Management of mucinous cystic neoplasms of the pancreas

The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the "pancreatic mucinous cystic neoplasm", "pancreatic mucinous cystic tumour", "pancreatic mucinous cystic mass", "pancreatic cyst", and "pancreatic cystic neoplasm" to identify English language articles describing the diagnosis and treatment of the mucinous cystic neoplasm of the pancreas. In total, 16 322 references ranging from January 1969 to December 2009 were analysed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucinproducing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis, and positron emission tomography-CT. Surgery is indicated for all MCNs.     

Large mucinous cystic neoplasm of the pancreas associated with pregnancy

Mucinous cystic neoplasms （MCNs） of the pancreas occur mostly in females and are potentially sex hormone-sensitive. However, a MCN occurring during pregnancy is quite rare. A 30-year-old woman in the tenth week of pregnancy was referred to us because of a rapid increase in left hypochondrial distending pain. On ultrasound, the patient had a large intra-abdominal cystic lesion. She was thereafter diagnosed with missed abortion and a computed tomography scan showed that the lesion was a cystic tumor 18 cm in diameter originating from the pancreatic tail. The patient subsequently underwent tumor resection with distal pancreatectomy, sparing the spleen. Histopathological analysis of the specimen revealed a pancreatic MCN with moderate dysplasia. Immunohistochemically, the tumor was positive for both estrogen and progesterone receptors. To our knowledge, this is the first reported case of pancreatic MCN with moderate dysplasia in association with pregnancy. Our case strongly indicates that pancreatic MCN is female-hormone dependent.     

A case report of anaplastic carcinoma of the pancreas with remarkable intraductal tumor growth into the main pancreatic duct

We herein report a case of anaplastic carcinoma of the pancreas with remarkable intraductal tumor growth into the main pancreatic duct.A 76-year-old male was referred to our hospital for treatment of a pancreatic tumor.Preoperative examinations revealed a poorly defined tumor in the main pancreatic duct in the body of the pancreas,accompanied with severe dilatation of the main pancreatic duct,which was diagnosed as an intraductal papillary-mucinous neoplasm.We performed distal pancreatectomy and splenectomy.The pathological examination revealed that the tumor consisted of a mixture of anaplastic carcinoma(giant cell type)and adenocarcinoma in the pancreas.There was a papillary projecting tumor composed of anaplastic carcinoma in the dilated main pancreatic duct.The patient is now receiving chemotherapy because liver metastasis was detected 12 mo after surgery.In this case,we could observe a remarkable intraductal tumor growth into the main pancreatic duct.We also discuss the pathogenesis and characteristics of this rare tumor with specific tumor growth.     

Diagnosis and management of relapsing pancreatitis associated with cystic neoplasms of the pancreas

One of the most important causes of relapsing pancreatitis is a cystic neoplasm of the pancreas. These low grade malignancies may cause pancreatitis by obstructing or communicating with a pancreatic duct. Patients with relapsing pancreatitis and a focal fluid fluid collection should be investigated for the possibility of a mucinous cystic neoplasm. Cross sectional imaging can provide a diagnosis with the imaging findings of a low attenuation cystic lesion containing mural calcification （CT scanning） or a lobular T2 enhancing lesion （MRCP）. Endoscopic ultrasound can provide more detailed imaging with the ability to guide fine needle aspiration of the cyst fluid. Cyst fluid analysis can provide a diagnosis of a mucinous cystic lesion with the combination of cytology （mucinous epithelium）, elevated carcinoembryonic antigen （CEA）, and the presence of DNA mutations. Management of these patients consists of surgical resection and monitoring in patients not able to withstand surgery.     

