膵管内乳頭粘液性腫瘍 intraductal papillary-mucinous neoplasm intraductal papillary mucinous neoplasm

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Intraductal papillary mucinous neoplasm (IPMN) is a type of tumor (neoplasm) that grows within the pancreatic ducts (intraductal) and is characterized by the production of thick fluid by the tumor cells (mucinous).^[1] Intraductal papillary mucinous neoplasms are important because if they are left untreated some of them progress to invasive cancer (transform from a benign tumor to a malignant tumor). Just as colon polyps can develop into colon cancer if left untreated, so too do some intraductal papillary mucinous neoplasms progress into an invasive pancreatic cancer. Intraductal papillary mucinous neoplasms can present an opportunity to treat a pancreatic tumor before it develops into an aggressive, hard-to-treat cancer.

History

In 1982, IPMN was reported as a "mucin-producing tumor" by Kazuhiko Ohashi of the Japanese Foundation for Cancer Research.

Classification

Pathologists classify intraductal papillary mucinous neoplasms (IPMNs) into two broad groups - those that are associated with an invasive cancer and those that are not associated with an invasive cancer.^[2] This separation has critical prognostic significance. Patients with a surgically resected intraductal papillary mucinous neoplasm without an associated invasive cancer have an excellent prognosis (>95% will be cured), while patients with a surgically resected intraductal papillary mucinous neoplasm with an associated invasive cancer have a worse prognosis. Intraductal papillary mucinous neoplasms without an associated invasive cancer can be further subcategorized into three groups. They are IPMN with low-grade dysplasia, IPMN with moderate dysplasia, and IPMN with high-grade dysplasia. This categorization is less important ^{[clarification needed]} than the separation of IPMNs with an associated cancer from IPMNs without an associated invasive cancer, but this categorization is useful as IPMNs are believed to progress from low-grade dysplasia to moderate dysplasia to high-grade dysplasia to an IPMN with an associated invasive cancer. ^{[clarification needed]}^{[citation needed]}

Diagnosis

Intraductal papillary mucinous neoplasm in magnetic resonance imaging.

Intraductal papillary mucinous neoplasms can come to clinical attention in a variety of different ways. The most common symptoms include abdominal pain, nausea and vomiting. The most common signs patients have when they come to medical attention include jaundice (a yellowing of the skin and eyes caused by obstruction of the bile duct), weight loss, and acute pancreatitis. These signs and symptoms are not specific for an intraductal papillary mucinous neoplasm, making it more difficult to establish a diagnosis. Doctors will therefore often order additional tests.

Once a doctor has reason to believe that a patient may have an intraductal papillary mucinous neoplasm, he or she can confirm that suspicion using one of a number of imaging techniques. These include computerized tomography (CT), endoscopic ultrasound (EUS), and magnetic resonance cholangiopancreatography (MRCP). These tests will reveal dilatation of the pancreatic duct or one of the branches of the pancreatic duct. In some cases a fine needle aspiration (FNA) biopsy can be obtained to confirm the diagnosis. Fine needle aspiration biopsy can be performed through an endoscope at the time of endoscopic ultrasound, or it can be performed through the skin using a needle guided by ultrasound or CT scanning.

IPMN forms cysts (small cavities or spaces) in the pancreas. These cysts are visible in CT scans (X-ray computed tomography). However, many pancreatic cysts are benign (see Pancreatic disease).

A growing number of patients are now being diagnosed before they develop symptoms (asymptomatic patients). In these cases, the lesion in the pancreas is discovered accidentally (by chance) when the patient is being scanned (x-rayed) for another reason. Up to 6% of patients undergoing pancreatic resection did so for treatment of incidental IPMNs.^[3]

Scientists at the Sol Goldman Pancreatic Cancer Research Center at Johns Hopkins ^[4] reported in the July 20, 2011 issue of Science Translational Medicine ^[5] that they have developed a gene-based test that can be used to distinguish harmless from precancerous pancreatic cysts. The test may eventually help patients with harmless cysts avoid needless surgery. Bert Vogelstein and his colleagues discovered that almost all of the precancerous cysts (intraductal papillary mucinous neoplasms) of the pancreas have mutations in the KRAS and/or the GNAS gene. The researchers then tested a total of 132 intraductal papillary mucinous neoplasms for mutations in KRAS and GNAS. Nearly all (127) had mutations in GNAS, KRAS or both. Next, the investigators tested harmless cysts such as serous cystadenomas, and the harmless cysts did not have GNAS or KRAS mutations. Larger numbers of patients must be studied before the gene-based test can be widely offered.

