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02 September 2021: Articles  Japan

Pancreatic Ductal Adenocarcinoma Concomitant with Main Duct Type Intraductal Papillary Mucinous Neoplasm of the Pancreas: A Case Report

Rare coexistence of disease or pathology

Keisuke Yonamine1E*, Shinsuke Koshita1E, Yoshihide Kanno ORCID logo1E, Takahisa Ogawa1E, Hiroaki Kusunose1E, Toshitaka Sakai1E, Kazuaki Miyamoto1E, Fumisato Kozakai1E, Haruka Okano1E, Takaho Okada2E, Masaya Oikawa2E, Takashi Tsuchiya2E, Takashi Sawai3E, Yutaka Noda13E, Kei Ito1E

DOI: 10.12659/AJCR.932565

Am J Case Rep 2021; 22:e932565

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Abstract

BACKGROUND: Intraductal papillary mucinous neoplasm of the pancreas (IPMN) and pancreatic ductal adenocarcinoma (PDAC) often coexist in the same pancreas. Almost all IPMNs involving PDACs concomitant with IPMN have been shown to be branch duct type IPMNs (BD-IPMNs), and their histological subtypes are gastric type. Therefore, PDACs concomitant with main duct type IPMNs (MD-IPMNs) are considered to be rare. We herein report a rare case preoperatively diagnosed as being a PDAC concomitant with MD-IPMN on the basis of imaging findings and histological findings of pancreatic specimens endoscopically obtained from 2 lesions.

CASE REPORT: A 67-year-old man was referred to our hospital due to an enlarged pancreas. Using imaging studies, a solid mass was found in the pancreatic head and intraductal papillary masses in the dilated main pancreatic duct of the body and tail with a fistula in the duodenum. On the basis of histological results using specimens endoscopically obtained from each of the 2 lesions, total pancreatectomy was planned due to suspected PDAC concomitant with an MD-IPMN. Finally, resected specimens were used to confirm the presence of a rare case of PDAC concomitant with MD-IPMN.

CONCLUSIONS: We encountered a rare case of a PDAC concomitant with an MD-IPMN which could be preoperatively diagnosed by using imaging studies and histological specimens endoscopically obtained. In addition to invasive cancers derived from IPMNs, PDACs concomitant with IPMNs can rarely develop in the pancreas involving MD-IPMNs.

Keywords: Carcinoma, Pancreatic Ductal, Endoscopy, Gastrointestinal, Pancreatectomy, Pancreatic Intraductal Neoplasms, Adenocarcinoma, Mucinous, Aged, Humans, Pancreas, Pancreatic Ducts, Pancreatic Neoplasms

Background

Intraductal papillary mucinous neoplasm of the pancreas (IPMN) and pancreatic ductal adenocarcinoma (PDAC) often coexist in the same pancreas. When these 2 pancreatic lesions can be separated by using imaging studies, histology, and genetic analyses, PDACs like this are defined as PDAC concomitant with IPMN [1]. On the other hand, when a PDAC is histologically or genetically proven to originate in an IPMN adjacent to the PDAC, this PDAC is defined to be derived from the IPMN [1]. From previous reports, almost all IPMNs involving PDACs concomitant with IPMN have been shown to be branch duct type IPMNs (BD-IPMNs), and their histological subtypes are gastric type [2–4]. Therefore, PDACs concomitant with main duct type IPMNs (MD-IPMNs) are considered to be rare. We herein report a rare case preoperatively diagnosed as being a PDAC concomitant with MD-IPMN on the basis of imaging findings and histological findings of pancreatic specimens endoscopically obtained from 2 lesions.

