今日の臨床サポート

膵管内乳頭粘液性腫瘍、粘液性嚢胞腫瘍

著者: 鈴木裕1) 杏林大学 消化器・一般外科

著者: 杉山政則2) 東京労災病院 院長

監修: 下瀬川徹 みやぎ県南中核病院企業団

著者校正/監修レビュー済:2021/01/20
参考ガイドライン:
  1. 国際膵臓学会(IEP):IPMN国際診療ガイドライン2017
患者向け説明資料

概要・推奨   

  1. 分枝膵管型IPMNの手術適応の決定に最も重要なのは嚢胞内の壁在結節である。壁在結節は有無のみでなく、その径も重要である(推奨度1)
  1. MCNは比較的予後良好な腫瘍であり、壁在結節などの悪性を示唆する所見があれば定型手術が必要であるが、それ以外は縮小手術が可能である(推奨度1)
  1. IPMNには通常型膵癌の合併が多く、IPMNのみでなく膵全体の精査が必要である(推奨度1)
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薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
鈴木裕 : 特に申告事項無し[2021年]
杉山政則 : 特に申告事項無し[2021年]
監修:下瀬川徹 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 定期レビューを行い、細かな文言について修正を行った。

病態・疫学・診察

疾患情報(疫学・病態)  
膵管内乳頭粘液性腫瘍(IPMN):
  1. IPMNとは膵管内に乳頭状に発育し、多量の粘液を産生、膵管拡張を来す疾患である。内視鏡上は、乳頭の開大と粘液排出が特徴である。IPMNは主膵管型と分枝膵管型に分類され、主膵管型では主膵管のびまん性拡張像、分枝膵管型では分枝膵管の嚢胞状拡張像(ブドウの房状)が特徴である。IPMNは壮年~高年男性の膵頭部に好発し、膵管との交通を認め、膵管内を進展し、随伴性膵炎を高率に合併する。
 
IPMNの乳頭像

乳頭は開大し、粘液の排出を認める。

出典

img1:  著者提供
 
 
 
主膵管型IPMN(ERCP)

主膵管は著明に拡張し、壁在結節が陰影欠損像として描出されている。

出典

img1:  著者提供
 
 
 
分枝膵管型IPMN(MRCP)

膵頭部にブドウの房状の嚢胞性病変を認めるも、主膵管の拡張は認めない。

出典

img1:  著者提供
 
 
 
膵粘液性嚢胞腫瘍(MCN):
  1. MCNは共通の線維性被膜を有する巨大単房性・多房性腫瘍(夏ミカン状)である。
  1. MCNは中年女性の膵体尾部に発生し、膵管の異常は認めないことが多い。
  1. MCNは病理組織学的にエストロゲンレセプターやプロゲステロンレセプターに染色される卵巣様間質を伴う。
 
MCN(MRCP)

膵体尾部に夏ミカン状の嚢胞性病変を認め、内部に小嚢胞の集簇がみられる(cyst in cyst)。

出典

img1:  著者提供
 
 
 
MCN病理組織像(H.E.染色)

粘液産生性の円柱状の腫瘍細胞に裏打ちされ、腫瘍細胞直下には卵巣様間質( “ovarian-type” stroma, OS)を認める。

出典

img1:  著者提供
 
 
問診・診察のポイント  
  1. IPMNは37%[1]、MCNは40%[1]に症状を認め、症状は腹痛、腰背部痛が多い。
  1. IPMNは63%が無症状であり、他疾患の精査中や人間ドックなどで発見される場合も少なくない[1]
  1. IPMNには消化器癌、特に胃癌や大腸癌の合併も多いため、上下部消化管のスクリーニング検査が必要である。

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文献 

著者: Yutaka Suzuki, Yutaka Atomi, Masanori Sugiyama, Shuji Isaji, Kazuo Inui, Wataru Kimura, Makoto Sunamura, Toru Furukawa, Akio Yanagisawa, Jo Ariyama, Tadahiro Takada, Hidenobu Watanabe, Koichi Suda, Japanese multiinstitutional study of intraductal papillary mucinous tumor and mucinous cystic tumor
雑誌名: Pancreas. 2004 Apr;28(3):241-6.
Abstract/Text The Japan Pancreas Society performed a multiinstitutional, retrospective study of 1379 cases of intraductal papillary mucinous tumor (IPMT) and 179 cases of mucinous cystic tumor (MCT) of the pancreas. Clinicopathologic features and postoperative long-term outcomes were investigated. IPMT were most frequently found in men and in the head of the pancreas. In contrast, all patients with MCT were women. Ovarian-type stroma were found in only 42.2% of the MCT cases. Prognostic indicators of malignant IPMT included advanced age, positive symptoms, abundant mucous secretion, presence of large nodules and/or large cysts, remarkable dilatation of the main pancreatic duct, and main duct- or combined-type IPMT. Advanced age, positive symptoms, and presence of large nodules and/or large cysts were predictive of malignant MCT. The 5-year survival rate of IPMT patients was 98%-100% in adenoma to noninvasive carcinoma cases, 89% in minimally invasive carcinoma cases, and 57.7% in invasive carcinoma cases. The 5-year survival rate of MCT patients was 100% in adenoma to minimally invasive carcinoma cases and 37.5% in invasive carcinoma cases. In conclusion, IPMT and MCT show distinct clinicopathologic and prognostic differences. The results from this study may contribute to the diagnosis and treatment of IPMT and MCT.