Treatment

The treatment of choice for main-duct IPMNs is resection due to approximately 50% chance of malignancy. Side-branch IPMNs are occasionally monitored with regular CT or MRIs, but most are eventually resected, with a 30% rate of malignancy in these resected tumors. Survival 5 years after resection of an IPMN without malignancy is approximately 80%, 85% with malignancy but no lymph node spread and 0% with malignancy spreading to lymph nodes.^[6] Surgery can include the removal of the head of the pancreas (a pancreaticoduodenectomy), removal of the body and tail of the pancreas (a distal pancreatectomy), or rarely removal of the entire pancreas (a total pancreatectomy).^[7] In selected cases the surgery can be performed using minimally invasive techniques such as laparoscopy^[8] or robotic surgery. A study using Surveillance, Epidemiology, and End Result Registry (SEER) data suggested that increased lymph node counts harvested during the surgery were associated with better survival in invasive IPMN patients.^[9]

References

^ "Intraductal Papillary Mucinous Neoplasms of the Pancreas". Johns Hopkins University. Retrieved 7 September 2009.
^ "Pathology of Neoplasms of the Pancreas". Johns Hopkins University. Retrieved 7 September 2009.
^ Winter, JM (May 2006). "Periampullary and pancreatic incidentaloma: a single institution's experience with an increasingly common diagnosis.". Annals of Surgery 243 (5): 673–80. doi:10.1097/01.sla.0000216763.27673.97. PMC 1570557. PMID 16633003.
^ "Sol Goldman Pancreatic Cancer Research Center". Johns Hopkins University.
^ "Science Translational Medicine". AAAS.
^ Sohn, Taylor (June 2004). "Intraductal Papillary Mucinous Neoplasms of the Pancreas". Annals of Surgery 239 (6): 788–799. doi:10.1097/01.sla.0000128306.90650.aa. PMC 1356287. PMID 15166958.
^ "Pancreatic Surgery". Johns Hopkins University. Retrieved 7 September 2009.
^ "Laproscopic Pancreatic Surgery". Johns Hopkins University. Retrieved 7 September 2009.
^ Wu, WM (Sep 2014). "An increased total resected lymph node count benefits survival following pancreas invasive intraductal papillary mucinous neoplasms resection: an analysis using the surveillance, epidemiology, and end result registry database.". PLoS One 9 (9): e107962. doi:10.1371/journal.pone.0107962. PMC 4179272. PMID 25264746.

External links

The Toronto Video Atlas of Liver, Pancreas and Transplant Surgery – Video of Whipple procedure in IPMN case

UpToDate Contents

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1. 膵管内乳頭粘液性腫瘍（IPMN）：病態生理および臨床症状 intraductal papillary mucinous neoplasm of the pancreas ipmn pathophysiology and clinical manifestations
2. 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
3. 膵外分泌腫瘍の病理 pathology of exocrine pancreatic neoplasms
4. 膵嚢胞性腫瘍 pancreatic cystic neoplasms
5. 膵嚢胞の分類 classification of pancreatic cysts

English Journal

Epidemiology, diagnosis, and management of cystic lesions of the pancreas.

de Jong K, Bruno MJ, Fockens P.SourceDepartment of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands.
Gastroenterology research and practice.Gastroenterol Res Pract.2012;2012:147465. Epub 2011 Oct 11.
Although little is known on the true prevalence of pancreatic cysts, physicians are currently more frequently confronted with pancreatic cysts because of the increasing use of sophisticated cross-sectional abdominal imaging. Cystic lesions of the pancreas comprise of a heterogeneous group of diagnos
PMID 22007199

Somatic Mutations in PIK3CA and Activation of AKT in Intraductal Tubulopapillary Neoplasms of the Pancreas.