Case Report

A 67-year-old asymptomatic man was referred to our medical center because his primary care physician detected the enlargement of the pancreas by using screening tests, including abdominal ultrasonography and computed tomography (CT). His past medical history included ulcerative colitis, atrial fibrillation and hypertension. He had a habit of smoking (a pack per day for 45 years) and drinking (pure alcohol of >72 g per day). He had no family history of pancreatic cancer. For laboratory data on admission, the serum levels of liver and pancreatic enzymes, except gamma glutamyl transpeptidase (64 IU/L), were within normal ranges. Serum carbohydrate antigen 19-9 and carcinoembryonic antigen levels were slightly elevated (54.8 and 5.4 IU/L, respectively). By using contrast-enhanced CT scan, a mass with a size of 30 mm in the pancreatic head was heterogeneously enhanced at the portal/ late phases. In addition, a main pancreatic ductal (MPD) dilation with intraductal air bubbles was detected in the pancreatic body and tail, suggesting the formation of fistula in the third part of the duodenum. Magnetic resonance imaging (MRI) showed the signal intensity of the mass lesion in the pancreatic head was low in both T1- and T2-weighted images, and there was a decrease in the apparent diffusion coefficient values in diffusion-weighted images (Figure 1). On the other hand, we detected the main pancreatic duct dilation with a maximum diameter of 16 mm in the pancreatic body and tail on T2-wighted image and a decrease in apparent diffusion coefficient values on diffusion-weighted image (Figure 2). By using endoscopic ultrasonography (EUS), a 30-mm low-echoic mass with a heterogeneous internal echo was visualized in the head of the pancreas, whereas papillary protrusions with a maximum height of 10 mm filled in the dilated MPD of the pancreatic body and tail. Although the intraductal lesions in the pancreatic body and tail were considered to be an MD-IPMN penetrating into the duodenum by using the above-mentioned imaging findings, the mass lesion in the pancreatic head was suspected of being a PDAC that did not originate from the possible MD-IPMN lesion due to lack of the continuity between the 2 lesions shown by imaging studies.

To obtain histological evidence for these 2 lesions, EUS-guided fine-needle aspiration (EUS-FNA) for the mass lesion in the pancreatic head was performed by using a 22-G needle (EZ shot 3™: Olympus, Tokyo, Japan). The specimens collected by using EUS-FNA were processed using a cell-block method [5] and were subjected to hematoxylin and eosin (HE) staining and immunostaining. From histological diagnosis of the specimens, the lesions were suspected to be adenocarcinoma. In addition, we performed duodenoscopy by using a short single-balloon enteroscope (SIF-290: Olympus Co., Tokyo, Japan) to obtain specimens from the intraductal lesions in the pancreatic body and tail through the fistula in the third portion of the duodenum. Under duodenoscopy guidance, the fistula with mucus discharge was endoscopically detected in the 3rd portion of the duodenum, and the tip of the enteroscope could be directly inserted into the MPD via this fistula. We could endoscopically observe papillary protrusions filling the inside of the MPD and performed biopsies on these intraductal lesions. By using HE staining, the biopsy specimens were found to include atypical epithelial cell clusters with papillary structure, which were histologically determined to have IPMN with high-grade dysplasia of an intestinal type on the basis of the presence of cells, which were positive for MUC2 and CDX2 stainings and negative for MUC1 staining and had a high Ki-67 labeling index of 30%. Based on these findings, the patient underwent total pancreatectomy due to the diagnoses of a PDAC in the pancreatic head and an MD-IPMN in the pancreatic body and tail. At this time, the transverse colon was partially resected due to the adhesion to the pancreas caused by colonic diverticulitis.

In the cross-section of the resected specimens, a white mass with a size of 45 mm was macroscopically identified in the pancreatic head, whereas the dilated MPD of the pancreatic body and tail was filled with papillary masses. Macroscopically, these 2 lesions were separated. In addition, the pancreatoduodenal fistula covered by mucus was detected in the third part of the duodenum. From histological findings of the resected specimens, a well-differentiated tubular adenocarcinoma occupied the pancreatic head (Figure 3). On the other hand, in the pancreatic body and tail, the inside of the MPD was packed with atypical papillary epithelium (Figure 4). From immunostaining findings, the atypical papillary epithelium was shown to be positive for MUC2 and CDX2 stainings and to have a high Ki-67 labeling index of 30%, resulting in a histological diagnosis of an MD-IPMN with high-grade dysplasia and an intestinal histological subtype. No other IPMN lesions were observed in the resected pancreas. Since both malignant lesions were found to be separated histologically, the patient was diagnosed as having a PDAC (fStage IIB) concomitant with a malignant MD-IPMN (fStage 0).

