Pancreatic acinar cell carcinoma with extension into the main pancreatic duct: a case report.

BACKGROUND: Pancreatic acinar cell carcinoma (PACC) is a rare exocrine malignant tumor. Its widespread intraductal extension into the main pancreatic duct (MPD) is also rare. CASE PRESENTATION: We report the case of a 71-year-old man with PACC with MPD extension. The patient was assessed with laboratory and radiographic investigations that facilitated a preoperative diagnosis. Endoscopic ultrasonography (EUS) and dynamic thin-slice multi-detector row computed tomography (MDCT) were useful for determining the resection line of the pancreas. EUS-guided fine needle aspiration (EUS-FNA) was also helpful in determining the tumor biology and treatment strategy. Distal pancreatectomy was performed. The MPD was occupied by the tumor 35 mm downstream and 5 mm upstream. Histopathologically, the pancreatic tail tumor extended continuously into the MPD. The tumor was solid with cells showing eosinophilic and granular cytoplasm, indicating the diagnosis of PACC. This is an interesting case of PACC with intraductal extension into the MPD. We discuss the possible mechanisms of tumor extension in this rare case together with a review of the literature. CONCLUSIONS: We describe a rare pancreatic acinar cell carcinoma that could be adequately treated using preoperative precise imaging and histopathological evaluations. When an intraductal tumor extension in the MPD is encountered, the diagnosis of a rare pancreatic tumor should be considered, as in our case.

A Case of Duodenal Neuroendocrine Tumor Accompanied by Gastrointestinal Stromal Tumors in Type 1 Neurofibromatosis Complicated by Life-Threatening Vascular Lesions.

BACKGROUND Type 1 neurofibromatosis (NF1) is known to be associated with not only neurogenic tumors but also gastrointestinal (GI) neoplasms. However, there are few reports on vascular lesions and the incidence is unknown. CASE REPORT We report here the case of a 45-year-old woman with a history of NF1 referred to our hospital for the purpose of detailed examination for positive fecal occult blood test. On the basis of the investigation reports, she was diagnosed with a neuroendocrine tumor (NET)-G1. We planned a subtotal stomach-preserving pancreaticoduodenectomy. The abdominal structures, including the vascular system, were abnormally fragile, and it was very difficult to achieve satisfactory hemostasis. The total amount of intraoperative blood loss was 7580 mL. Fulminant intra-abdominal bleeding occurred on postoperative day (POD) 3. Urgent angiography showed a rupture of the gastroduodenal artery. Transarterial embolization was performed, but the patient died of multiorgan failure on POD5. On histological examination, neurofibroma cells proliferating into the surrounding blood vessels were seen; moreover, immunohistochemistry staining with S-100 antibody showed positive neurofibroma cells surrounding the vascular wall. The pathological diagnosis was duodenal NET-G1 with multinodal involvement. CONCLUSIONS This case is a rare presentation of a NET with multiple gastrointestinal stromal tumors associated with NF1, which led to a fatal outcome due to the extreme fragility of the vessel walls. Since patients with NF1 might have vulnerable vessel walls, adequate surgical preparation for major surgical treatment is necessary.

A case of midgut volvulus related to adult intestinal malrotation found with weight loss after streptococcus infection: A case report and literature review.

INTRODUCTION AND IMPORTANCE: The incidence of intestinal malrotation is 1 in 6000 births, and 90% of cases occur within the first year of life. Adult cases are rare, with a reported incidence of 0.2%-0.5% of all cases. The significance of reporting this case is to recognize that some adult-onset cases require surgery even in the absence of intestinal necrosis. CASE PRESENTATION: A 36-year-old man was infected with streptococcus and treated with antibiotics. He developed appetite loss and his weight decreased 12 kg in 4 months. His abdomen was flat and soft with no tenderness. A computed tomography scan showed that the horizontal duodenal leg was not anchored to the retroperitoneum. Rotation of the mesentery, which was wrapped around the superior mesenteric artery in a clockwise direction, was observed, suggesting midgut volvulus. We performed emergency surgery and Ladd's procedure. CLINICAL DISCUSSION: A previous study reported that the most common symptom in the chronic course of intestinal malrotation was abdominal pain in 41.2% of cases, and weight loss was observed in only 2.6% of patients. The high degree of intestinal adhesion suggests that repeated torsion and release and the development of collateral vessels may have contributed to the asymptomatic course. CONCLUSION: Adult-onset intestinal malrotation should be considered as a differential diagnosis in the presence of weight loss and gastrointestinal symptoms. The timing of surgery is still controversial. In chronic cases, severe adhesion might be expected and laparoscopic surgery should be considered carefully.

[A Case of Chemotherapy with Low-Dose Paclitaxel and Bevacizumab for Elderly Recurrent Breast Cancer Patient].

