A case of intraductal papillary-mucinous neoplasm of the pancreas penetrating into the stomach and spleen successfully treated by total pancreatectomy
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Intraductal papillary-mucinous neoplasms (IPMNs) are potentially malignant intraductal epithelial neoplasms that sometimes penetrate into other organs. To the best of our knowledge, no report has yet described a case with penetration into the spleen. We recently encountered a case of IPMN with penetration of the stomach and spleen that was successfully treated by total pancreatectomy.
A 70-year-old female visited our hospital with a complaint of fever and abdominal pain. Contrast-enhanced computed tomography (CT) revealed dilatation of the main pancreatic duct in the entire pancreas and penetration into the stomach and spleen. Upper gastrointestinal endoscopy revealed mucin extruding from four openings of the fistula in the stomach. No malignancy was detected based on cytology of the mucin. Inflammation markers and tumor markers (CEA, CA19–9) were elevated in the blood. The pre-operative diagnosis was IPMN of main pancreatic duct type penetrating into the stomach and spleen. A total pancreatectomy and splenectomy were performed, combined with distal gastrectomy including resection of the fistulas between the pancreas and stomach. No postoperative complications were noted. Histopathological examination of the resected specimen revealed atrophy of the pancreatic parenchyma, and the main duct of the pancreas was filled with mucin. Mucin-producing malignant tumor cells were detected in the epithelium of the main pancreatic duct with no signs of invasion. No malignancy was found at the fistulas between the pancreas and stomach or spleen. The patient was finally diagnosed with non-invasive intraductal papillary-mucinous carcinoma (IPMC) of main pancreatic duct type. Mechanical penetration was suspected as a mechanism of the penetration. The patient remained disease-free without evidence of recurrence more than 15 months after the operation.
Though IPMNs sometimes penetrate into other adjacent organs, penetration into two organs, including the spleen, is rare. The rare case of IPMC penetrating into the stomach and spleen presented here was treated successfully by total pancreatectomy.
KeywordsIntraductal papillary-mucinous neoplasm Pancreas Penetration Fistula
In 1982, intraductal papillary-mucinous neoplasm (IPMN) was reported by Ohashi et al. as a mucus-producing pancreatic carcinoma characterized by a favorable prognosis . IPMNs are potentially malignant, grossly visible intraductal epithelial neoplasms composed of mucin-producing columnar cells. The lesions exhibit papillary proliferation, cyst formation, and varying degrees of cellular atypia. IPMNs can be classified into three types based on imaging studies and/or histopathology: main duct, branch duct, and mixed type.
Kimura et al. initially reported nine cases of IPMN penetrating into other organs, such as the common bile duct, or developed fistula formation . Kobayashi et al. reported that the incidence of fistula formation is 6.6% (18 of 274 cases) ; the organs penetrated were the duodenum (67%), stomach (44%), common bile duct (33%), colon (6%), and small intestine (6%). Notably, 39% of the cases with fistula formation developed into multiple organ fistula formation. To the best of our knowledge, there have been no reports of penetration into the spleen. In this context, penetration into multiple organs including the spleen is very rare. Here, we report a case of IPMN with penetration not only into the stomach, but also the spleen, that was successfully treated by total pancreatectomy.
During the last three decades, an increasing number of reports of IPMN of the pancreas have been published [2, 4, 5]. Though IPMNs originate from the pancreatic duct cells similar to invasive ductal adenocarcinoma of the pancreas, IPMN exhibits a unique clinical feature different from invasive ductal adenocarcinoma, such as secretion of a large quantity of mucin by the neoplasm, and slow and expansive growth associated with low malignant potentials for metastasis and invasion compared to invasive ductal adenocarcinoma. Fistula formation into other organs is also one of the characteristic features of IPMNs. With regard to its incidence, Kimura et al. initially reported nine cases with IPMN which penetrated into other organs such as common bile duct or developed fistula formation . Kobayashi et al. also investigated that the incidence of the fistula formation was 6.6% (18 out of 274 cases) . The organs penetrated were also reported in their investigation: duodenum (67%), stomach (44%), common bile duct (33%), colon (6%), and small intestine (6%). Notably, 39% of the cases with fistula formation developed into multiple organs fistula formation. In the report, the spleen is not reported as the organ penetrated into by IPMN, and furthermore, to the best of our knowledge, there have been no reports describing IPMN cases penetrating into the spleen. In this context, our IPMN case, which exhibited penetration into multiple organs including the spleen, is very rare, suggesting significance of reporting the case. The pathogenesis of fistula formation in IPMN is generally considered to be divided into two main types based on the underlying mechanism: invasive penetration of cancer cells and mechanical penetration. Though invasive penetration is derived from direct invasion of organs by cancer cells, mechanical penetration is due to the high inner pressure of a mucus-filled pancreatic duct [3, 6]. Kobayashi et al. reported that three out of nine cases (33%) had invasive penetration, and mechanical penetration was shown in the remaining six cases (67%) . In the current case, cancer cells did not exist in the area of the fistulas, suggesting mechanical penetration as the underlying mechanism in the development of the fistula. Our finding that the mucus in the MPD swelled out when the resected specimen was divided may be associated with the high inner pressures, which may support mechanical penetration as the pathogenesis of fistula formation in this case. Several previous studies have reported that inflammation is also involved in mechanical penetration [7, 8, 9, 10]. Based on our finding of inflammatory cells at the fistulas in this case, inflammation may exist at the fistula, resulting in mechanical penetration. Furthermore, when considering mechanical penetration apart from invasive penetration, a pressure gradient seems to be necessary for fistula development. Lumen organs, including the duodenum, stomach, and bile duct, may easily be under lower pressure than solid organs, such as the spleen, which could be one reason why fistula formation into the spleen is rare compared to the lumen organs. Kawarada et al. reported a 5-year survival rate of IPMC with penetration of 46.5% in Japan . Kimura et al. reported a 5-year survival rate of IPMC with penetration or invasion of neighboring organs of 28% . In our case, the follow-up period was just 15 months. Although the previously reported prognosis might not be applied to our case since the abovementioned prognosis was concerning about cases with invasive carcinoma, not about non-invasive carcinoma, further observation would be necessary in our case.
We experienced a case of IPMN of the pancreas penetrating into the stomach and spleen that was successfully treated by total pancreatectomy. This case could contribute to improving our understanding of this type of neoplasm.
All authors are in agreement with the content of the manuscript.
TH and YT conceived of the case presentation and drafted the manuscript. HT and SA wrote the histopathological details of the manuscript. KN, HN, TO, MH, KO, TT, SN, HI, TI, and KA organized the manuscript. MY and TN prepared the endoscopic details of the manuscript. KD supervised the writing of the manuscript. All authors read and approved the final manuscript.
Ethics approval and consent to participate
Written informed consent was obtained from the patient for the participation. The Human Ethics Review Committee of Toyonaka Municipal Hospital approved this study.
Consent for publication
Written informed consent was obtained from the patient for the publication of this case report and any accompanying images.
The authors declare that they have no competing interests.
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