Simultaneous mucinous cystadenoma of ovary and mucinous cystadenocarcinoma of pancreas

Mucinous cystic tumors were discovered synchronously in the tail of the pancreas and in the right ovary of an adult female. Both tumors were amenable to surgical resection. The pancreatic tumor was a noninvasive mucinous cystadenocarcinoma and the ovarian tumor was a mucinous cystadenoma. We feel these tumors represent two primaries, an uncommon occurrence, and not a single primary tumor with metastasis.     

The prognosis of intraductal papillary mucinous tumors of the pancreas

BACKGROUND/AIMS: Intraductal papillary mucinous tumors of the pancreas have been recognized as a distinct clinical entity. However, their biological behavior has not been clearly defined. The aim of this study was to examine the prognosis of this tumor, to clarify the biological behavior and determine the most appropriate treatment. METHODOLOGY: Correlations between prognosis of operated cases and histopathologic features were investigated. RESULTS: In 105 patients with characteristic clinical features of intraductal papillary mucinous tumors, the lesions were classified as hyperplasias in 21%, intraductal tumors in 48% and invasive carcinomas in 31%. Minimal invasion was apparent in 25%, lymph node metastasis in 21%, and fistula formation in 31% of the invasive lesions. Non-invasive and minimally invasive intraductal papillary mucinous tumors were essentially free from risk of tumor recurrence. Other invasive intraductal papillary mucinous tumors showed a significantly poor prognosis. CONCLUSIONS: Because of the variation in pathological characteristics, patient outcome and the possibility of differential diagnosis, the treatment might be recommended as follows: the case of hyperplasia can be followed-up with close surveillance. Non-invasive and minimally invasive intraductal papillary mucinous tumors should be operated with function-preserving minimal pancreatectomy. For patients with invasive intraductal papillary mucinous tumors evident with preoperative imaging modalities, radical operations with lymph node dissection might be needed.     

Primary retroperitoneal mucinous cystadenoma:A case report

Primary retroperitoneal mucinous cystic tumors are extremely rare.These tumors can be classified as a primary retroperitoneal mucinous cystadenoma with or without borderline malignancy or primary retroperitoneal mucinous cystadenocarcinoma.The most common of these is primary retroperitoneal mucinous cystadenoma,which almost always occurs in female patients;only ten cases have been reported in males.The most common clinical findings for this tumor type include nonspecific abdominal pain and a palpable abdominal mass.A definitive diagnosis is usually obtained from histopathology after surgical excision.Here,we report the case of a 60-year-old female patient who complained of abdominal pain that had been present for 3 mo and presented with a palpable abdominal mass.Multidetector computed tomography scanning revealed a large,unilocular cystic mass in the left retroperitoneal space.Surgical intervention was performed and the tumor was completely removed.Histopathologic examination confirmed that the tumor was a primary retroperitoneal mucinous cystadenoma.Two years after surgery,the patient remains disease free.     

Simultaneous liver mucinous cystic and intraductal papillary mucinous neoplasms of the bile duct:A case report

Cystic hepatic neoplasms are rare tumors,and are classified into two separate entities:mucinous cystic neoplasms(MCNs)and intraductal papillary mucinous neoplasms of the bile duct(IPMN-B).We report the case of a 56-year-old woman who presented with abdominal pain and jaundice due to the presence of a large hepatic multilocular cystic tumor associated with an intraductal tumor.Partial hepatectomy with resection of extrahepatic bile ducts demonstrated an intrahepatic MCN and an intraductal IPMN-B.This is the first report of the simultaneous occurrence of these two histologically distinct entities in the liver.     

p53 protein expression in intraductal papillary mucinous tumors (IPMT) of the pancreas as an indicator of tumor malignancy