Carcinoembryonic antigen-producing adrenal adenoma resected using combined lateral and anterior transperitoneal laparoscopic surgery

A 74-year-old woman presented with symptoms consistent with hyperadrenocorticism and hyperca- techolaminism. She had a cushingoid appearance and her cortisol level was elevated. Her serum dopamine and noradrenalin levels were also elevated. Computed tomography detected a left adrenal mass measuring 3.5 cm x 3.0 cm in diameter. Metaiodobenzylguanidine scintigraphy was negative. Unexpectedly, the serum Serum carcinoembryonic antigen （CEA） level waselevated. Fluorodeoxyglucose positron emission tomography showed increased uptake in the adrenal tumor only, with a maximum standardized uptake value of 2.8. Selective venography and blood sampling revealed that the concentrations of cortisol, catecholamines and CEA were significantly elevated in the vein draining the tumor. A diagnosis of CEA-producing benign adenoma was made. After preoperative management, we performed a combined lateral and anterior transpedtoneal laparoscopic adrenectomy. Her vital signs remained stable during surgery. Histopathological examination revealed a benign adenoma. Her cortisol, catecholamine and CEA levels normalized immediately after surgery. We present, to the best of our knowledge, the first case of CEA-producing adrenal adenoma, along with a review of the relevant literature, and discuss our laparoscopic surgery techniques.     

Giant biliary cystadenoma complicated with polycystic liver:A case report

Biliary cystadenoma(BCA)is a rare hepatic neoplasm.Although considered a benign cystic tumor of the liver,BCA has a high risk of recurrence with incomplete excision and a potential risk for malignant degeneration.Correct diagnosis and complete tumor excision with negative margins are the mainstay of treatment.Unfortunately,due to the lack of presenting symptoms,and normal laboratory results in most patients,BCA is hard to distinguish from other cystic lesions of the liver such as biliary cystadenocarcinoma,hepatic cyst,hydatid cyst,Caroli disease,undifferentiated sarcoma,intraductal papillary mucinous tumor,and hepatocellular carcinoma.Ultrasound(US),computed tomography(CT)and magnetic resonance imaging(MRI)may be necessary.They demonstrate intrahepatic cystic lesions with features such as mural nodules,varying wall thickness,papillary projections,and internal septations.Nevertheless,surgery is still the only means of accurate diagnosis.Definitive diagnosis requires histological examination following formal resection.We describe a57-year-old woman initially diagnosed with polycystic liver who was subsequently diagnosed with giant intra-hepatic BCA in the left hepatic lobe.This indicates that both US physicians and hepatobiliary specialists should attach importance to hepatic cysts,and CT or MRI should be performed for further examination when a diagnosis of BCA is suspected.     

Destructive granuloma derived from a liver cyst： A case report

We herein report the case of an idiopathic liver cystic mass which aggressively infiltrated the thoraco-abdominal wall. A 74-year-old woman who had a huge cystic lesion in her right hepatic lobe was transferred to our hospital for further examinations. Imaging studies revealed a simple liver cyst, and the cytological findings of intracystic fluid were negative. She was followed up periodically by computed tomography (CT) scans. Seven years later, she complained of a prominence and dull pain in her right thoraco-abdominal region. CT revealed an enlargement of the cystic lesion and infiltration into the intercostal subcutaneous tissue. We suspected the development of a malignancy inside the liver cyst such as cystadenocarcinoma, and she therefore underwent surgery. A tumor extirpation was performed, including the chest wall, from the 7th to the 10th rib, as well as a right hepatic lobectomy. Pathologically, the lesion consisted of severe inflammatory change with epi-thelioid cell granuloma and bone destruction without any malignant neoplasm. No specific pathogens were evident based on further histological and molecular examinations. Therefore the lesion was diagnosed to be a destructive granuloma associated with a long-standing hepatic cyst. Since undergoing surgery, the patient has been doing well without any signs of recurrence.     

Aggressive behaviour of solid-pseudopapillary tumor of the pancreas in adults：A case report and review of the literature