Discussion

IPMNs have recently been shown to be a risk factor for PDACs in addition to malignant transformation of IPMN itself [6–8]. From a systematic review conducted by Choi et al, pancreatic malignancies, including PDACs, developed in almost 8% of the cases of low-risk IPMNs (those defined as being without MPD involvements or mural nodules) and in almost 25% of cases of high-risk IPMNs (those defined to have MPD involvement and/or mural nodules) within 10 years [9]. In other words, pancreatic malignancies can develop even in the pancreas involving IPMNs unlikely to develop malignant transformations of the IPMNs themselves. In 2009, Yamaguchi et al proposed the concept of “PDACs concomitant with IPMNs” by analyzing 765 patients who underwent surgery for IPMNs [10]. In that study, PDACs concomitant with IPMNs were defined as lacking histological transitions between PDAC and IPMN lesions and were discriminated from invasive cancers derived from IPMNs. In addition, the study indicated the following histo-logical characteristics of PDACs concomitant with IPMNs by comparing those of invasive cancers derived from IPMNs: 30% of invasive cancers derived from IPMNs were colloid carcinomas, whereas all PDACs concomitant with IPMNs were tubular adenocarcinomas.

Based on the international consensus guidelines revised in 2017, IPMNs are morphologically classified into 3 types on the basis of imaging and/or histological findings: MD-IPMN, BD-IPMN, and mixed type IPMN [1]. MD-IPMNs can be determined from those 3 types when all the following imaging findings are met: 1) segmental or diffuse MPD dilation of ≥5 mm without being caused by other diseases, 2) branch duct dilation of <5 mm, and 3) the findings indicative of mucus production or the detection of mural nodules in the dilated pancreatic duct. With regard to the morphological type of IPMNs involving PDACs, Ingkakul et al reported that 22 of 236 patients who underwent surgery for their IPMNs could be histologically diagnosed with PDACs concomitant with IPMNs and that the morphological type of involved IPMNs was a BD-IPMN for all 22 patients [3]. This tendency applies to a case series related to PDACs concomitant with IPMNs reported by Mandai et al [4]. In addition, from the histological database of our medical center between January 1985 and December 2016, a total of 160 patients who underwent pancreatic surgery and were histo-logically found to have PDACs were identified. Although 47 of those patients were determined to have PDACs concomitant with IPMNs, all the coexisting IPMNs in the pancreas involving PDACs were branch duct type (unpublished date). In contrast, Yamaguchi et al have reported that 50% of patients (61/122) with PDACs derived from IPMNs had MD-IPMNs [10]. In addition, some reports have described the relationship between pancreatic malignancies associated with IPMNs and histological subtypes of those IPMNs [2,11,12]. From a report by Ideno et al, 16 of 110 patients who underwent surgery for their IPMNs were PDAC concomitant with IPMN and the histo-logical subtype of those IPMN were all gastric type [2], whereas Furukawa et al found that approximately 30% of invasive cancers derived from IPMNs developed in IPMNs with an intestinal type [11]. From the viewpoint of the morphological and histological types of background IPMNs involving pancreatic malignancies, those results may support that those 2 types of pancreatic malignancies are different in their pathogeneses.

Unlike the above-mentioned reports, a PDAC in the pancreatic head of this patient was histologically shown to develop apart from an MD-IPMN in the same pancreas. To the best of our knowledge, there are no case reports or clinical studies involving a case of PDAC concomitant with MD-IPMN. Although it may be rare that PDAC and MD-IPMN lesions separately develop in the same pancreas, it may be due to a very low frequency of developing MD-IPMNs compared with that of developing BD-IPMNs or mixed-type IPMNs. In any case, it is meaningful for clinical practice to recognize that PDACs concomitant with IPMNs can occasionally develop even in the pancreas involving MD-IPMNs. In other words, it may be necessary to investigate whether or not PDAC lesions are involved, even in the pancreas where MD-IPMNs develop before determining the surgical procedures for MD-IPMN lesions.

In addition, this case is meaningful because preoperative diagnoses of those 2 separate pancreatic diseases have been determined using imaging findings and specimens obtained endoscopically. When 2 or more pancreatic lesions, including pancreatic mass lesions and IPMNs, coexist in the same pancreas, preoperative evaluations for IPMNs may become important determinants for surgical procedures because IPMNs are sometimes difficult to determine to be suitable for surgery only by using imaging findings. In such cases, endoscopic approaches to obtain histocytological specimens for IPMNs may be effective for determining treatment plans for pancreatic diseases [13–17]. Although surgery is generally indicated when an MD-IPMN lesion has mural nodules on the basis of imaging findings, endoscopic approaches to obtain histocytological specimens may be necessary for some patients having MDIPMNs with mural nodules, like elderly patients and patients with several comorbidities, because approximately 40% of MD-IPMNs with mural nodules have been shown to be benign [15]; therefore, an MD-IPMN lesion with mural nodules may not be a prognostic factor for some patients with a high risk for surgery [18]. For this case, since total pancreatectomy, which was indicated for the patient on the basis of imaging findings, is a highly-invasive surgical procedure, endoscopic tissue acquisitions were performed at the 2 pancreatic lesions separately to determine whether or not total pancreatectomy should be performed.