BACKGROUND/AIMS: Intraductal papillary mucinous tumors, as a cystic disease in the pancreas, clinically has a more indolent and favorable course than invasive ductal pancreas carcinoma. However, some cases of intraductal papillary mucinous tumors show invasive and rapid progression like ductal pancreas carcinoma and the prognosis of such patients is sometimes poor. In the current study, we carried out immunohistochemical staining of intraductal papillary mucinous tumor tissues for p53 and investigated whether positive staining indicates tumor malignancies and has a prognostic value for intraductal papillary mucinous tumors. METHODOLOGY: Nineteen (19) patients who underwent pancreatic resection under the diagnosis of intraductal papillary mucinous tumors at the Chiba University Hospital between April 1992 and December 1996 were studied. We performed immunohistochemical staining of p53 as well as of PCNA, Ki-67 and Bcl-2 using their respective antibodies. Pathological findings revealed that 9 cases were intraductal papillary adenoma, 9 were intraductal papillary adenocarcinoma, and one was invasive ductal papillary adenocarcinoma. RESULTS: p53 expression could only be detected in the 1 case with invasive ductal papillary adenocarcinoma. Significant association could not be found between histological features and immunohistochemical staining of PCNA, Ki-67 and Bcl-2. CONCLUSIONS: p53 protein expression could be detected after progression to invasive type of intraductal papillary mucinous tumors. The present results demonstrate that p53 expression might be an indicator of invasive progression in intraductal papillary mucinous tumors, and might represent a surgical indicator of intraductal papillary mucinous tumors.     

Giant cell tumor of the pancreas arising in the ovarian-like stroma of a mucinous cystadenocarcinoma

Summary We describe a malignant mucinous cystic neoplasm of the pancreas with ovarian-like stroma within which an osteoclast-like giant cell rich tumor arose. This rare tumor had a unique immunohistochemical profile with the giant cells staining for vimentin, leukocyte common antigen, and the monocyte/macrophage marker CD68, whereas the mucinous epithelium stained for epithelial membrane antigen and cytokeratin. The immunohistochemical findings are consistent with two lines of differentiation, one epithelial and the other suggesting mesenchymal differentiation of the giant cell tumor with an immunophenotype similar to giant cell tumor of bone. The coexistence of these two rare tumors suggests that they are histogenetically related. The finding of a giant cell tumor arising in the ovarian stroma indicates that the stroma of mucinous tumors is not always an innocuous component of the tumor.     

P53 mutation but not p16/MTS1 mutation occurs in intraductal papillary mucinous tumors of the pancreas

BACKGROUND/AIMS: Intraductal papillary mucinous tumors of the pancreas are rare lesions, which typically show a benign clinical course. However, some of these tumors have a malignant nature and grow in an invasive manner. The purpose of the study was to determine the prevalence of p53-, p16/MTS1- and K-ras mutations in benign and malignant intraductal papillary mucinous tumors with intent to value their importance for tumor progression. METHODOLOGY: Thirteen different archival tumor specimens were obtained at the Department of Pathology, University of Ulm. Three cases showed an invasive component of the tumor. Genomic DNA was extracted after laser capture microdissection of tumor cells from paraffin-embedded tissue sections. The corresponding sequences of p53 (exon 5, 6, 7, 8) and p16/MTS1 (exon 2) were amplified by polymerase chain reaction and subjected to single strand conformation polymorphism analysis. Codon 12 of K-ras was analyzed by the enrichment polymerase chain reaction-restriction fragment length polymorphism method. Positive samples were further investigated by sequencing. RESULTS: K-ras mutations occurred in benign and malignant intraductal papillary mucinous tumors (4/13), whereas an alteration of the coding p53 gene sequence could only be detected in the intraductal and invasive component of one malignant tumor. None of the tissue specimens revealed mutations in exon 2 of p16/MTS1. CONCLUSIONS: In contrast to K-ras mutations, alterations in the p53 gene may characterize ductal papillary mucinous carcinomas, which could be of major interest for their early diagnosis. The lack of mutations in the p16/MTS1 gene suggests that other genes may be involved in the formation of intraductal papillary mucinous neoplasias.     

