A case of focal autoimmune pancreatitis (AIP) mimicking an intraductal papillary mucinous neoplasm (IPMN)

The present case involved a 76-year-old man with a cystic mass in the head of his pancreas. The cystic lesion, which measured 17.7 × 9.8 mm, was first detected by ultrasonography (US) at the age of 72 years. Follow-up endoscopic ultrasonography (EUS) performed at 4 years after the lesion had first been detected revealed a mural nodule measuring 14.0 × 8.4 mm in the cyst. Endoscopic retrograde pancreatography (ERP) imaging revealed that the main pancreatic duct was in communication with the cyst and that there was no irregular narrowing of the main pancreatic duct. On the basis of these results, the patient was diagnosed with an intraductal papillary mucinous neoplasm (IPMN), and stomach-preserving pancreaticoduodenectomy was performed. A histopathological examination revealed that the interior of the cystic part of the lesion was lined by a pancreatic ductal epithelium. A pathological examination of the nodular lesion detected storiform fibrosis, severe lymphoplasmacytic infiltration, and hyperplasia in the pancreatic duct epithelium together with a small amount of mucus. On immunohistological staining, the infiltrating lymphoplasmacytes were found to be positive for IgG4. Accordingly, the patient was diagnosed with focal autoimmune pancreatitis (AIP). In conclusion, we reported a case of focal AIP mimicking IPMN. This case showed neither enlargement of the pancreas nor irregular narrowing of the main pancreatic duct.     

Synchronous pancreatic solid pseudopapillary neoplasm and intraductal papillary mucinous neoplasm

Solid pseudopapillary neoplasm (SPN) is a rare and low-grade malignant pancreatic neoplasm composed of poorly cohesive monomorphic neoplastic cells forming solid and pseudopapillary structures with frequent hemorrhagic-cystic degeneration. Intraductal papillary mucinous neoplasm (IPMN) is a pancreatic exocrine tumor composed of intraductal papillary growth of mucin containing neoplastic cells in the main pancreatic duct or its major branches. In the case presented here, a 53-year-old, Japanese man was found to have multiple cystic lesions and dilatation of the main pancreatic duct in the neck of the pancreas. Histological examination revealed a main-duct and branch-duct type IPMN, of the gastric-type, involving the neck of the pancreas, associated with a 0.5 cm SPN in the caudal side of the IPMN. We diagnosed this case as synchronous SPN and IPMN. As far as we know, only one other case of synchronous SPN and IPMN has been reported. Both the present case and the previously reported case showed abnormal nuclear expression of β-catenin in SPN, whereas IPMN showed no abnormal nuclear expression. These results suggest that β-catenin abnormality is not a common pathogenetic factor of synchronous SPN and IPMN.     

Laparoscopic resection of synchronous intraductal papillary mucinous neoplasms: A case report

We describe herein a 68-year-old woman who was diagnosed with a quite rare entity of intraductal papillary mucinous neoplasms (IPMNs) occurring simultaneously in the left lateral lobe of liver and the tail of pancreas. Abdominal computed tomography and magnetic resonance cholangiopancreatography showed a cystic dilatation of the pancreatic duct in the pancreatic tail, which suggested an IPMN, and multiple intrahepatic duct stones in the left lateral lobe. The patient underwent a laparoscopic left lateral hepatolobectomy and spleen-preserving distal pancreatectomy. Intra-operative finding of massive mucin in the dilated bile duct implied an intraductal mucinous tumor in the liver. The diagnosis of synchronous IPMNs in the liver and pancreas was confirmed by pathological examination. The patient was followed up for 6 mo without signs of recurrence. Although several cases of IPMN of liver without any pancreatic association have been reported, the simultaneous occurrence of IPMNs in the liver and pancreas is very rare. To the best of our knowledge, it is the first reported case treated by laparoscopic resection.     

Cystic micropapillary neoplasm of peribiliary glands with concomitant perihilar cholangiocarcinoma

We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5 AC and S100 P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma.     

A case of unilocular serous cystic neoplasm mimicking mucinous cystic neoplasm

A 40's woman had a cystic lesion in the tail of the pancreas that had grown over a 1.5-year period. Endoscopic ultrasound revealed a partition structure and "cyst-in-cyst" like lesion, and a diagnosis of mucinous cystic neoplasm was made. The patient underwent distal pancreatectomy with splenectomy. Following histological examination, our final diagnosis was revised to unilocular serous cystic neoplasm since the increase in cysts was due to hemorrhage and the partition structure was in fact granulation tissue. We here discuss this rare case with reference to previous published reports.     