Conclusions

We encountered a rare case of a PDAC concomitant with an MD-IPMN which could be preoperatively diagnosed by using imaging studies and histological specimens endoscopically obtained. In addition to invasive cancers derived from IPMNs, PDACs concomitant with IPMNs can rarely develop in the pancreas involving MD-IPMNs.

References:

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2.. Ideno N, Ohtsuka T, Matsunaga T, Clinical significance of GNAS mutation in intraductal papillary mucinous neoplasm of the pancreas with concomitant pancreatic ductal adenocarcinoma: Pancreas, 2015; 44; 311-20

3.. Ingkakul T, Sadakari Y, Ienaga J, Predictors of the presence of concomitant invasive ductal carcinoma in intraductal papillary mucinous neoplasm of the pancreas: Ann Surg, 2010; 251; 70-75

4.. Mandai K, Uno K, Yasuda K, Pancreatic ductal adenocarcinoma concomitant with intraductal papillary mucinous neoplasm: A report of 8 cases: Clin J Gastroenterol, 2013; 6; 248-54

5.. Noda Y, Fujita N, Kobayashi G, Diagnostic efficacy of the cell block methods in comparison with smear cytology of tissue samples obtained by endoscopic ultrasound-guided fine-needle aspiration: J Gastroenterol, 2010; 45; 868-75

6.. Yamaguchi K, Ohuchida J, Ohtsuka T, Intraductal papillary-mucinous tumor of the pancreas concomitant with ductal carcinoma of the pancreas: Pancreatology, 2002; 2; 484-90

7.. Tanno S, Nakano Y, Sugiyama Y, Incidence of synchronous and meta-chronous pancreatic carcinoma in 168 patients with branch duct intraductal papillary mucinous neoplasm: Pancreatology, 2010; 10; 173-78

8.. Oyama H, Tada M, Takagi K, Long-term risk of malignancy in branch-duct intraductal papillary mucinous neoplasms: Gastroenterology, 2020; 158; 226-37

9.. Choi SH, Park SH, Kim KW, Progression of unresected intraductal papillary mucinous neoplasms of the pancreas to cancer: A systematic review and meta-analysis: Clin Gastroenterol Hepatol, 2017; 15; 1509-20

10.. Yamaguchi K, Kanemitsu S, Hatori H, Pancreatic ductal adenocarcinoma derived from IPMN and pancreatic ductal adenocarcinoma concomitant with IPMN: Pancreas, 2011; 40; 571-80

11.. Furukawa T, Hatori T, Fujita I, Prognostic relevance of morphological types of intraductal papillary mucinous neoplasms of the pancreas: Gut, 2011; 60; 509-16

12.. Mino-Kenudson M, Femandez-del Castillo C, Baba Y, Prognosis of invasive intraductal papillary mucinous neoplasm depends on histological and precursor epithelial subtypes: Gut, 2011; 60; 1712-20

13.. Koshita S, Noda Y, Ito K, Pancreatic juice cytology with immunohistochemistry to detect malignancy and histologic subtypes in patients with branch duct type intraductal papillary mucinous neoplasms of the pancreas: Gastrointest Endosc, 2017; 85; 1036-46

14.. Koshita S, Noda Y, Kanno Y, Value of repeated cytology for intraductal papillary mucinous neoplasms of the pancreas with high risk potential of malignancy: Is it a promising method for monitoring a malignant transformation?: Pancreatology, 2020; 20; 1164-74

15.. Masaki Y, Koshita S, Noda Y, Should we regard all main duct type intraductal papillary mucinous neoplasm of pancreas (MD-IPMN) as an indication of surgery? A retrospective study in 29 patients with MD-IPMN showing mural nodules: Pancreatology, 2019; 19; 352-59

16.. Ogura T, Masuda D, Kurisu Y, Potential predictors of disease progression for main-duct intraductal papillary mucinous neoplasms of the pancreas: J Gastroenterol Hepatol, 2013; 28; 1782-86

17.. Takuma K, Kamisawa T, Anjiki H, Predictors of malignancy and natural history of main-duct intraductal papillary mucinous neoplasms of the pancreas: Pancreas, 2011; 40; 371-75

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923