Biliary papillary tumors share pathological features with intraductal papillary mucinous neoplasm of the pancreas

Recently, attention has been drawn to papillary neoplasm of the pancreatobiliary systems. In the pancreas, the disease entity of intraductal papillary mucinous neoplasm (IPMN-P) is widely recognized. In contrast, the pathological characteristics of biliary papillary tumors, such as biliary papilloma(tosis) and papillary cholangiocarcinoma, have not yet been well documented. In this study, we compared the pathological features and post-operative prognosis among biliary papillary tumors (10 cases of biliary papilloma(tosis) and 22 cases of papillary cholangiocarcinoma), conventional non-papillary cholangiocarcinoma (15 cases), and IPMN-P (31 cases). Macroscopically, all biliary papillary tumors were characterized by the prominent intraductal papillary proliferation, and macroscopic mucin-hypersecretion was seen in 9 of 32 cases (28%). Histologically, biliary papillary tumors consisted of three types of tumor cells (pancreaticobiliary, intestinal and gastric types), whereas only the pancreaticobiliary type was observed in non-papillary cholangiocarcinoma. Immunohistochemically, biliary papillary tumors were characterized by the common expression of MUC2, CDX2 and cytokeratin 20. In addition, biliary papillary tumors could be associated with two types of invasive lesions: tubular adenocarcinoma (9 cases) and mucinous carcinoma (5 cases). Patients with tubular adenocarcinoma had a poor prognosis compared to non-invasive papillary tumor or papillary tumor with mucinous carcinoma. These pathological characteristics and the survival status of biliary papillary tumors were different from those of non-papillary cholangiocarcinoma, and rather closely resembled those of IPMN-P. In conclusion, biliary papillary tumors may be the biliary counterpart (intraductal papillary neoplasm of the bile duct) of IPMN-P.     

Clinicopathology and prognosis of mucinous gastric carcinoma

BACKGROUND/AIMS: Mucinous gastric carcinoma (MGC) is a rare histopathological type of gastric carcinoma, for which the clinicopathological features and prognosis remain controversial. To clarify the clinical significance of mucinous histological type in gastric cancer, we studied clinicopathological characteristics of MGC tumors and prognosis of patients. METHODOLOGY: Forty-one patients with MGC and 1,407 patients with non-mucinous gastric carcinoma (NGC) were included in the study. Tumors were evaluated against patient gender and age, tumor location, size, and macroscopic type, depth of gastric wall invasion, lymph node metastasis, liver metastasis, peritoneal dissemination, distant metastasis, stage, and operative curability. RESULTS: Compared with NGC tumors, MGC tumors were larger, showed more serosal invasion, were associated with a higher incidence of lymph node metastasis, and peritoneal dissemination, and tended to be at a more advanced stage. However, multivariate analysis demonstrated that the mucinous histological type was neither an independent prognostic factor nor an independent risk factor for lymph node metastasis in patients with gastric cancer. CONCLUSIONS: The mucinous histological type had no influence on patient outcome or the frequency of lymph node metastasis. MGC tumors are therefore biologically similar to those in NGC.     

Intraductal papillary mucinous tumors of the pancreas

Cystic neoplasms of the exocrine pancreas are a small fraction of pancreatic tumors. Within that group of cystic neoplasms, intraductal papillary mucinous tumors (IPMTs) can be distinguished from mucinous cystic neoplasms, serous cystic neoplasms, and pseudopapillary cystic tumors. Awareness of IPMTs has increased since the World Health Organization classified these tumors as its own group in 1996. Because of their favorable prognosis, an extensive diagnostic workup for IPMTs should be performed in patients presenting with cystic lesions of the pancreas. This workup often leads to the diagnosis and the predominant tumor location and size, although the extent of the ductal changes can only be established by histopathology. Surgical resection is the therapy of choice for IPMTs. The type of resection depends upon the extent of the quantitative and qualitative ductal involvement. Total pancreatectomy is currently the treatment for an IPMT that comprises the entire main duct.     