A case of intraductal papillary mucinous neoplasm of the pancreas arising from pancreas divisum without ventral pancreatic duct of Wirsung]

Most reported cases of intraductal papillary mucinous neoplasms (IPMNs) originate from Wirsung's duct or their branches. IPMNs arising from Santorini's duct and its branches have rarely been reported. Eight cases of IPMN arising from Santorini's duct have been published worldwide. However, these cases are associated with incomplete type of pancreas divisum. Recently, one report of IPMN with complete absence of Wirsung's duct has been reported. This patient was a 57-year-old woman who was admitted to the hospital due to progressive jaundice. On endoscopic retrograde cholangiopancreatography, there was a severely bulging ampulla of Vater and patulous minor papilla draining mucinous material and a cystic lesion communicating with the dilated Santorini's duct without any communication with Wirsung's duct. A pancreaticoduodenectomy was performed and the pathologic examination of resected specimen showed no evidence of Wirsung's duct, but an IPMN arising from Santorini's duct with peripancreatic lymph node metastasis. Herein, we report a case of invasive IPMN arising from pancreatic head without ventral pancreatic duct with a review of the relevant literatures.     

Pseudomyxoma peritonei arising from intraductal papillary neoplasm after surgical pancreatectomy: report of 2 cases and review of the literature

We report 2 cases of pseudomyxoma peritonei (PMP) combined with an intraductal papillary mucinous neoplasm (IPMN) and a review of the literature. In both cases, PMP emerged after surgical resection of the IPMN. In one case, neoplastic foci were present in the surgical margin and PMP was found 1 year after the initial resection, while PMP emerged 5 years after surgical resection in the other case. Including these 2 cases, 8 cases of PMP arising from IPMN have been reported. This condition occurs more frequently in males (7 males, 1 female), while the age at diagnosis ranges from 49 to 82 years, with a mean of 63.3 years. IPMNs occur more commonly in the tail of the pancreas (62.5%). Two different patterns regarding the mechanism of PMP arising from IPMN have been indicated; a rupture of the pancreatic duct, after which the neoplasm spreads through the fistula into the peritoneal cavity, and post-surgical development of PMP after insufficient surgical treatment for an IPMN. Our findings indicate that attention must be given to avoid mucous leakage and obtain a negative surgical margin during surgical treatment of an IPMN.     

内镜诊治胰腺导管内乳头状粘液性肿瘤的应用

目的探讨胰腺导管内乳头状粘液性肿瘤(IPMN)的诊断、内镜下治疗,以提高对该疾病的认识及内镜对其诊断及治疗的意义。方法回顾性分析经内镜诊治的IPMN患者12例,总结并分析他们的一般情况、临床症状、影像学检查、实验室检查、内镜下治疗等方面资料。结果 12例IPMN患者以老年男性为主,无特异性临床表现,主要发生部位为胰头或钩突部,CT主要表现为囊实性低密度影,其内可有壁结节,胰管扩张伴或不伴胆管扩张,2例侵及胰腺实质。磁共振胰胆管造影(MRCP)主要表现为胰管扩张,胰腺萎缩,胰头部圆形高信号影。内镜下逆行胰胆管造影(ERCP)显示十二指肠乳头膨大,开口扩张,可见胶冻样粘液流出,胰管造影显示胰管全程扩张或胰头部囊状扩张,3例伴胆总管、肝内胆管扩张。9例行ERCP治疗,清除粘液栓并置入胰管支架,3例同时置入胆管支架。结论 IPMN是一种特殊类型的胰腺囊性疾病,影像学检查有其独特的表现,ERCP对其诊断及治疗有重要意义。     

Hemosuccus pancreaticus caused by a mucinous cystic neoplasm of the pancreas

Hemosuccus pancreaticus is a gastrointestinal hemorrhage through the main pancreatic duct. Here, we report a rare case of hemosuccus pancreaticus due to a mucinous cystic neoplasm of the pancreas. A 62-year-old woman who had been followed for a branch duct intraductal papillary mucinous neoplasm visited our emergency room due to severe abdominal pain and bloody discharge. Computed tomography revealed that the pancreatic cyst increased the tension of the wall and a high-density area indicative of bleeding into the cyst was observed. Endoscopy showed opening of and hemorrhaging from the papilla of Vater. The patient was diagnosed with hemosuccus pancreaticus caused by hemorrhaging into the cyst from the branch duct intraductal papillary mucinous neoplasm. Based on this diagnosis, elective distal pancreatectomy was performed. The histopathological diagnosis was a mucinous cystic neoplasm with intermediate-grade dysplasia based upon the pathological findings that fibrous ovarian-type stroma existed abundantly and the stroma cells were positive for progesterone receptor and inhibin. Hemosuccus pancreaticus caused by a mucinous cystic neoplasm is extremely rare and there has been only one case reported to date. In conclusion, it should be recognized that pancreatic cystic neoplasms including mucinous cystic neoplasms may cause hemosuccus pancreaticus.     