Yamaguchi H, Kuboki Y, Hatori T, Yamamoto M, Shiratori K, Kawamura S, Kobayashi M, Shimizu M, Ban S, Koyama I, Higashi M, Shin N, Ishida K, Morikawa T, Motoi F, Unno M, Kanno A, Satoh K, Shimosegawa T, Orikasa H, Watanabe T, Nishimura K, Harada Y, Furukawa T.Source*Institute for Integrated Medical Sciences ‡Institute of Gastroenterology §Department of Pathology, Tokyo Women's Medical University, Tokyo Departments of †Pathology ¶Surgery, Saitama International Medical Center, Saitama Medical University, Hidaka Departments of #Pathology **Hepato-Biliary-Pancreatic Surgery, Saitama Medical Center, Saitama Medical University, Kawagoe ∥Department of Pathology, Saiseikai Kawaguchi General Hospital, Kawaguchi ††Department of Pathology ‡‡Division of Hepato-Biliary Pancreatic Surgery, Tohoku University Hospital §§Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai Departments of ∥∥Pathology ¶¶Surgery, Tobu Chiiki Hospital, Tokyo ##Todachuo General Hospital, Toda, Japan.
The American journal of surgical pathology.Am J Surg Pathol.2011 Dec;35(12):1812-1817.
Intraductal tubulopapillary neoplasm (ITPN) is a recently recognized rare variant of intraductal neoplasms of the pancreas. Molecular aberrations underlying the neoplasm remain unknown. We investigated somatic mutations in PIK3CA, PTEN, AKT1, KRAS, and BRAF. We also investigated aberrant expressions
PMID 21945955

Japanese Journal

症例報告画像上拡大傾向を示した膵漿液性嚢胞腺腫の1例

中本充洋,皆川紀剛,山口幸二
Journal of UOEH 34(2), 201-206, 2012-06-01
NAID 40019348948

症例腫瘍出血を契機に発見された膵頭部浸潤性膵管内乳頭粘液性腫瘍(IPMN)の1例 (特集消化器最新画像情報2012)

橋本彩,高濱潤子,中込将弘 [他]
臨床放射線 57(5), 721-725, 2012-05
NAID 40019321199

Related Pictures

View full size version of IPMN IPMN vom Haupt- und Seitengang-Typ size version of IPMN - tail of pancreas Case Discussion: イメージ 1 IPMN

★リンクテーブル★

国試過去問	「108I065」「104A004」「105I019」「113A009」
リンク元	「膵嚢胞」「膵管内乳頭粘液性腫瘍」「粘液性嚢胞腫瘍」「膵管」「intraductal papillary mucnous neoplasm」
関連記事	「IP」「IPMNs」

「108I065」

　　[★]

58歳の男性。腹部CTで異常を指摘され来院した。 55歳時に自宅近くの医療機関で早期胃癌に対し幽門側胃切除術を受け、その後の定期検査の腹部 CTで異常を指摘され、紹介されて受診した。自覚症状はない。体温 36.2℃。脈拍 88/分、整。腹部は平坦、軟で、肝・脾を触知しない。血液所見：赤血球 415万、 Hb 13.6 g/dl、Ht 42%、白血球 5,800、血小板 22万。血液生化学所見：総ビリルビン 0.8 mg/dl、AST24 IU/l、ALT 32 IU/l、ALP 246 IU/l(基準 115～359)、 γ -GTP 44 IU/l(基準 8～50)、アミラーゼ 155 IU/l(基準 37～160)、 CEA 2.2 ng/ml(基準 5以下 )、 CA19-9 32 U/ml(基準 37以下 )。 CRP 0.1 mg/dl。MRCP(別冊 No. 26)を別に示す。
最も考えられるのはどれか。

[正答]

※国試ナビ4※　［108I064］←［国試_108］→［108I066］

「104A004」

　　[★]

膵管内乳頭粘液性腫瘍 IPMNの検査所見で誤っているのはどれか。

a　造影CT－全体に肥厚した被膜
b　内視鏡的逆行性胆管膵管造影 ERCP－膵管の拡張
c　磁気共鳴胆管膵管撮影 MRCP－ブドウの房状の形態
d　十二指腸内視鏡検査－Vater乳頭口開大
e　膵管鏡検査－イクラ状上皮

[正答]

※国試ナビ4※　［104A003］←［国試_104］→［104A005］

「105I019」

　　[★]

膵管内乳頭粘液性腫瘍(IPMN)で誤っているのはどれか。

a　主膵管内のイクラ状隆起性病変
b　主膵管のびまん性狭窄
c　膵管内の乳頭状増生
d　膵管分枝のブドウの房状拡張
e　Vater乳頭口の開大

[正答]