Giant biliary mucinous cystadenoma of the liver

Biliary mucinous cystadenomas (BMC) of the liver are rare benign cystic tumors, however an estimated 20% undergo malignant transformation. They have recently been redefined as mucinous cystic neoplasms in the 2010 WHO classification. The preferred treatment is through radical resection, as there are high recurrence rates with other treatment modalities; however this is often not possible in patients with bilobar or giant cysts, and liver transplantation may be indicated. We present a patient with a giant biliary mucinous cystadenoma of the liver and discuss the management with reference to the literature. A 47 year-old woman presented with a 6-week history of moderate epigastric discomfort on a background of 12 months of symptom-free abdominal distension. A giant cystic bilobar tumor of the liver measuring 22 × 23 × 17 cm was diagnosed and characterised by ultrasound scan and magnetic resonance imaging. Serum bilirubin, alkaline phosphatase and gamma-glutamyl transpeptidase were elevated, though other laboratory data including tumor markers (CEA, aFP, CA19-9) were within normal limits. Total excision of the cyst was not possible due to its size and position, and the patient underwent cyst drainage, a sub-total cyst excision and omentoplasty. Histology confirmed a benign biliary mucinous cystadenoma with an ovarian stroma. Though the patient remained clinically well, routine post-operative computed tomography (CT) surveillance showed an 11 cm recurrent cyst at 6 months. A partial cyst resection with close follow-up, regular CA19-9 serology and ultrasound/CT imaging, may be a reasonable alternative for bilobar or giant cysts. However should any features pathognomonic of malignancy develop, then a liver transplantation is indicated.     

EUS FNA in intraductal papillary mucinous tumors of the pancreas

BACKGROUND/AIMS: There is little information concerning the potential role of fine-needle aspiration guided by endoscopic ultrasonography in the pathologic diagnosis of intraductal papillary mucinous tumors of the pancreas. METHODOLOGY: Patients with an intraductal papillary mucinous tumor of the pancreas suggested by endoscopic ultrasonography underwent fine-needle aspiration guided by endoscopic ultrasonography in order to investigate the presence of mucin and/or cytologic changes consistent with this diagnosis. A group of 111 patients with other pancreatic lesions explored during the same period of time was used as a control group. RESULTS: Fine-needle aspiration guided by endoscopic ultrasonography was safely performed in 19 patients and supported the diagnosis in 17 of them. Nine out of the 17 patients with suspicion of intraductal papillary mucinous tumors of the pancreas went to surgery and this diagnosis was confirmed in the resected specimen in all of them. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of EUS FNA in the diagnosis of IPMT were 82%, 100%, 100%, 92% and 94% respectively. CONCLUSIONS: Fine-needle aspiration guided by endoscopic ultrasonography is a good technique to support the diagnosis of intraductal papillary mucinous tumors of the pancreas and should be considered in this group of patients if pathologic confirmation is judged to be necessary.     

Clinicopathologic review of 41 cases of pancreatic mucinous cystic neoplasms]

BACKGROUND/AIMS: Intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms are included in mucin-producing pancreatic tumors. The reports about IPMN are not uncommon but those about the mucinous cystic neoplasms are relatively few. The aims of this study were to define the natural history of resected mucinous cystic neoplasms of the pancreas and to identify the findings which suggest malignancy. METHODS: The authors retrospectively evaluated the clinical outcomes of 41 patients with mucinous cystic neoplasms who were surgically resected at Asan Medical Center between 1995 and 2004. RESULTS: Women (n=33) were more frequently affected than men (n=8). Thirty three patients (80.6%) had adenoma, 1 (2.4%) borderline malignancy, 1 (2.4%) carcinoma in situ, and 6 (14.6%) invasive mucinous cystadenocarcinoma. The most frequent symptom was abdominal pain (39%). About half of the enrolled patients were asymptomatic. Unilocular type (79%) was more frequent than the multilocular type (21%) on gross morphology. The tumor size of invasive mucinous cystic neopolasms was larger than that of non-invasive mucinous cystic neoplalsms (p=0.01). Abdominal pain was more frequent in invasive mucinous cystic neoplasms (p=0.026). On gross morphology, mural nodules were detected in 4 of 6 patients with invasive mucinous cystic neoplasms. However, they were not detected in any patients with non-invasive mucinous cystic neoplasms. Recurrence developed in none of the 35 patients with non-invasive mucinous cystic neoplasms, however 2 of the 6 patients with invasive mucinous cystic neoplasms died within 5 years. CONCLUSIONS: Clinical predictors of invasive mucinous cystic neoplasms are suggested to be tumor size and abdominal pain. The prognosis of the non-invasive mucinous cystic neoplasms is excellent when curative resection is performed.     

