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.     

Huge mucinous cystadenoma of the pancreas developing during pregnancy: a case report

A 33-year-old woman, gravida 2, para 1, was found to have a huge intra-abdominal cyst (2619 ml) in the 15th week. The cyst increased in volume to 3660 ml during the next 31 days. A tentative diagnosis of ovarian or pancreatic mucinous cystadenoma was made. Since the cyst was considered to cause fetal intra-uterine growth restriction, the patient underwent surgery in the 23rd week of pregnancy. The cyst was found to originate from the pancreas, and distal pancreatectomy with splenectomy was performed. The specimen weighed 5500 gm, which is the largest so far reported. Her postoperative course was uneventful, and she had a healthy full-term infant. Histological diagnosis was a benign mucinous cystadenoma. Up to the present, there have been three reported cases of pancreatic mucinous cystadenoma, including our case, and two cases of pancreatic mucinous cystadenocarcinoma, in association with pregnancy. Our case is the third reported of successful resection of the tumor during pregnancy resulting in a healthy infant.     

Mucinous cystic neoplasm of the pancreas during pregnancy: the importance of proper management

We describe a case of huge mucinous cystic tumor of the pancreas in a 26-year-old woman during pregnancy. Ultrasonography demonstrated a well-delimited cystic mass in the left upper abdominal quadrant, suggestive of benignity. Magnetic resonance imaging showed a large cystic mass resembling a mucinous cystic tumor of the pancreas. After this assessment the patient underwent surgical exploration and a huge cystic tumor of the pancreas was discovered. The tumor was enucleated and distal pancreatectomy was performed. The resected margin of the specimen was free of tumor. In this case report we discuss the management of mucinous pancreatic tumors during pregnancy and we briefly review the previously reported cases of mucinous pancreatic tumors in pregnant patients. We conclude that surgical resection of these tumors should be strongly considered in pregnancy. Removal of the tumor appears to be a safe procedure without harmful effects to the fetus.     

Mucinous cystadenocarcinoma of the pancreas during pregnancy

The occurrence of a mucinous cystadenocarcinoma of the pancreas during pregnancy is extremely rare, reported in only twice in the English literature. We describe a 34-year-old patient with this rare condition who was successfully managed by resection of the tumor with distal pancreatectomy and splenectomy. The operation was performed at 15 weeks of gestation, and the patient went on to deliver a healthy infant at 39 weeks. To our knowledge, this is the first report of successful resection of a cystadenocarcinoma of the pancreas during pregnancy. The specimen weighed 2500 g and measured 20 cm across, making it the largest reported mucinous cystadenocarcinoma of the pancreas encountered during pregnancy to date.     

Anaplastic carcinoma associated with a mucinous cystic neoplasm of the pancreas during pregnancy： Report of a case and a review of the literature

Oncogenesis of anaplastic carcinoma of the pancreas is a subject of controversy, because it shows sarcomatous nature with extremely poor prognosis. We herein report an unusual case of anaplastic carcinoma occurring with a recurrent mucinous cystic neoplasm in a 38-year-old female. A 10-cm retroperitoneal cystic mass was pointed out in the first pregnancy and a probable diagnosis of mucinous cystic neoplasm was made in October 2000. She refused surgery first and delivered her baby uneventfully. During her second pregnancy in 2002, however, she presented hematemesis and underwent urgent distal pancreatectomy, splenectomy and partial resection of the gastric wall where the tumor perforated. A diagnosis of borderline-type mucinous cystic neoplasm with ovarian-like stroma was made. Nine months later, CT visualized a recurrent cystic tumor near the pancreatic stump, which was subsequently resected. Pathology revealed that the tumor was composed of two different components of borderline-type mucinous cystic neoplasm and anaplastic carcinoma. The latter was intensely positive for vimentin, CD68, p53 and focally for cytokeratin, suggesting both sarcomatous and carcinomatous differentiation. She survived four years after the second surgery without tumor recurrence. Although the origin of anaplastic carcinoma has not been determined yet, it should be remembered that anaplastic carcinoma can occur in association with mucinous cystic neoplasm of more benign histology.     

A resected case of multiple intraductal papillary mucinous tumors of the pancreas with US-guided ductal branch-oriented partial pancreatectomy.