Neuroendocrine neoplasm of pancreas with cystic degeneration mimicking mucinous cystic neoplasm

Endoscopic ultrasound is increasingly being used for evaluation of pancreatic diseases and pancreatic tumors. Among various pancreatic cystic lesions, cystic degeneration of pancreatic neuroendocrine neoplasm is of the challenge in making diagnosis. Although unique characteristic of each type of pancreatic cystic lesions has been proposed abundantly, typical morphology of cystic degeneration of pancreatic neuroendocrine neoplasm is still unclear. We, herein, reported a case of 66-year-old woman who was incidentally found to have a cystic lesion in the tail of pancreas upon screening transabdominal ultrasonography. A well-defined cystic lesion with rim calcification was noted on subsequent abdominal computed tomography. Endoscopic ultrasound revealed a markedly thick-wall cystic lesion containing solid nodule inside which was not enhanced following contrast-enhanced study. A mucinous cystic neoplasm was suspected and the patient was proceeded with distal pancreatectomy. A definite diagnosis of neuroendocrine neoplasm was confirmed after staining with synaptophysin and chromogranin A. We performed a meticulous review on current literatures focusing on endoscopic characteristics of pancreatic neuroendocrine neoplasms with cystic degeneration.     

Immunohistochemical staining as supportive diagnostic tool for pseudomyxoma peritonei arising from intraductal papillary mucinous neoplasm: A report of two cases and literature review

Background/ObjectivesPseudomyxoma peritonei (PMP) arising from an intraductal papillary mucinous neoplasm of the pancreas (IPMN) is a rare condition. The diagnosis of IPMN as the origin of PMP is mainly inferred from the clinical course and the exclusion of PMP from other organs. The pathological diagnosis has not yet been established. To evaluate the usefulness of immunohistochemical staining for the diagnosis of the primary lesion of PMP as IPMN.MethodsThere are 2 cases of PMP arising from IPMN between March 2010 and December 2019 at National Center for Global Health and Medicine. A PubMed search that reported PMP arising from IPMN identified 16 additional cases. Diagnostic methods and clinicopathological features of 18 cases were compared.ResultsFour cases including our two cases used immunohistochemical staining for the diagnosis of PMP arising from IPMN. The correspondence of the immunohistochemical staining between PMP and IPMN was shown in the three cases including previously reported two cases and one of our two cases to identify the primary lesion of PMP as IPMN. In addition, we revealed that the comparison of the immunostaining pattern of PMP with the representative immunostaining pattern of the candidate primary lesions is helpful for the diagnosis of the primary lesion of PMP.ConclusionsImmunohistochemical staining is helpful to identify the primary lesion of PMP as IPMN.     

A case of minimal invasive carcinoma from intraductal papillary mucinous neoplasm with invasive ductal carcinoma of the pancreas]

A 69-year-old man was referred to our hospital for epigastralgia. He was found to have elevation of serum amylase and CA19-9. Ultrasonography, abdominal CT, MRCP, ERCP and EUS showed the cystic lesion and a possibility of an other tumor. There was a stenosis of the main pancreatic duct (MPD) at the pancreas head and dilatation of the MPD from the body to the tail. Intraductal papillary mucinous neoplasm (IPMN) of the branch pancreatic duct was diagnosed, and there was a likelihood of ductal carcinoma of the pancreas. We therefore performed pancreatoduodenectomy. Pathological finding showed invasive carcinoma from an intraductal papillary mucinous neoplasm with invasive ductal carcinoma of the pancreas.     

Rapid growth of mucinous cystic adenoma of the pancreas following pregnancy

A 25-yr-old woman delivered a healthy child by cesarean section. At 8 mo postpartum, she became aware of an upper abdominal tumor. Abdominal computed tomography and upper abdominal ultrasonography revealed a large cystic mass in the body of the pancreas. Endoscopic retrograde pancreatography showed no connection between the main pancreatic duct and the cystic lesion. The patient underwent tumor resection at 11 mopostpartum. Pathological examination of the tumor revealed mucin-producing columnar epithelial cells lining the cystic wall with ovarian-type stromal tissue and no findings indicative of malignancy, giving a diagnosis of mucinous cystic adenoma of the pancreas. Immunohistochemical studies revealed positive staining for progesterone receptor but not for estrogen receptor in the stromal cell nuclei. Postpartum rapid growth of a benign mucinous cystic neoplasm might be linked to the production of female sex hormones during lactation.     