※国試ナビ4※　［105I018］←［国試_105］→［105I020］

「113A009」

　　[★]

膵管内乳頭粘液性腫瘍(IPMN)でみられないのはどれか。

a　Vater乳頭口の開大
b　膵管内の乳頭状増生
c　主膵管のびまん性狭窄
d　膵管分枝のブドウの房状拡張
e　主膵管内のイクラ状隆起性病変

[正答]

※国試ナビ4※　［113A008］←［国試_113］→［113A010］

「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

参考

1. [charged] 膵嚢胞性腫瘍 - uptodate [5]

造影CT

http://www.uptodate.com/contents/image?imageKey=GAST/7321

「膵管」

　　[★]

英: pancreatic duct (Z)
ラ: ductus pancreaticus
関: 膵臓、副膵管、主膵管

解剖

主膵管：ウィルズング管：ふつう膵管といえばこちらをさす。
腹膵管：サントリーニ管

膵管の拡張　→　膵管拡張

SRA.515

造影CTでは拡張した胆管は黒く抜けて見える
膵管全体が拡張蛇行：(1)慢性膵炎、(2)主膵管型の粘液産生腫瘍(膵管内乳頭粘液性腫瘍)
(3)膵石症：

慢性膵炎：膵石症は慢性膵炎を合併することがあり、主膵管の拡張をきたし、数珠状拡張をきたす。(YN.B)
主膵管型の粘液産生腫瘍：拡張が高度 or 壁から内宮に充実性腫瘍が濃染される場合

Henry Gray (1825-1861). Anatomy of the Human Body. 1918.

臨床関連

膵管拡張：慢性膵炎、膵管内乳頭粘液性腫瘍 IPMN、膵石症
膵管狭窄：自己免疫性膵炎

「intraductal papillary mucnous neoplasm」

　　[★] 膵管内乳頭粘液性腫瘍 IPMN

「IP」

　　[★]

「IPMNs」

　　[★] 膵管内乳頭粘液性腫瘍 intraductal papillary mucinous neoplasms

[1] "Intraductal Papillary Mucinous Neoplasms of the Pancreas". Johns Hopkins University. Retrieved 7 September 2009.

[2] "Pathology of Neoplasms of the Pancreas". Johns Hopkins University. Retrieved 7 September 2009.

[3] Winter, JM (May 2006). "Periampullary and pancreatic incidentaloma: a single institution's experience with an increasingly common diagnosis.". Annals of Surgery 243 (5): 673–80. doi:10.1097/01.sla.0000216763.27673.97. PMC 1570557. PMID 16633003.

[4] "Sol Goldman Pancreatic Cancer Research Center". Johns Hopkins University.

[5] "Science Translational Medicine". AAAS.

[6] Sohn, Taylor (June 2004). "Intraductal Papillary Mucinous Neoplasms of the Pancreas". Annals of Surgery 239 (6): 788–799. doi:10.1097/01.sla.0000128306.90650.aa. PMC 1356287. PMID 15166958.

[7] "Pancreatic Surgery". Johns Hopkins University. Retrieved 7 September 2009.

[8] "Laproscopic Pancreatic Surgery". Johns Hopkins University. Retrieved 7 September 2009.

[9] Wu, WM (Sep 2014). "An increased total resected lymph node count benefits survival following pancreas invasive intraductal papillary mucinous neoplasms resection: an analysis using the surveillance, epidemiology, and end result registry database.". PLoS One 9 (9): e107962. doi:10.1371/journal.pone.0107962. PMC 4179272. PMID 25264746.

匿名

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案内

IPMN

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Contents

History

Classification

Diagnosis

Treatment

See also

References

External links

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「108I065」

「104A004」

「105I019」

「113A009」

「膵嚢胞」

疫学

分類

検査

画像検査

治療

「膵管内乳頭粘液性腫瘍」

概念

分類と性質

病態

治療

予後

粘液性嚢胞腫瘍と膵管内乳頭粘液腫瘍

参考

国試

「粘液性嚢胞腫瘍」

分類

粘液性嚢胞腫瘍と膵管内乳頭粘液腫瘍

参考

「膵管」

解剖

膵管の拡張 → 膵管拡張

臨床関連

「intraductal papillary mucnous neoplasm」

「IP」

「IPMNs」

膵管の拡張　→　膵管拡張