An 86-year-old woman underwent mastectomy with sentinel lymph node biopsy for cStage ⅡA breast cancer. The subtype of tumor was triple negative breast cancer. Pulmonary metastasis was found 1 month after surgery. Chemotherapy was done because of her good performance status(PS)and her hope. Administration of S-1 produced SD status of tumor for 8 months. However, NCC-ST-439 was increased and tumor size was enlarged. Therefore, the second line of chemotherapy by low-dose- biweekly paclitaxel and bevacizumab was planned because of her high age and good PS. Thereafter, tumor maker levels dramatically decreased and lung metastasis turned to be small. This therapy had been continued without any severe adverse events for 9 months. Unfortunately, this therapy was failed because of proteinuria, but pulmonary metastasis kept favorable efficacy during administration. Biweekly low-dose paclitaxel and bevacizumab therapy can be safe and effective therapy even for elderly patient with recurrent and metastatic breast cancer.

Roles of the hexosamine biosynthetic pathway and pentose phosphate pathway in bile acid-induced cancer development.

Esophageal squamous cell carcinomas (ESCCs) as well as adenocarcinomas (EACs) were developed in rat duodenal contents reflux models (reflux model). The present study aimed to shed light on the mechanism by which bile acid stimulation causes cancer onset and progression. Metabolomics analyses were performed on samples of neoplastic and nonneoplastic tissues from reflux models, and K14D, cultivated from a nonmetastatic, primary ESCC, and ESCC-DR, established from a metastatic thoracic lesion. ESCC-DRtca2M was prepared by treating ESCC-DR cells with taurocholic acid (TCA) to accelerate cancer progression. The lines were subjected to comprehensive genomic analyses. In addition, protein expression levels of glucose-6-phosphate dehydrogenase (G6PD), nuclear factor kappa B (NF-κB) (p65) and O-linked N-Acetylglucosamine (O-GlcNAc) were compared among lines. Cancers developed in the reflux models exhibited greater hexosamine biosynthesis pathway (HBP) activation compared with the nonneoplastic tissues. Expression of O-GlcNAc transferase (OGT) increased considerably in both ESCC and EAC compared with nonneoplastic squamous epithelium. Conversely, cell line-based experiments revealed the greater activation of the pentose phosphate pathway (PPP) at higher degrees of malignancy. G6PD overexpression in response to TCA exposure was observed. Both NF-κB (p65) and O-GlcNAc were expressed more highly in ESCC-DRtca2M than in the other cell lines. Moreover, ESCC-DRtca2M cells had additional chromosomal abnormalities in excess of ESCC-DR cells. Overall, glucose metabolism was upregulated in both esophageal cancer tissue and cell lines. While bile acids are not mutagenic, chronic exposure seems to trigger NF-κB(p65) activation, potentially inducing genetic mutations as well as facilitating carcinogenesis and cancer progression. Glucose metabolism was upregulated in both esophageal cancer tissue and cell lines, and the HBP was activated in the former. The cell line-based experiments demonstrated upregulation of the pentose phosphate pathway (PPP) at higher degrees of malignancy. While bile acids are not mutagenic, chronic exposure seems to trigger G6PD overexpression and NF-κB (p65) activation, potentially inducing genetic mutations as well as facilitating carcinogenesis and cancer progression.

Recurrent biliary dissemination of colon cancer liver metastasis: a case report.

BACKGROUND: Most colorectal cancer liver metastases form nodules within the hepatic parenchyma, and hepatectomy is the only radical treatment for synchronous metastases. There is concern about intrabiliary tumor growth which may affect the surgical margin, resulting in local recurrence after hepatectomy for colorectal cancer liver metastasis; however, there has been no report of the dissemination in the bile duct after hepatectomy. Here, we report an unusual case of biliary dissemination of colorectal cancer that caused recurrent intrabiliary growth after hepatectomy, and discuss the management of intrabiliary metastasis of colorectal cancer. CASE PRESENTATION: A 69-year-old Japanese man underwent treatment for liver dysfunctions 3 years after aortic valve replacement. Computed tomography revealed an enhanced tumor within the hilar bile duct and dilatation of the left hepatic duct, typical of hilar cholangiocarcinoma. Endoscopic retrograde cholangiopancreatography revealed tumor shadow in his bile duct, and the cytology confirmed malignant cells in the bile. We performed extended left hepatectomy with bile duct resection; his postoperative course remained good without acute complications. After 3 months postoperatively, he was readmitted for subacute cholangitis and obstructive jaundice. Immediately, percutaneous transhepatic cholangiography drainage was performed, followed by cholangiography that exhibited intrabiliary tumor growth in the remnant liver. On immunohistochemical examination, tumor cells were positive for cytokeratin 20 and CDX2 but negative for cytokeratin 7. Then, computed tomography revealed an enhanced tumor-like lesion at the descending colon. After 3 months, left hemicolectomy was performed. Meanwhile, the percutaneous transhepatic cholangiography drainage fluid turned bloody, which was considered to be bleeding from a residual bile duct tumor. Accordingly, radiotherapy was initiated to prevent tumor bleeding around the hilar bile duct, but, unfortunately, the effects were short-lived, and cholangitis rebooted after 1 month leading to our patient's death due to septic liver failure. Autopsy revealed a remnant tumor in the bile duct, but no noticeable nodular metastasis was observed, except for a single small metastasis in the lower lobe of the left lung. CONCLUSIONS: The intrabiliary growth of metastatic colorectal cancer mimics cholangiocarcinoma occasionally. To date, as the effect of chemotherapy or radiotherapy remains uncertain, the complete resection of a bile duct tumor is the only method which could result in a better prognosis.