Solid-pseudopapillary tumor （SPT） is a rare neoplasm of the pancreas that usually occurs in young females. It is generally considered a low-grade malignant tumor that can remain asymptomatic for several years. The occurrence of infiltrating varieties of SPT is around 10%-15%. Between 1986 and 2006, 282 cystic tumors of the pancreas were observed. Among them a SPT was diagnosed in 8 patients （2.8%） with only one infiltrating variety. This was diagnosed in a 49-year-old female 13 years after the sonographic evidence of a small pancreatic cystic lesion interpreted as a pseudocyst. The tumor invaded a long segment of the portal- mesenteric vein confluence, and was removed with a total pancreatectomy, resection of the portal vein and reconstruction with the internal jugular vein. Histological examination confirmed the R-0 resection of the primary SPT, although a vascular invasion was demonstrated. The postoperative course was uneventful, but 32 mo after surgery the patient experienced diffuse liver metastases. Chemotherapy with different drugs was started. The patient is alive and symptom-free, with stable disease, 75 mo after surgery. Twenty-five patients with invasion of the portal vein and/or of mesenteric vessels were retrieved from the literature, 16 recent patients with tumor relapse after potentially curative resection were also retrieved. The best treatment remains a radical resection whenever possible, even in locally advanced or metastatic disease. The role of chemotherapy, and/or radiotherapy, is still to be defined.     

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.     

Primary retroperitoneal mucinous cystadenoma： Report of a case and review of the literature

Primary retroperitoneal mucinous cystadenomas （RMCs） are very rare and their biological behavior and histogenesis remain speculative. An accurate preoperative diagnosis of these tumors is difficult because no effective diagnostic measures have been established. We describe a 29-year-old woman with abdominal pain and a palpable mass. Computed tomography （CT） of the abdomen revealed a retroperitoneal cystic mass which was resected successfully at laparotomy. Histopathological examination of the resected mass confirmed the diagnosis of RMC. There was no evidence of disease 2 years after surgery.     

Mucinous cystic neoplasm of the pancreas associated with pregnancy: report of two cases

Pancreatic neoplasms during pregnancy are rare. We describe two cases of mucinous cystic neoplasm (MCN) associated with pregnancy. A 34-year-old woman in the 26th week of pregnancy was given a diagnosis of MCN measuring 19 cm in diameter. We decided to resect it post partum in consideration of the risk of abortion. She had a healthy infant in the 40th week of pregnancy and three months later, distal pancreatectomy was performed. Pathological analysis revealed a mucinous cystadenoma of the pancreas. The second case was a 36-year-old woman. She was given a diagnosis of MCN, measuring 16 cm in diameter, post partum and distal pancreatectomy was performed. Pathologically, the tumor was an invasive mucinous cystadenocarcinoma of the pancreas. In this report, we did an extensive literature review and discussed the management of MCN during pregnancy with special reference to the timing of the operation and the relationship between tumor growth and sex hormones.     

Undifferentiated (anaplastic) carcinoma of the pancreas showing sarcomatous change and neoplastic cyst formation

Summary Conclusion Pancreatic undifferentiated (anaplastic) carcinoma contained two components: sarcomatous change of spindle-cell type and a neoplastic cyst. Immunohistochemical analysis suggested that both the sarcomatous and the cystic portions were of epithelial origin. This case showed a “dual” differentiation both into the sarcomatous change and into the cystic lesion. Background Pancreatic sarcoma or sarcomatous change is very rate. The origin of the sarcomatous change is unknown. On the other hand, pancreatic adenocarcinoma sometimes shows necrosis and forms a cystic lesion during its growth, but a neoplastic cyst formation is very rare except for cystadenomas. Methods We report a case of pancreatic undifferentiated (anaplastic) carcinoma associated with sarcomatous change and neoplastic cyst formation. Clinicopathological and immunohistochemical analyses were performed. Results A 75-yr-old male was admitted because of low back pain and body weight loss. He died 7 d after admission presenting obstructive jaundice. Autopsy disclosed that the large mass was a poorly differentiated ductal adenocarcinoma, which mostly consisted of sarcomatous components of spindle-cell type. The cystic lesion was a neoplastic cyst with the wall composed of epithelial tumor cells. There was no necrosis or hemorrhage in the cystic cavity. Immunohistochemical analyses suggested that both the tumor and the cystic lesion were of epithelial origin. Sarcomatous changes were recognized also in the metastatic lesions in the liver and lymph nodes. The tumor is considered to be labeled undifferentiated (anaplastic) carcinoma of the pancreas.     