A 69-year-old man with epigastralgia was admitted on August 26, 2002 and diagnosed with multiple intraductal papillary mucinous tumors by various imagings. The cystic tumor of pancreas head had a diameter of 2 cm, and the mural nodule of the cystic tumor measured only 3 mm. In the pancreas body the cystic tumor was measured at 1.5 cm with the mural nodule of the cystic tumor measuring 3 mm. It was believed that the tumors were benign. However, a mural nodule of the cystic lesion was recognized, thus, the possibility of malignancy could not be completely ruled out. The reduction operation for preservation of pancreatic parenchyma should be selected for these circumstances. Ductal branch-oriented partial pancreatectomy was performed on September 6, 2002 with intraoperative ultrasonography and a Cavitron Ultrasonic Aspirator, preserving the main pancreatic duct and normal pancreatic parenchyma. The operation was successful, and the histopathological diagnosis of the tumors was intraductal papillary adenoma of the pancreas.     

A case of mucinous cystadenoma of the pancreas resected after a 10-year follow-up

A 40-year-old woman was hospitalized because of acute pancreatitis. CT showed a monolocular cyst thought to be a pseudocyst. The patient was followed as an outpatient. The size of the cyst decreased 6 years later. Ultrasonography 10 years later demonstrated an enlarged cyst with a cyst-in-cyst formation. A tendency to increase in size and the irregular wall thickening indicated that the cyst was a malignant mucinous cystic neoplasm (MCN) and distal pancreatectomy was performed. The tumor was diagnosed histologically as a mucinous cystadenoma. A case of MCN resected after long-term follow-up is extremely rare. The present case is thought to be important for elucidation of the natural history of MCNs.     

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.     

胰腺黏液性囊腺瘤的诊断和外科治疗

目的 探讨胰腺黏液性囊腺瘤(MCN)的诊断和外科治疗效果.方法 回顾性分析2003年1月至2008年6月问在青岛大学医学院附属医院手术治疗的20例MCN患者的临床表现、病理特征、治疗方法和生存率等临床和随访资料.结果 患者以腹痛、上腹饱胀不适、恶心、呕吐为主要临床表现,11例有上腹部压痛,6例可触及上腹肿块.术前均行B超及CT检查,诊断13例为良性MCN,4例为胰腺浆液性囊腺瘤,3例为黏液性囊腺癌.肿瘤主要位于胰腺体、尾部,直径4～14 cm.20例均行手术治疗,以胰十二指肠切除术、胰体尾切除术为主.术后病理证实,良性MCN 10例,交界性MCN 3例,黏液性囊腺癌7例.术后平均随访26个月,良性MCN和交界性MCN患者均健在,无复发;黏液性囊腺癌患者3年生存率50%.结论 MCN主要好发于女性,临床表现无特征性,术前B超和CT检查可进行诊断,手术切除是惟一有效的治疗方法,预后较好.     

A case of mucinous cystic neoplasm of the gallbladder

The patient was a 70-year-old woman in whom examination revealed a high level of carbohydrate antigen 19-9. Abdominal ultrasonography and computed tomography (CT) revealed a multilocular cystic lesion compressing the gallbladder. CT indicated the presence of a multilocular cystic tumor (67?×?68?×?72 mm) in contact with the right hepatic lobe. Intraoperative findings indicated that the cyst diameter was 8.5?×?6.0 cm, and the cyst was continuous with the gallbladder. The gallbladder was resected along with the cyst. The cyst was multilocular and originated from the cystic duct and gallbladder wall. The cyst wall contained cuboidal to columnar mucin-producing epithelial cells and ovarian-like stroma (OS). The final diagnosis was mucinous cystic neoplasm (MCN) of the gallbladder with low-grade dysplasia. In the 2010 WHO classification of tumors of the digestive system, MCN have been newly defined as a type of hepatobiliary tract epithelial neoplasms. MCN of the gallbladder with OS is extremely rare. Only three cases have been published in the literature. The presence of OS is necessary for diagnosis of MCN.     