[Highly Advanced Gastric Cancer Leading to Immediate Perforation on Day Three of Chemotherapy with S-1 plus Oxaliplatin].

A 68-year-old woman was diagnosed with advanced gastric cancer with a type 3 deep ulcer of the middle stomach by endoscopy. An abdominal computed tomography scan revealed multiple lymph node metastases and peritoneal disseminations. The clinical stage was determined to be T4a(SE), N2P1M1(PER), H0 and stage IV . A gastrectomy was scheduled after 2 courses of S-1 plus oxaliplatin(SOX)with curative intent. On day 3 after initiatingSOX therapy, the patient complained of severe abdominal pain. Because the abdominal CT scan showed intra-abdominal free air and a defect in the gastric wall, we performed an emergency total gastrectomy. The defect in the gastric wall was about 1 cm in diameter and was located in the anterior wall of the lower body, consistent with the center of the tumor. The operative findings suggested that the perforation was caused by chemotherapy-induced necrosis of gastric cancer cells. The patient was discharged 16 days after surgery and received post-operative chemotherapy. Our findings suggest that the risk of gastric perforation should be considered when administeringchemotherapy to patients with advanced gastric cancer and a deep ulcer.

Impact of histone deacetylase 1 and metastasis-associated gene 1 expression in esophageal carcinogenesis.

Animal models are important for the development of novel therapies for esophageal cancer. Histone deacetylase 1 (HDAC1)/metastasis-associated gene (MTA1) complexes inhibit p53 acetylation and thus, inhibit p53-induced apoptosis. The aim of the present study was to evaluate HDAC1 and MTA1 expression in esophageal carcinogenesis in rats. The rats underwent a total gastrectomy followed by esophagojejunostomy to induce chronic duodenal content reflux esophagitis. The rats were sacrificed sequentially at 20, 30, 40 and 50 weeks post-surgery and the esophagi were examined. Immunohistochemical analysis was conducted to assess the expression and localization of HDAC1 and MTA1. At 20 weeks post-surgery, squamous proliferative hyperplasia and Barrett's metaplasia (BM) were observed. While, adenocarcinoma-associated BM and squamous cell carcinoma were observed at 30-50 weeks post-surgery. The nuclear expression of HDAC1 and MTA1 was observed in all of the stages of squamous carcinogenesis and adenocarcinogenesis, although not in the normal esophageal epithelium. The expression of HDAC1 and MTA1 may be involved in duodenoesophageal reflux-induced neoplastic transformation of the esophageal mucosa into cancer cells with squamous and adeno differentiation.

[A case of recurrence after resection of gastric cancer successfully treated by combination chemotherapy with CPT-11 and CDDP].

A 62-year-old man received total gastrectomy and splenectomy for gastric cancer in April 2008. The pathological diagnosis was stage IIIA(pT3N2M0). After the surgery, he was treated with oral administration of S-1 as postoperative adjuvant chemotherapy, but stopped after five months because of a drug eruption and general fatigue. He visited our hospital with complaints of abdominal pain and appetite loss in January 2010. Tumor marker(CEA)was elevated, and he was diagnosed as local recurrence with metastasis to the mediastinal/Virchow lymph node and thoracic vertebra after several examinations. He started to undergo combination chemotherapy with CPT-11 and CDDP. After three courses of treatment, local recurrence had almost disappeared and CEA level was normalized. After five courses of treatment, FDG-PET showed that all recurrent tumors had decreased in size and FDG uptake. We finished this combination chemotherapy after ten courses. After the treatment, PET-CT detected a slowly-growing right lung tumor. He received S6 segmentectomy of the right lung in May 2012. The pathological diagnosis was squamous cell carcinoma, and he was diagnosed as primary lung cancer(pT1aN0M0, stage I A). He was followed without showing any recurrence of gastric cancer in October 2012.

Management of postoperative hemorrhage associated with factor VIII inhibitor: report of a case.

This report presents a case that was successfully treated for acquired factor VIII inhibitor after extensive visceral surgery. A 71-year-old male who underwent surgery for bile duct cancer had active bleeding in the abdominal drainage tube on postoperative day (POD) 5, and prolonged activated partial thromboplastin time (aPTT) was detected (83.1 s) on POD 7. An extensive coagulation work-up revealed factor VIII deficiency (1 %), and a diagnosis of an acquired factor VIII deficiency was established when a factor VIII inhibitor of 8 Bethesda units was demonstrated. The patient was treated with activated prothrombin complex concentrate (aPCCs) and bloody discharge was stopped within 3 days. Inhibitor elimination was started using prednisolone on POD 20; rituximab, was administered on POD 74 and 81. Factor VIII inhibitor had disappeared by POD 124, and factor VIII (72 %) and aPTT recovered to 45.9 s. This case report demonstrated the efficacy of aPCCs and rituximab in the treatment of acquired hemophilia associated with visceral surgery.