A Biliary Mucinous Cystic Neoplasm with Intrahepatic and Lymph Node Metastases

A 51-year-old woman who presented with a large cystic liver tumor with mural nodules in the lateral segment developed Trousseau''s syndrome. A mural nodule directly invaded her liver parenchyma. Metastatic nodules were detected in the right lobe and portal/paraaortic lymph nodes. The pathological findings showed mucin-producing adenocarcinoma cells to have invaded the fibrous stroma forming a micropapillary cluster. She developed obstructive jaundice due to tumor progression and subsequently died of hepatic failure. Invasive biliary mucinous cystic neoplasm (MCN) is a rare form of a malignant tumor with a relatively favorable prognosis. This is a very rare case biliary MCN with invasive carcinoma that showed intrahepatic and lymph node metastases.     

Serous cystic neoplasm of the pancreas-indications for surgery

BACKGROUND/AIMS: Serous cystic neoplasm is a rare pancreatic tumor. Almost all of these tumors are benign and only 9 cases of serous cystadenocarcinoma have been reported. Although serous cystic neoplasm is typically a microcystic lesion, there is a wide range of cyst sizes from micro to macro and even unilocular cysts have been reported. Thus, the diagnosis is difficult and indications for surgery are controversial. We aimed to elucidate the clinicopathological and imaging features of serous cystic neoplasm of the pancreas. METHODOLOGY: We investigated 15 cases of resected and 6 cases of nonresected cases of serous cystic neoplasm, evaluating the symptoms, imaging findings, preoperative diagnosis, macroscopic morphology, microscopic findings, and results of follow-up. RESULTS: Imaging diagnosis of serous cystic neoplasm was not easy, because not so many tumors had the typical microcystic pattern. Most of the resected serous cystic neoplasms were non-microcystic or were small tumors, which could not be precisely evaluated. CONCLUSIONS: Small serous cystic neoplasms, which can be diagnosed by imaging, do not need to be resected because serous cystadenocarcinoma is rare. Tumors of the pancreas that cannot be confirmed to be serous cystic neoplasm should be resected because of the possibility of pancreatic cancer, mucinous cystadenocarcinoma, or mucinous cystadenoma with malignant potential.     

胰腺肿瘤的病理诊断和鉴别诊断

胰腺肿瘤发病率近年呈上升趋势,本文仅就胰腺外分泌的上皮性肿瘤的主要类型加以介绍。其中包括胰腺导管腺癌及其主要的变型和腺泡细胞癌、胰母细胞瘤等。文中还包括了浆液性囊性肿瘤、黏液性囊性肿瘤、导管内乳头状黏液肿瘤（IPMN）和导管内乳头状嗜酸性肿瘤以及实性一假乳头状肿瘤,对上述个性肿瘤的免疫组化特点和分子特征也进行了阐述。     

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.     

Serous cystadenocarcinoma of the pancreas

Summary Conclusion A rare case of granulocyte-colony stimulating factor (G-CSF) produced by carcinoma of the pancreas has been reported. Background This is the first case showing high G-CSF concentration in the aspirated tumor fluid (mucin) at its early stage without leukocytosis. Methods The tumor, detected incidentally in a 64-yr-old male, was removed by a distal pancreatectomy. The mass was 7.0×6.5×4.5 cm, and was histologically diagnosed as cystadenocarcinoma with prominent sarcomatous transformation. It was classified as anaplastic carcinoma. Results After 4 wk of resection, progressive leukocytosis was observed. Seven weeks after the operation, the peripheral leukocyte count increased to 126,000/mL. After 8 wk of resection, the patient died of recurrence. The serum G-CSF concentration was elevated after recurrence. The preserved mucin contained in the cystic components of the resected specimen had a G-CSF concentration higher than 2400 pg/mL. G-CSF is a known cytokine and an etiologic agent in paraneoplastic syndromes. An early diagnosis can, therefore, be made prior to the manifestation of clinical symptoms by the evaluation of the aspirated tumor fluid. This can lead to the prevention of the paraneoplastic syndrome with inhibitory cytokines in future.     