Prevalence,Diagnosis and Management of Pancreatic Cystic Neoplasms: Current Status and Future Directions

Cystic neoplasms of the pancreas are found with increasing prevalence, especially in elderly asymptomatic individuals. Although the overall risk of malignancy is very low, the presence of these pancreatic cysts is associated with a large degree of anxiety and further medical investigation due to concerns about malignancy. This review discusses the different cystic neoplasms of the pancreas and reports diagnostic strategies based on clinical features and imaging data. Surgical and nonsurgical management of the most common cystic neoplasms, based on the recently revised Sendai guidelines, is also discussed, with special reference to intraductal papillary mucinous neoplasm (IPMN; particularly the branch duct variant), which is the lesion most frequently identified incidentally. IPMN pathology, its risk for development into pancreatic ductal adenocarcinoma, the pros and cons of current guidelines for management, and the potential role of endoscopic ultrasound in determining cancer risk are discussed. Finally, surgical treatment, strategies for surveillance of pancreatic cysts, and possible future directions are discussed.     

Localized type 1 autoimmune pancreatitis superimposed upon preexisting intraductal papillary mucinous neoplasms

A 70-year-old woman was found to have 2 cystic lesions in the head of the pancreas on abdominal ultrasonography during a routine medical examination.Endoscopic ultrasonography(EUS)and magnetic resonance cholangiopancreatography showed multilocular cysts in the head of the pancreas without dilation of the main pancreatic duct.The patient was followed-up semiannually with imaging studies for suspected branch duct-type intraductal papillary mucinous neoplasm(IPMN).At 3 years after initial presentation,hypoechoic lesions were observed around each pancreatic cyst by EUS.Diffusion-weighted imaging showed high-intensity regions corresponding to these lesions.Therefore,a diagnosis of invasive carcinoma derived from IPMN could not be excluded,and subtotal stomach-preserving pancreaticoduodenectomy was performed.The macroscopic examination of the surgical specimen showed whitish solid masses in the head of the pancreas,with multilocular cysts within each mass.Microscopically,each solid mass consisted of inflammatory cells such as lymphocytes and plasma cells.Furthermore,immunochemical staining revealed immunoglobulin G4-positive cells,and many obliterating phlebitides were observed.The cysts consisted of mucus-producing epithelial cells and showed a papillary growth pattern.Based on these findings,we diagnosed multiple localized type 1 autoimmune pancreatitis occurring only in the vicinity of the branch ducttype IPMN.     

Epidemiology,clinical features and diagnostic work-up of cystic neoplasms of the pancreas: Interim analysis of the prospective PANCY survey

IntroductionA prospective survey to evaluate the diagnostic workup of cystic pancreatic neoplasms (CPNs) according to the Italian guidelines.MethodsAn online data sheet was built.ResultsFifteen of the 1385 patients (1.1%) had non cystic neoplastic lesions. Forty percent (518/1295) had at least one 1st degree relative affected by a solid tumor of the digestive and extra-digestive organs. Symptoms/signs associated with the cystic lesion were present in 24.5% of the patients. The cysts were localized in the head of the pancreas in 38.5% of patients. Of the 2370 examinations (1.7 examinations per patient) which were carried out for the diagnosis, magnetic resonance imaging was performed as a single test in 48.4% of patients and in combination with endoscopic ultrasound in 27% of the cases. Of the 1370 patients having CPNs, 89.9% had an intraductal papillary mucinous neoplasm (IPMN) (70.1% a branch duct IPMN, 6.2% a mixed type IPMN and 4.6% a main duct IPMN), 12.7% had a serous cystadenoma, 2.8% a mucinous cystadenoma, 1.5% a non-functioning cystic neuroendocrine neoplasm, 0.7% a solid-pseudopapillary cystic neoplasm, 0.3% a cystic adenocarcinoma, and 1.2% an undetermined cystic neoplasm. Seventy-eight (5.7%) patients were operated upon after the initial work-up.ConclusionsThis prospective study offers a reliable real-life picture of the diagnostic work-up CPN.     

Duodenum-preserving total pancreatic head resection for cystic neoplastic lesions in the head of the pancreas

Background/Purpose

Cystic neoplastic lesions of the pancreas are now found with increasing frequency. Duodenumpreserving pancreatic head resection with segmental resection of the duodenum has been introduced for the surgical treatment of inflammatory and neoplastic lesions. We report the following data from 15 patients treated surgically for cystic neoplastic lesions of the pancreas head.