PMID 15084964  Pancreas. 2004 Apr;28(3):241-6.
著者: M Sugiyama, Y Atomi, J Hachiya
雑誌名: Am J Gastroenterol. 1998 Feb;93(2):156-9. doi: 10.1111/j.1572-0241.1998.00156.x.
Abstract/Text OBJECTIVE: We analyzed the findings of intraductal papillary tumors of the pancreas by magnetic resonance cholangiopancreatography (MRCP).
METHODS: Twelve patients with intraductal papillary tumors (main duct type, n = 3; branch duct type, n = 8; combined type, n = 1) underwent endoscopic retrograde cholangiopancreatography (ERCP) (n = 11) and MRCP, using half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences (n = 12). Imaging findings were compared with operative and pathological findings in all the patients.
RESULTS: Although ERCP failed to fully depict the main pancreatic duct and cystic tumors in six of 11 patients because of copious intraductal mucin, MRCP provided complete images of these structures in all 12 patients. In main duct type tumors, MRCP demonstrated moderate-marked, diffuse dilation of the main pancreatic duct. Branch duct type tumors showed "grape-like" clusters of cysts with no or only mild, diffuse dilation of the main duct. In combined type tumors, MRCP demonstrated a markedly dilated main duct with a large unilocular cyst of the collateral duct. MRCP detected more mural nodules (75%, three of four patients) than ERCP (25%, one of four). MRCP allowed more precise diagnosis of the type, size, and extent of tumors than ERCP, as confirmed by pathologic findings.
CONCLUSION: MRCP demonstrates intraductal papillary tumors less invasively and more completely than ERCP.

PMID 9468232  Am J Gastroenterol. 1998 Feb;93(2):156-9. doi: 10.1111/・・・
著者: M Sugiyama, Y Atomi, M Saito
雑誌名: Gastrointest Endosc. 1998 Aug;48(2):164-71.
Abstract/Text BACKGROUND: We retrospectively analyzed the endoscopic ultrasonographic characteristics of intraductal papillary tumors of the pancreas and identified signs indicative of malignant tumors.
METHODS: Twenty-eight patients with intraductal tumors and 38 with other pancreatic cystic lesions underwent endoscopic ultrasonography, conventional ultrasonography, and endoscopic retrograde cholangiopancreatography (ERCP).
RESULTS: Intraductal tumors were classified into three subtypes, and endoscopic ultrasonographic characteristics were assessed. In main duct type tumors, the main pancreatic duct showed a segmental or diffuse moderate-to-marked dilatation, often associated with intraductal nodules. Pancreatic parenchymal atrophy was frequently recognized. Branch duct type tumors consisted of multiple 5 to 20 mm cysts with the appearance of a cluster of grapes. The main duct was mildly dilated or nondilated. Combined type tumors had features of both main duct and branch duct types. Endoscopic ultrasonography (92%) differentiated pancreatic cystic lesions more precisely than ultrasonography (82%) and ERCP (89%). Main duct or combined type tumors, large tumors (> or = 30 mm for branch duct type), marked dilatation of the main pancreatic duct (> or = 10 mm for other types), and mural nodules suggested malignancy. Endoscopic ultrasonography demonstrated these signs more accurately than ultrasonography or ERCP.
CONCLUSION: Endoscopic ultrasonography is useful for diagnosing intraductal papillary tumors, particularly for predicting malignancy.