Clinicopathological and immunohistochemical analysis of malignant features in mucinous cystic tumors of the pancreas

BACKGROUND/AIMS: To investigate the malignancy of mucinous cystic tumors (MCTs) of the pancreas, we examined clinicopathological features and immunohistochemical findings of MCT. METHODOLOGY: We analyzed the expression of p53 protein, proliferating cell nuclear antigen, alpha6-integrin subunit, alpha5beta1-integrin, and interleukin-1 receptor type I in tumor specimens from eight patients with MCT. RESULTS: The tumors were classified as mucinous cyst adenoma (n=6) or mucinous cyst adenocarcinoma (n=2). The actuarial five-year survival rate was 83.3%. All in eight MCTs had 'ovarian-type' stroma in the cyst wall. The alpha6-integrin subunit and p53 protein were expressed in adenocarcinoma tissues of MCTs, and in two adenomas the alpha6-integrin subunit and p53 protein were also co-expressed. CONCLUSIONS: Our present results indicate that coexpression of the alpha6-integrin subunit and p53 protein should be appreciated as an indicator of malignancy in MCTs.     

Intraductal papillary mucinous tumor: diagnostic and therapeutic approach

Primary cystic pancreatic neoplasms are rare tumors, with an approximate prevalence of 10% of cystic pancreatic lesions. Most of these lesions correspond to mucinous cystic neoplasm, serous cystoadenoma and intraductal papillary mucinous tumor (IPMT). IPMT is characterized by diffuse dilatation of the main pancreatic duct and/or side branches with inner defects related to mucin or tumor, or mucin extrusion from a patent ampulla. IPMT has a low potential for malignancy, with a low growth rate, a low rate of metastatic spread and postsurgical recurrence. Over the last few years, major advances have been made in the diagnostic and therapeutic management of this tumor.     

Intraductal papillary mucinous tumor with pancreatobiliary and pancreatodigestive fistulae: a case report

Pancreatobiliary or pancreatodigestive fistulae are rare during the course of intraductal papillary mucinous tumors. The mechanism of the fistula is either malignant involvement or mechanical. We report the case of an 81-year-old patient with intraductal papillary mucinous tumor complicated by fistulae between the main pancreatic duct and common bile duct, duodenum and stomach. These fistulae were treated endoscopically and radiologically by several metallic stents.     

Calcification in mucinous cholangiocellular carcinoma

Calcification is rarely seen in cholangiocellular carcinoma. We herein report the case of a 53 year-old man with calcification in a cholangiocellular carcinoma. Because imaging studies had revealed coarse calcified foci, hepatolithiasis was suspected pre-operatively. The patient underwent a laparotomy in which intra-operative cholangioscopy revealed no gallstones but did reveal an unsuspected tumor with abundant mucin. A left hepatic lobectomy with resection of the extrahepatic bile duct was performed. The tumor histology was mucinous adenocarcinoma with calcification. In the English language literature, we found 9 cases of cholangiocellular carcinoma with macroscopic calcification. Six of these cases were mucinous adenocarcinomas. Roentgenologic examination revealed coarse calcification in 7 cases and fine calcification in 2 cases. Clinicians should note that cholangiocellular carcinoma, especially the mucinous variant, may be accompanied by coarse calcification.     

Intraductal mucinous tumors occurring simultaneously in the liver and pancreas

A case of simultaneous intraductal mucinous tumors of the liver and pancreas in a 67-year-old man is described. Abdominal ultrasonography and computed tomography (CT) revealed the presence of cystic lesions with intraluminal septae both in the caudate lobe of the liver and in the uncinate process of the pancreas; these cystic lesions communicated with the hepatic duct and pancreatic duct, respectively. Mucin retention was observed in the cysts, and cholestasis was induced by mucin secretion into the common bile duct. The lesions were resected by left hepatic lobectomy with caudate lobectomy, and segmental pancreatectomy. Both lesions were multilocular cystic tumors with no papillary projections or focal mass effect in their walls. Histologically, both cystic lesions were a mixture of hyperplasia and adenoma lined by low papillary columnar epithelium. There were no cellular or histological features to suggest malignant change. The fibrous intratumor interstitium lacked any mesenchymal or ovarian-like stroma. The hepatic lesion was considered to be of a similar nature to intraductal papillary mucinous tumor (IPMT) of the pancreas. However, the two lesions occurred simultaneously in the liver and pancreas. This case is of interest in regard to the diagnosis and management of mucinous hepatopancreatobiliary lesions. Received: March 16, 2001 / Accepted: September 14, 2001     

