplasia,” “borderline mucinous cystic neoplasm,” and “mucinous cystic neoplasm with high-grade dysplasia.” These are all curable when completely removed surgically. About a third of mucinous cystic neoplasms have progressed or “degenerated” into an invasive cancer. These are designated either “mucinous cystadenocarcinoma” or “mucinous cystic neoplasm with an associated invasive carcinoma.” These latter lesions have the potential to spread (metastasize) to other organs.

Treatment

A multi-disciplinary team approach, involving the coordinated efforts of clinicians, radiologists and pathologists is often the best way to evaluate a tumor involving the pancreas.^[2] In general, if the patient is healthy enough, the treatment of choice is surgical resection. Patients with an invasive cancer arising in association with a mucinous cystic neoplasm are often treated post-operatively with chemotherapy or radiation therapy.

References

^ Theruvath TP, Morgan KA, Adams DB (August 2010). "Mucinous cystic neoplasms of the pancreas: how much preoperative evaluation is needed?". Am Surg 76 (8): 812–7. PMID 20726409.
^ ^a ^b "Pancreatic Cancer Multi-Disciplinary Clinic at Johns Hopkins University".
^ "Pancreatic Cancer Frequently Asked Questions".

UpToDate Contents

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1. 膵嚢胞性腫瘍 pancreatic cystic neoplasms
2. 膵嚢胞の分類 classification of pancreatic cysts
3. 膵管内乳頭粘液性腫瘍の診断および治療 diagnosis and treatment of intraductal papillary mucinous neoplasm of the pancreas
4. Intraductal papillary mucinous neoplasm of the pancreas (IPMN): Pathophysiology and clinical manifestations
5. Cystic fibrosis: Hepatobiliary disease

English Journal

Pdx1 expression in pancreatic precursor lesions and neoplasms.

Park JY, Hong SM, Klimstra DS, Goggins MG, Maitra A, Hruban RH.Source*Department of Pathology, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, TX Departments of †Pathology §Oncology ∥Medicine, The Sol Goldman Pancreatic Cancer Research Center ¶The McKusick-Nathans Institute of Genetic Medicine, The Johns Hopkins Medical Institutions, Baltimore, MD ‡Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY.
Applied immunohistochemistry & molecular morphology : AIMM / official publication of the Society for Applied Immunohistochemistry.Appl Immunohistochem Mol Morphol.2011 Oct;19(5):444-9.
Pancreatic and duodenal homeobox (Pdx1) is a homeobox transcription factor required for the embryonic development of the pancreas. Pdx1 expression has been earlier identified in pancreatic ductal adenocarcinomas and endocrine neoplasms. This study characterizes Pdx1 protein expression in pancreatic
PMID 21297446

Intraductal papillary mucinous neoplasm and acute pancreatitis.

Venkatesh PG, Navaneethan U, Vege SS.Source*Digestive Disease Institute, The Cleveland Clinic, Cleveland, OH †Miles and Shirley Fiterman Center for Digestive Diseases, Mayo Clinic, Rochester, MN.
Journal of clinical gastroenterology.J Clin Gastroenterol.2011 Oct;45(9):755-8.
Intraductal papillary mucinous neoplasms (IPMNs) are cystic pancreatic tumors that arise from the pancreatic ducts and are increasingly reported worldwide. Both benign and malignant tumors of the pancreas are thought to contribute to recurrent pancreatitis possibly by pancreatic duct obstruction, an
PMID 21602701

Japanese Journal

長期経過観察中に画像所見の変化を認めた膵粘液性嚢胞腫瘍の1例

福田順子,田中幸子,仲尾美穂,上田絵理,鈴木玲子,高倉玲奈,高野保名,井岡達也,吉岡二三,冨田裕彦
Japanese journal of medical ultrasonics = 超音波医学 38(1), 19-24, 2011-01-15
NAID 10027890581

Epithelial Cyst Arising in an Intrapancreatic Accessory Spleen: A Diagnostic Dilemma

Yamanishi Hirofumi,Kumagi Teru,Yokota Tomoyuki,Koizumi Mitsuhito,Azemoto Nobuaki,Watanabe Jota,Mizuno Yosuke,Sugita Atsuro,Abe Masanori,Ikeda Yoshio,Matsuura Bunzo,Hiasa Yoichi,Onji Morikazu
Internal Medicine 50(18), 1947-1952, 2011
… The cases were mainly diagnosed as mucinous cystic neoplasm (MCN) preoperatively based on clinical and imaging features, such as, a woman in middle age; …
NAID 130000969301

Related Pictures

★リンクテーブル★

リンク元	「膵嚢胞」「膵管内乳頭粘液性腫瘍」「粘液性嚢胞腫瘍」「粘液嚢腺腫」
関連記事	「neoplasm」「mucinous」「cystic」

「膵嚢胞」

　　[★]

英: pancreatic cyst
同: 膵嚢胞症？、(国試)嚢胞性膵疾患？

疫学

膵嚢胞の80%が仮性嚢胞、20%が真性嚢胞

仮性嚢胞は急性膵炎後のものが最も多く、膵炎後2-3週後に生じる物が多い。

分類

仮性嚢胞：内腔面が上皮細胞でおおわれない
真性嚢胞：内腔面が上皮細胞で覆われる

先天性
貯留性
腫瘤性　→　造影CTで隔壁が濃染する

漿液性嚢胞腫瘍

漿液性嚢胞腺腫
漿液性嚢胞腺癌)