Combined hepatocellular carcinoma and neuroendocrine carcinoma with sarcomatous change of the liver after transarterial chemoembolization

Primary hepatic neuroendocrine carcinoma is rare and its origin is not clearly understood. An admixture of hepatocellular carcinoma (HCC) and neuroendocrine carcinoma is particularly rare. Here, we report a patient with an extremely rare combination of HCC and neuroendocrine carcinoma of the liver. To our knowledge, this is the first reported case in which the carcinoma showed sarcomatous change. The patient was a 76‐year‐old man who had received outpatient treatment for chronic hepatitis C. On abdominal computed tomography (CT), the hepatic tumor was enhanced in the arterial phase but its density was lower than that of normal liver in the portal phases. His serum α‐fetoprotein (AFP) level was very high. Therefore, transarterial chemoembolization (TACE) was performed based on the diagnosis of HCC. Ten months after TACE, his serum AFP level had increased to the level measured before TACE. Partial hepatectomy was performed because CT revealed poor enhancement of the recurrent tumor. Histopathologically, the tumor consisted of two distinct components: moderately differentiated HCC was intermingled with a neuroendocrine carcinoma, which was accompanied by sarcomatous changes. Immunohistochemically, the neuroendocrine carcinoma cells were positive for CD56, chromogranin A and neuron‐specific enolase, and negative for AFP. The sarcomatous area was positive for AE1/3 and CD56, consistent with sarcomatous change of neuroendocrine carcinoma. The neuroendocrine carcinoma and/or sarcomatous change may have been due to phenotypic changes and/or dedifferentiation of HCC induced by TACE. Six months after surgery, the patient was diagnosed with metastasis of the neuroendocrine carcinoma to sacral bone. He died 7 months after surgery.     

Cyst fluid analysis obtained by EUS-guided FNA in the evaluation of discrete cystic neoplasms of the pancreas: a prospective single-center experience

BACKGROUND: Accurate assessment of pancreatic cystic neoplasms is imperative before selecting available treatment options, such as surgical resection, drainage, or conservative therapy. Available modalities, CT and magnetic resonance imaging, have been inconsistent in diagnosis. Reports involving EUS and cyst fluid analysis have been encouraging, including studies of EUS features and/or cyst fluid analysis, which may differentiate pancreatic cystic neoplasms. OBJECTIVE: To retrospectively determine cyst fluid characteristics that differentiate cystic neoplasms. DESIGN: Patient evaluation included (1) EUS features (reported elsewhere) and (2) cyst fluid analysis (carcinoembryonic antigen [CEA], carbohydrate antigen 19-9 [CA 19-9], amylase and lipase, viscosity [VIS], mucin stain, and cytology). Exclusion criteria included the following: intraductal papillary mucinous tumor lesions, bloody cyst aspirate, neuroendocrine tumors, and patients without surgical histopathology. SETTING: Pancreatic Biliary Center, St Luke's Medical Center, Milwaukee, Wisconsin. PATIENTS: A total of 102 patients (60 women, 42 men; age, 23-76 years) presented for evaluation of pancreatic cystic neoplasm; 71 underwent surgical resection. RESULTS: Seventy-one of 102 patients who underwent surgery presented the following histopathologic correlates: 23 pseudocysts (PC), 13 serous cystadenoma (SCyA), 21 mucinous cystadenoma (MCyA), and 14 mucinous cystadenocarcinoma (MCyA-CA). Cyst fluid analysis of these patients showed the following: VIS was lower in PC (mean, 1.3) and SCyA (1.27) when compared with MCyA (1.84) and MCyA-CA (1.9). All mucinous neoplasms had VIS >1.6, whereas only 2 mucinous cystic neoplasms (MCN) had VIS = 1.6 (both PC). The CEA level was significantly higher in MCyA (adenoma [878 ng/mL], carcinoma [27,581 ng/mL]) vs PC (189 ng/mL), and SCyA (121 ng/mL). Amylase levels were higher in PC (7210 U/L) compared with cystic neoplasm (SCyA, 679 U/L; MCyA, 1605 U/L; MCyA-CA, 569 U/L). CONCLUSIONS: Differential diagnosis of pancreatic cystic neoplasm is significantly enhanced by cyst fluid analysis. Elevated CEA (> or =480 ng/mL) and VIS (>1.6) accurately predict MCN from SCyA and PC. Malignant from benign MCN can be differentiated by CEA levels > or =6000 ng/mL.