PMID 9717782  Gastrointest Endosc. 1998 Aug;48(2):164-71.
著者: Koji Yamaguchi, Shuichi Kanemitsu, Takashi Hatori, Hiroyuki Maguchi, Yasuhiro Shimizu, Minoru Tada, Toshio Nakagohri, Keiji Hanada, Manabu Osanai, Yutaka Noda, Akihiko Nakaizumi, Toru Furukawa, Shinichi Ban, Bunsei Nobukawa, Yo Kato, Masao Tanaka
雑誌名: Pancreas. 2011 May;40(4):571-80. doi: 10.1097/MPA.0b013e318215010c.
Abstract/Text OBJECTIVES: Pancreatic ductal adenocarcinoma (PDAC) may derive from an intraductal papillary mucinous neoplasm (IPMN) of the pancreas or may develop in the pancreatic duct apart from IPMN. The purpose of this study was to define the clinicopathological features of these 2 entities and compare them with those of ordinary PDAC.
METHODS: Of 765 patients who had surgical resection for IPMN, 122 were diagnosed as having PDAC derived from IPMN and 31 with PDAC concomitant with IPMN. In addition, 7605 patients with PDAC who were registered in the Japan Pancreas Society pancreatic cancer registry were compared with the above patients.
RESULTS: Pancreatic ductal adenocarcinomas derived from IPMN and concomitant with IPMN were significantly smaller, less invasive, and less extensive than ordinary PDAC. The median survival of patients with the 2 conditions was significantly longer than for those with ordinary PDAC when compared overall or when limited to TS2 (2.0 cm < tumor size ≤ 4.0 cm) or TS3 (4.0 cm < tumor size ≤ 6.0 cm) cases.
CONCLUSIONS: These findings suggest that PDAC concomitant with IPMN and PDAC derived from IPMN may have more favorable biological behaviors or be diagnosed earlier than ordinary PDAC.

PMID 21499212  Pancreas. 2011 May;40(4):571-80. doi: 10.1097/MPA.0b013・・・
著者: Masao Tanaka, Carlos Fernández-Del Castillo, Terumi Kamisawa, Jin Young Jang, Philippe Levy, Takao Ohtsuka, Roberto Salvia, Yasuhiro Shimizu, Minoru Tada, Christopher L Wolfgang
雑誌名: Pancreatology. 2017 Sep - Oct;17(5):738-753. doi: 10.1016/j.pan.2017.07.007. Epub 2017 Jul 13.
Abstract/Text The management of intraductal papillary mucinous neoplasm (IPMN) continues to evolve. In particular, the indications for resection of branch duct IPMN have changed from early resection to more deliberate observation as proposed by the international consensus guidelines of 2006 and 2012. Another guideline proposed by the American Gastroenterological Association in 2015 restricted indications for surgery more stringently and recommended physicians to stop surveillance if no significant change had occurred in a pancreatic cyst after five years of surveillance, or if a patient underwent resection and a non-malignant IPMN was found. Whether or not it is safe to do so, as well as the method and interval of surveillance, has generated substantial debate. Based on a consensus symposium held during the meeting of the International Association of Pancreatology in Sendai, Japan, in 2016, the working group has revised the guidelines regarding prediction of invasive carcinoma and high-grade dysplasia, surveillance, and postoperative follow-up of IPMN. As the working group did not recognize the need for major revisions of the guidelines, we made only minor revisions and added most recent articles where appropriate. The present guidelines include updated information and recommendations based on our current understanding, and highlight issues that remain controversial or where further research is required.

Copyright © 2017 IAP and EPC. Published by Elsevier B.V. All rights reserved.
PMID 28735806  Pancreatology. 2017 Sep - Oct;17(5):738-753. doi: 10.10・・・
著者: Hiroyuki Maguchi, Satoshi Tanno, Nobumasa Mizuno, Keiji Hanada, Go Kobayashi, Takashi Hatori, Yoshihiko Sadakari, Taketo Yamaguchi, Kosuke Tobita, Ryuichiro Doi, Akio Yanagisawa, Masao Tanaka
雑誌名: Pancreas. 2011 Apr;40(3):364-70. doi: 10.1097/MPA.0b013e31820a5975.
Abstract/Text OBJECTIVE: The aim of this study was to evaluate the long-term follow-up results of patients with branch duct intraductal papillary mucinous neoplasms (BD-IPMNs) without mural nodules (MNs) at 10 representative institutions in Japan.
METHODS: We analyzed 349 follow-up BD-IPMN patients who had no MNs on endoscopic ultrasonography at initial diagnosis.
RESULTS: Observation periods ranged from 1 to 16.3 years (median, 3.7 years). Sixty-two (17.8%) patients exhibited disease progression during follow-up. Twenty-two underwent surgery, leading to a pathological diagnosis of carcinoma in 9 and adenoma in 13. Although the remaining 287 (82.2%) showed no changes, 7 underwent surgery because of symptoms (n = 2), choice (n = 2), or development of pancreatic ductal adenocarcinoma (n = 3); all of them were diagnosed pathologically as adenomas. Of the 29 patients undergoing surgery, all 9 with carcinoma exhibited signs of progression, such as increased main pancreatic duct diameter and/or appearance of MNs. Pancreatic ductal adenocarcinomas and additional BD-IPMNs developed in 7 (2.0%) and 13 (3.7%), respectively. Overall, 320 (91.7%) patients were followed without surgery.
CONCLUSIONS: Most BD-IPMN patients who had no MNs on endoscopic ultrasonography could be managed without surgery. However, careful attention should be paid to disease progression and the development of pancreatic ductal adenocarcinomas during follow-up.