Laparoscopic distal pancreatectomy as the total biopsy of the pancreas: tool of minimally invasive surgery

Because of recent progress in imaging modalities, the opportunities to detect pancreatic cystic neoplasms are increasing. However, serous cystadenoma is still uncommon. We report a case of serous cystadenoma treated by laparoscopic distal pancreatectomy. A 52-year-old woman presented with mild upper abdominal pain. Dynamic computed tomography (CT) revealed a solitary cystic lesion 3?cm in diameter in the pancreatic tail. Endoscopic ultrasound showed a honeycomb pattern, indicative of serous cystadenoma. To obtain the final diagnosis of the tumor, we performed laparoscopic distal pancreatectomy. A histopathological study showed microcystadenoma with no evidence of malignancy.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Mixed serous cystadenoma with mucinous cystadenoma of the pancreas

We report a case of serous cystadenoma of the pancreas mixed with mucinous cystadenoma. A 65-year-old woman was admitted to our hospital for evaluation of a palpable, elastic, hard mass measuring 6 cm in diameter in the right upper quadrant of the abdomen. A diagnosis of mucinous cystadenocarcinoma of the pancreas was made, and pancreatoduodenectomy was performed. The tumor was composed of a dominant compartment of macroscopic cyst, and its thick wall was filled with numerous microscopic cysts. The light microscopy findings with hematoxylin and eosin staining, and by the periodic acid-Schiff reaction, were almost perfectly consistent with the characteristics of microcystic or glycogen-rich cystadenoma, but the apical portion of the cytoplasm of the neoplastic cells was stained with Alcian blue at pH 2.4 and by the mucicarmine method. Neoplastic cells containing epithelial acidic mucin are usually found in mucinous cystadenomas. No K-ras point mutations were detected at the sites where neoplastic cells were present, whether or not they contained epithelial acidic mucin. Pancreatic serous cystadenomas that include a mucinous-cystadenoma component are extremely rare, and the difference between serous and mucinous cystadenomas is not always distinct.     

Macrocystic type of serous cystadenoma with a communication between the cyst and pancreatic duct

A 42-year-old woman with a cystic lesion in the head of the pancreas was evaluated by using abdominal ultrasonography, a computed tomographic scan, magnetic resonance imaging and endoscopic retrograde pancreatography. Multiple cystic lesions, 5 cm in diameter, which had papillary protrusion inside the cyst in the head of the pancreas and had the communication between the cysts and pancreatic duct, were determined. Pylorus-preserving pancreaticoduodenectomy was performed under the diagnosis of mucinous cystic neoplasm of the pancreas. Although the cut surface of the tumor showed a macrocystic tumor of 3 cm in diameter, part of the cyst wall was cavernous. A histopathological examination showed single-layered cuboidal cells, which lead to the diagnosis as being serous cystadenoma of the pancreas. Serous cystadenoma is a rare, almost benign pancreatic tumor. The macrocystic subtype of serous cystadenoma is even more rare. We describe a patient who had this macrocystic subtype of serous cystadenoma with a communication between the cyst and pancreatic duct. This case illustrates the difficulty in the diagnosis of cystic lesions in the pancreas, and might support the single category of cystic lesions of the pancreas.     

Spleen-preserving laparoscopic distal pancreatectomy for cystic adenoma

For borderline malignant diseases of the pancreas such as cystic adenoma, partial pancreatectomy or pancreatoduodenectomy including pylorus-preserving pancreaticoduodenectomy have been performed depending on tumor location under large median laparotomy. To investigate the feasibility of a technique with minimal skin incision, while retaining safety equivalent to conventional resection of the pancreatic tail, by making use of the advantages of laparoscopic procedure, we performed a minimally invasive laparoscopic resection of the pancreatic tail with preservation of the spleen. A 69-year-old woman underwent surgery for a diagnostic therapy for a cystic lesion of the pancreatic tail. The procedure was performed as follows: All procedures were performed completely laparoscopically under CO2 insufflation. After dissection of the omentum, laparoscopic ultrasound was performed to identify the location of the tumor and splenic vessels. The splenic hilus was dissected with preservation of the splenocolic ligament to maintain the lower blood supply to the spleen. The left gastroepiploic artery and the short gastric arteries and veins could be preserved. After division of the splenic hilus, the splenic artery and vein were identified from behind the pancreas by being held up and dissected individually by intracorporeal ligation by 3-0 Nylon. Then, pancreatic transection was performed 1 cm proximal to the tumor with the Endo-GIAII. The duration of operation was 4.5 hours. Intraoperative blood loss was under 50 mL. Histological examination revealed mucinous cytadenoma. She could walk the day after surgery and was discharged from the hospital uneventfully. CT prior to discharge from the hospital revealed sufficient blood flow in the spleen. Thus, it may be feasible to select laparoscopic spleen-preserving distal pancreatectomy as a first choice for diagnostic therapy for cystic lesions of the pancreatic tail.     