Intraductal papillary mucinous tumors of the pancreas: the preoperative value of cytologic and histopathologic diagnosis

BACKGROUND: The preoperative diagnosis of intraductal papillary mucinous tumors of the pancreas must be as certain as possible because removal of a large portion of the pancreas or even total pancreatectomy may be necessary. The value of cytologic and histopathologic analysis of specimens obtained by preoperative endoscopic investigations is unknown. The aim of this study was to assess the value of such analyses of specimens obtained by EUS-guided FNA and/or biopsy, or transpapillary biopsy specimens obtained during endoscopic retrograde pancreatography for the diagnosis of intraductal papillary mucinous tumors of the pancreas and for the detection of malignancy. METHODS: Between 1992 and 2001, 42 patients (22 men, 20 women; median age 64 years) underwent surgical resection for intraductal papillary mucinous tumors of the pancreas and had preoperative pancreatic tissue sampling. In the case of isolated dilatation of pancreatic ducts, pancreatic juice was obtained by EUS-guided FNA for cytologic analysis. In the presence of a solid lesion or main pancreatic duct stenosis, biopsy specimens were obtained by EUS-guided FNA biopsy or endoscopic retrograde pancreatography, which permitted histopathologic assessment. The accuracy of cytology and histopathology was evaluated for the following: (1) positive diagnosis of intraductal papillary mucinous tumors of the pancreas and (2) assessment of malignancy, by comparison with histopathologic examination of surgical resection specimens. RESULTS: Cytologic analysis was performed in 19 patients; it was positive in 4 (21%) and noninformative in 15 (79%). Histopathologic analysis was performed in 23 patients; it was positive in 21 (91%) and negative in 2 (9%). Histopathologic analysis yielded a positive result in 83% of patients who did not have extrusion of mucus from a patulous papilla. The sensitivity, specificity, and positive and negative predictive values of histopathologic analysis for the diagnosis of malignancy were, respectively, 44%, 100%, 100%, and 33%. When histopathologic analysis was positive, the tumor grade was similar to that determined by final histopathologic examination in 38% of patients, whereas the grade was underestimated in 62%. No complication occurred as a result of tissue sampling. CONCLUSIONS: The sensitivity of histopathologic analysis of EUS-guided FNA biopsy specimens or transpapillary biopsy specimens is 91% for the positive diagnosis of intraductal papillary mucinous tumors of the pancreas with a solid component, which is of particular interest as extrusion mucus from the papilla was absent in most patients. Histopathologic analysis of biopsy specimens of malignant intraductal papillary mucinous tumors of the pancreas often underestimates tumor grade. The result for cytologic analysis of juice obtained from dilated pancreatic ducts is disappointing.     

Difficulties in vivo diagnosis of mucinous adenocarcinoma of vermiform appendix with metastasis in the navel

The article presents a clinical case of mucinous adenocarcinoma in vivo diagnosis of vermiform appendix with metastasis in the navel. Neoplasms vermiform appendix--an extremely rare tumor pathology: the total share of all malignant tumors are less than 1%. Mucinous carcinoma is a rare tumor, according to various authors the frequency of its occurrence ranges from 3 to 10% of all malignancies. By Localization mucinous carcinoma more frequently is found in the colon and rectum (about 20% of cases), ovary (5-10% of all malignant ovarian tumors), stomach, uterus and also in the pancreas (only about 2%). Metastasis in the navel is a very rare disease and is known as Sister Mary Joseph's Nodule, the primary site often localized in the digestive tract (52%), much less--in the female reproductive organs (28%), unspecified localization is approximately 15-20% of cases. The difficulty of diagnosis in the present case was that, in spite of life conducted during survey of the colon, organic pathology was not identified. The presence of hepatosplenomegaly and ascites at the initial examination gave cause to the erroneous diagnostic conception of the presence of liver cirrhosis. It is also was not paid due attention to the presence of changes in the umbilical ring, which were regarded as an umbilical hernia. To verify the diagnosis allowed the infiltrate biopsy of the umbilical region, followed by histological examination of the preparation and identification of a characteristic picture of mucinous cancers.