Methods

Duodenum-preserving total pancreatic head resection (DPPHRt) with segmental resection of the duodenum (SD) was performed in eight patients, five with intraductal papillary mucinous neoplasm (IPMN), two with mucinous cystic neoplasm (MCN), and one with cystic endocrine neoplasm (EN). In four patients, a subtotal pancreatic head resection was performed, but recurrence of the IPMN lesion was observed in two patients. Ten patients suffered cystadenoma, three patients had a borderline lesion, and two patients had an in-situ carcinoma.

Results

Eight patients had a DPPHRt with SD resection, two patients had a resection of the uncinate process including segmental resection of the inferior duodenal segment, and one patient had a duodenum-and spleen-preserving total pancreatectomy. In four patients a DPPHR with subtotal pancreatic head resection was carried out. Postoperative local complications occurred in eight patients: there was a recurrence of the IPMN lesion in the remnant pancreatic head in two patients; and there was intraabdominal bleeding in one patient, pancreatic fistula in one patient, and delay of gastric emptying in four patients. Seven patients showed signs of acute pancreatitis. Hospital mortality was 0%, and postoperative length of hospital stay was 10. 4 days (range, 8–18 days).

Conclusions

Duodenum-preserving total pancreatic head resection for IPMN, MCN, serous cystadenoma (SCA), and cystic EN lesions is a safe and beneficial surgical procedure. Segmental resection of the duodenum was applied for an oncologically complete resection. In regard to long-term outcome, the procedure is, additionally, a pancreatic cancer preventive strategy.

     

Tumeurs intracanalaires papillaires et mucineuses du pancréas découvertes fortuitement : à propos d’un cas

Intraductal papillary mucinous neoplasms of pancreas (IPMN) are cystic lesions of the pancreas than can be associated with extra-pancreatics malignancies. We report a case of intraductal papillary mucinous neoplasm discovered incidentally during the staging of rectal adenocarcinoma. This observation illustrates the high frequency of IPMN incidentally discovered, for instance during the work-up of an extrapancreatic tumor.     

胰腺囊性病变的临床特征和诊治分析

背景:随着影像学技术的进步,胰腺囊性病变的检出率逐年升高,但鉴别不同类型的胰腺囊性病变仍是临床的棘手问题。目的:分析胰腺囊性病变的临床特征和诊治方法。方法:纳入2003年1月~2013年1月上海交通大学附属第一人民医院收治的胰腺囊性病变患者,对入组患者的临床表现、实验室检查、影像学特征以及治疗方法进行回顾性分析。结果:共49例患者纳入研究,其中假性囊肿13例、潴留囊肿4例、先天性囊肿2例、浆液性囊性肿瘤(SCN)9例、黏液性囊性肿瘤(MCN)11例、导管内乳头状黏液性肿瘤(IPMN)7例、实性-假乳头状肿瘤(SPN)3例。49例患者中16例患者无症状,其余33例患者表现为腹痛、腹胀、恶心、呕吐、发热等。13例胰腺假性囊肿患者均有急、慢性胰腺炎或外伤病史,3例IPMN患者血清CA19-9升高。B超、CT、MRI/MRCP、EUS诊断胰腺囊性病变的准确率分别为24.5%、32.7%、61.1%、100%。49例患者均接受手术治疗,分别行内引流术、囊肿切除术、胰十二指肠切除术、胰体(尾)切除术以及节段性胰腺切除术。结论:胰腺囊性病变无特异性临床表现。CT、MRI/MRCP作为无创检查手段,应广泛用于囊性病变的诊断和评估,EUS可作为进一步检查手段。选择合理的手术方案是治疗胰腺囊性病变的关键。     

Total dorsal pancreatectomy for intraductal papillary mucinous neoplasm in a patient with pancreas divisum.

We report a case of intraductal papillary mucinous neoplasm confined to the dorsal (Santorini) pancreatic duct. A 51-year-old woman presented with a cystic lesion in the head of her pancreas and pancreas divisum. A biopsy taken during cyst-enteric drainage revealed dysplastic epithelium so the patient was scheduled for resection. At operation, excision of the entire dorsal pancreas was performed with preservation of the unaffected ventral pancreas and the spleen and its vessels. Over 6 years later she remains well with stable weight and a good quality of life. This case illustrates the benefits of anatomical preservation in pancreatic resection, and was performed some years prior to the only other reported similar case.