PMID 21289527  Pancreas. 2011 Apr;40(3):364-70. doi: 10.1097/MPA.0b013・・・
著者: Kenji Yamao, Akio Yanagisawa, Kuniyuki Takahashi, Wataru Kimura, Ryuichiro Doi, Noriyoshi Fukushima, Nobuyuki Ohike, Michio Shimizu, Takashi Hatori, Bunsei Nobukawa, Michio Hifumi, Yuji Kobayashi, Kosuke Tobita, Satoshi Tanno, Masanori Sugiyama, Yoshihiro Miyasaka, Toshio Nakagohri, Taketo Yamaguchi, Keiji Hanada, Hideki Abe, Minoru Tada, Naotaka Fujita, Masao Tanaka
雑誌名: Pancreas. 2011 Jan;40(1):67-71. doi: 10.1097/MPA.0b013e3181f749d3.
Abstract/Text OBJECTIVE: The aim of this study was to elucidate the clinicopathological features and prognosis of mucinous cystic neoplasms (MCNs).
MATERIALS AND METHODS: We performed a multi-institutional, retrospective study on a collected series of patients with MCN pathologically defined by ovarian-type stroma. Clinicopathological features and prognosis were investigated.
RESULT: Mucinous cystic neoplasm was confirmed in 156 cases, including 129 adenomas (82.7%) and 21 noninvasive (13.4%) and 6 invasive carcinomas (3.9%). Patients with MCN were exclusively women (98.1%) with the mean age of 48.1 years. All but 1 MCN were in the pancreatic body/tail region with a mean size of 65.3 mm. Communication between the cyst and the pancreatic duct was found in 18.1%. The 3-, 5-, and 10-year survival rates were 97.6%, 96.6%, and 96.6%, respectively. A significant difference in the survival rates was observed between adenomas and carcinomas and between minimally invasive carcinomas and invasive carcinomas. Cyst diameter and presence of mural nodule were predictive of malignant MCN.
CONCLUSIONS: Mucinous cystic neoplasm is a rare but distinctive pancreatic cystic neoplasm with a favorable overall prognosis. All MCNs should be resected to prevent malignant changes but can be observed for an appropriate time when the lesion is small without the presence of mural nodules.

PMID 20924309  Pancreas. 2011 Jan;40(1):67-71. doi: 10.1097/MPA.0b013e・・・
著者: M Sugiyama, Y Atomi, A Kuroda
雑誌名: Surgery. 1997 Sep;122(3):617-25.
Abstract/Text BACKGROUND: This study focuses on clinicopathologic, imaging, and prognostic differences between two types of mucin-producing cystic tumors of the pancreas, with the aim of appropriate management of these tumors.
METHODS: Forty-six patients with mucin-producing cystic tumors underwent operation. The types of tumors were as follows: mucinous cystic neoplasm, adenoma (6) and adenocarcinoma (12); intraductal papillary tumor, adenoma (10) and adenocarcinoma (18).
RESULTS: Gender, age, symptoms, signs, tumor location and size, and the presence or absence of communication with the pancreatic duct differed between the two types. Mucinous cystadenocarcinomas showed deep invasion more often than intraductal papillary adenocarcinomas. Lymph node involvement was seen in 58% of mucinous cystadenocarcinomas but in only 22% of intraductal papillary adenocarcinomas. Tumors with mural nodules tended to show deep invasion and nodal metastasis. All four intraductal papillary tumors smaller than 3 cm without mural nodules were adenomas. Imaging studies allowed accurate differentiation between the two types but not between adenomas and adenocarcinomas. Five-year survival rates for patients with adenomas, mucinous cystadenocarcinomas, and intraductal papillary adenocarcinomas were 100%, 33%, and 81%, respectively.
CONCLUSIONS: Mucinous cystic neoplasm necessitates complete tumor excision with wide dissection of lymph nodes including paraaortic nodes. Intraductal papillary tumor requires only peripancreatic node dissection; for tumors smaller than 3 cm without mural nodules, node dissection may be unnecessary.

PMID 9308621  Surgery. 1997 Sep;122(3):617-25.

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