粘液性嚢胞腫瘍

粘液性嚢胞腺腫
粘液性嚢胞腺癌

SSUR.628

	真性嚢胞						仮性嚢胞
	先天性嚢胞	貯留性嚢胞	腫瘍性嚢胞				急性仮性嚢胞	慢性仮性嚢胞
			粘液性嚢胞腫瘍	漿液性嚢胞腫瘍	膵管内乳頭粘液性腫瘍
	congenital cyst	retention cyst	mucinous cystic neoplasm	serous cystic neoplasm	intraductal papillary mucinous neoplasm		acute pseudocyst	chronic pseudocyst
			MCN	SCN	IPMN
					主膵管型	分枝型
病因	例えば、遺伝性(嚢胞線維症)	膵管閉塞(腫瘍、膵石、炎症など)					急性膵炎、外傷など	慢性膵炎、慢性の貯留嚢胞
特徴		長期の経過で上皮が剥脱して仮性嚢胞との区別が困難になる		星芒状中心瘢痕、石灰化多い	粘液を産生。乳頭状増殖。
疫学			閉経前後の女性
部位			膵尾部に好発
嚢胞の大きさ				小嚢胞
被膜			共通	共通
腫瘍化			壁在結節出現や隔壁肥厚	稀	70%が悪性	70%以上が良性
内容物			粘液	漿液	粘液
治療			悪性の可能性があり切除		悪性の可能性があり切除	経過観察も可能	自然消失が多い。	自然消失はない
治療						条件により切除	6週経過を見る。

検査

画像検査

膵嚢胞腺腫　→　see RNT.223

[show details]

治療

真性嚢胞:

嚢胞摘出術

仮性嚢胞

消化管との内瘻形成術

「膵管内乳頭粘液性腫瘍」

　　[★]

英: intraductal papillary-mucinous neoplasm intraductal papillary mucinous neoplasm IPMN
関: 膵嚢胞、粘液性嚢胞腺腫

図：SSUR.630(分類)

概念

膵管内で乳頭状に増殖し、粘液を産生する腫瘍

分類と性質

主膵管型IPMN：70%が悪性
分枝型IPMN：70%以上が良性

病態

膵管内乳頭粘液性腫瘍は乳頭状に増殖し、膵管内視鏡でイクラ状隆起性病変が認められる。
膵管内乳頭粘液性腫瘍の膵管内増殖による膵管の狭窄と腫瘍の粘液産生により膵管のびまん性拡張が見られる。また、このために膵管分枝のブドウの房状拡張が認められる。
主膵管型膵管内乳頭粘液性腫瘍ではファーター乳頭が開口し粘液の分泌を見ることがある。

の開大

治療

主膵管型、混合型は悪性の可能性が高く切除
分枝型は(1)粘液による主膵管閉塞により急性膵炎を反復する場合、(2)壁在結節のある場合、(3)主膵管径の大きい場合、(4)嚢胞径が大きい場合(>3-4cm)に切除(SSUR.631)。 (also see 参考4)

予後

増殖速度は遅く手術切除できた場合、予後は良好である。

粘液性嚢胞腫瘍と膵管内乳頭粘液腫瘍

	粘液性嚢胞腫瘍 mucinous cystic neoplasm MCN	膵管内乳頭粘液性腫瘍 intraductal papillary mucinous neoplasm IPMN
特徴	粘液産生する上皮が嚢胞を形成	粘液を産生。乳頭状増殖。
疫学	閉経前後の女性	高齢の男性
部位	膵尾部	膵頭部
共通被膜	被膜あり	被膜なし
腫瘍化	壁在結節出現や隔壁肥厚	70%が悪性
内容物	粘液	粘液
治療	悪性の可能性があり切除	悪性の可能性があり切除
画像	http://www.uptodate.com/contents/image?imageKey=GAST/7321	http://www.uptodate.com/contents/image?imageKey=GAST/6504

参考

1. シェーマ

http://www.teikyo-hbps.jp/suizou_2.html

2. ERCP 主膵管型

http://www.kuhp.kyoto-u.ac.jp/~pancreas/pan-1-2.jpg

3. [charged] 膵管内乳頭粘液性腫瘍の病態生理および臨床症状 - uptodate [1]
4. [charged] 膵管内乳頭粘液性腫瘍の診断および治療 - uptodate [2]
5. [charged] 膵嚢胞の分類 - uptodate [3]
6. [charged] 膵嚢胞性腫瘍 - uptodate [4]

国試

105I019

「粘液性嚢胞腫瘍」

　　[★]

英: mucinous cystic neoplasm MCN、mucinous cystic tumor, MCT
関: 粘液嚢腺腫、膵嚢胞。膵腫瘍

分類

粘液性嚢胞腫瘍と膵管内乳頭粘液腫瘍

	粘液性嚢胞腫瘍 mucinous cystic neoplasm MCN	膵管内乳頭粘液性腫瘍 intraductal papillary mucinous neoplasm IPMN
特徴	粘液産生する上皮が嚢胞を形成	粘液を産生。乳頭状増殖。
疫学	閉経前後の女性	高齢の男性
部位	膵尾部	膵頭部
共通被膜	あり	なし
腫瘍化	壁在結節出現や隔壁肥厚	70%が悪性
内容物	粘液	粘液
治療	悪性の可能性があり切除	悪性の可能性があり切除
画像	http://www.uptodate.com/contents/image?imageKey=GAST/7321	http://www.uptodate.com/contents/image?imageKey=GAST/6504

http://saigaiin.sakura.ne.jp/sblo_files/saigaiin/image/E886B5E38080E58E9FE69CAC20-20E382B3E38394E383BC.pdf

	MCN	IPMN(分枝型)
性別	男性＜＜女性	男性＞女性
好発年齢	中年	高齢
部位	尾部	頭部＞体尾部
嚢胞形態	嚢胞内に小嚢胞の形成 cyst-in-cyst	多房性ブドウの房状
嚢胞壁	厚い	薄い
膵管との交通	±	＋
卵巣様間質	＋	ー