Near fatal puerperal thrombosis on Bj?rk-Shiley mitral valve prosthesis

A 22-year-old woman required emergency mitral valve replacement three weeks post partum because of thrombotic obstruction of her prosthetic mitral valve. Low dose subcutaneous heparin was administered from the 17th week of pregnancy. Though there was a successful fetal outcome, heparin did not prevent thrombosis on the prosthesis and its continuation into the puerperium proved nearly fatal.     

Near fatal puerperal thrombosis on Björk-Shiley mitral valve prosthesis.

A 22-year-old woman required emergency mitral valve replacement three weeks post partum because of thrombotic obstruction of her prosthetic mitral valve. Low dose subcutaneous heparin was administered from the 17th week of pregnancy. Though there was a successful fetal outcome, heparin did not prevent thrombosis on the prosthesis and its continuation into the puerperium proved nearly fatal.     

A case of mucinous noncystic carcinoma of the pancreas]

Mucinous (colloid) carcinoma is defined as pools of stromal extracellular mucin containing scanty, floating carcinoma cells. It is a well-defined entity in breast or large bowel. However, mucinous noncystic carcinoma of the pancreas (MNCC) is uncommon, comprising between 1% and 3% of all carcinomas of the pancreas. In the past, MNCC generally had been categorized together with ordinary ductal adenocarcinoma or misdiagnosed as mucinous cystadenocarcinoma or signet-ring cell carcinoma. The new WHO classification lists MNCC as a variant of ductal adenocarcinoma. Herein, we report a 32-year-old woman with incidentally found pancreatic body mass who underwent subtotal pancreatectomy. She was diagnosed as MNCC histologically.     

Successful surgical resection of solid cystic tumor of the pancreas with multiple liver metastases and a tumor thrombus in the portal vein

We report a case of solid cystic tumor of the pancreas with widespread liver metastases and a tumor thrombus in the portal vein. The patient was a 43-year-old woman. She was referred because of an upper abdominal mass and weight loss. Computed tomography disclosed a 10-cm cystic and calcified mass in the body and tail of the pancreas and multiple masses in the liver. She underwent a distal pancreatectomy with splenectomy, extended right lobectomy, and partial resection of the liver. All the tumors were completely resected despite the presence of 20 liver metastases. Histopathological studies showed a tumor thrombus in the intrahepatic portal vein. The patient is well without any signs of recurrence 8 months after the operation. Aggressive surgical resection is considered to yield a good outcome for solid cystic tumor with liver metastases and tumor thrombus of the portal vein.     

Spleen-preserving distal pancreatectomy with preservation of the splenic artery and vein for intraductal papillary-mucinous tumor (IPMT): three interesting cases

Preservation of the spleen at distal pancreatectomy has recently attracted considerable attention. Since our first trial and success with spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein for tumors of the pancreas and chronic pancreatitis, this procedure has been performed more frequently. Three patients with intraductal papillary-mucinous tumor underwent spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein. In this procedure, the splenic vein is identified behind the pancreas and the connective tissue membrane is cut longitudinally above the splenic vein. An important point is to remove the splenic vein from the pancreas from the body of the pancreas toward the spleen. In one patient with intraductal papillary-mucinous tumor in the body of the pancreas who had undergone distal gastrectomy for duodenal ulcer 32 years previously, residual proximal gastrectomy could be avoided with this procedure. In this case, the histological diagnosis was a pseudocyst, and epithelial dysplasia was found in other pancreatic ductuli. In another case, epithelia were borderline between hyperplasia and adenoma. In both of these cases, the histological diagnosis was different from the preoperative diagnosis. Even with advances in imaging techniques, diagnosis of a cystic lesion of the pancreas is still very difficult. Ordinary distal pancreatectomy with splenectomy would have been oversurgery in these two cases, which could be avoided using our procedure. Severe complications were not found in any of the three cases and the postoperative course was uneventful. The patients have been followed as outpatients without any recurrence. Spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein is easy and safe, and should be performed for some patients with intraductal papillary mucinous tumor of the pancreas.