Intrahepatic cholangiocarcinoma with arterial phase hyperenhancement and specialized tumor microenvironment associated with good prognosis after radical resection: A single-center retrospective study.

BACKGROUND: This single-center retrospective study aimed to clarify the clinical and pathologic background of mass-forming intrahepatic cholangiocarcinomas. METHODS: A total of 53 patients with mass-forming intrahepatic cholangiocarcinomas were selected from 2007 to 2021 and analyzed based on several parameters, including the preoperative computed tomography pattern (enhancement in the arterial phase of dynamic contrast-enhanced computed tomography), clinical data, and tumor microenvironment evaluated by immunohistochemistry. The hyperenhancement (n = 13) and hypoenhancement (n = 40) groups were defined using the 50% cutoff of tumors with higher attenuation than the liver parenchyma. RESULTS: The hyperenhancement group was characterized by a better overall survival than the hypoenhancement group (5-year survival: 86% vs 27%, respectively; P < .001) and by a higher infiltration of peritumoral (92% vs 58%; P = .020) and intratumoral CD3-positive T lymphocytes (85% vs 35%; P = .002). Conversely, the hypoenhancement group was characterized by a higher infiltration versus peritumoral CD163-positive tumor-associated macrophages (60% vs 8%; P = .001), peritumoral pentraxin 3-positive tumor-associated macrophages (50% vs 15%; P = .024), and intratumoral α-smooth muscle actin-positive cancer-associated fibroblasts (15% vs 68%; P = .001). A multiple regression analysis was performed to predict overall survival from the microenvironment, and the independent poor predictor factors were low intratumoral CD3-positive T lymphocytes (hazard ratio = 2.75), high peritumoral (hazard ratio = 2.38), and intratumoral CD163-positive tumor-associated macrophages (hazard ratio = 2.81) (all P values < 0.05). CONCLUSION: Compared with hypovascular, hypervascular mass-forming intrahepatic cholangiocarcinomas have better tumor immunity and prognosis.

Neoadjuvant and Adjuvant Treatments for Resectable and Borderline Resectable Pancreatic Ductal Adenocarcinoma: The Current Status of Pancreatic Ductal Adenocarcinoma Treatment in Japan.

Resection is the only curative treatment for pancreatic ductal adenocarcinoma (PDAC). Although the outcome of technically resectable PDAC has improved with advances in surgery and adjuvant therapy, the 5-year survival rate remains low at 20% to 40%. More effective therapy is needed. Almost 15 years ago, the National Comprehensive Cancer Network guidelines proposed a resectability classification of PDAC based on preoperative imaging. Since then, treatment strategies for PDAC have been devised based on resectability. The standard of care for resectable PDAC is adjuvant chemotherapy after R0 resection, as shown by the results of pivotal clinical trials. With regard to neoadjuvant treatment, several recent clinical trials comparing neoadjuvant treatment with upfront resection have been conducted on resectable PDAC and borderline resectable PDAC, and the benefits and efficacy of neoadjuvant treatment for pancreatic cancer has become clearer. The significance of neoadjuvant treatment for resectable PDAC remains controversial, but in borderline resectable PDAC the efficacy of neoadjuvant treatment has been further recognised, although the standard of care has not yet been established. Several promising clinical trials for PDAC are ongoing. This review presents previous and ongoing trials of perioperative treatment for resectable and borderline resectable PDAC, focusing on the difference between Asian and Western countries.

Prognostic Impact of Margin Status in Distal Cholangiocarcinoma.

BACKGROUND: A positive ductal margin is strongly associated with poor survival in patients with distal cholangiocarcinoma. However, the significance of the radial margin status and its effect on survival are not fully clarified. METHODS: All patients with distal cholangiocarcinoma who underwent pancreatoduodenectomy between January 2000 and December 2018 at Tokai University Hospital were retrospectively analyzed. Positive margins were divided into positive ductal margin and positive radial margin. RESULTS: One hundred and eight consecutive patients with distal cholangiocarcinoma underwent pancreatoduodenectomy. Margin-negative R0 resection was performed in 85 patients (79%). Twenty-three patients (21%) had a positive resection margin (R1 resection). The 5-year survival rate and median overall survival for patients with R0 resection and those with R1 resection was 64%, 98 months and 25%, 26 months, respectively. There was a significant difference in survival between patients with R0 resection and those with R1 resection (p < 0.001). Patients with positive radial margin (n = 10) had a significantly worse outcome than those with positive ductal margin (n = 13) (p = 0.016). Univariate analysis showed that R1 resection, lymph node metastasis, tumor depth, portal vein invasion, pancreatic invasion, lymphatic invasion, and venous invasion were significant prognostic factors. Multivariate analysis confirmed that R1 resection and nodal involvement were significant independent prognostic indicators after surgical resection for distal cholangiocarcinoma. CONCLUSIONS: Positive surgical margin and nodal involvement were the strongest predictors of poor survival in patients with distal cholangiocarcinoma. Patients with a positive radial margin had a significantly worse outcome than those with a positive ductal margin.

Prognostic Impact of Positive Peritoneal Lavage Cytology on Resectable Pancreatic Body and Tail Cancer: A Retrospective Study.

BACKGROUND: The prognostic impact of positive peritoneal lavage cytology on pancreatic cancer is unclear. Therefore, this study aimed to evaluate its impact in resectable pancreatic body and tail cancer. METHODS: Between January 2006 and December 2019, 97 patients with pancreatic body and tail cancer underwent peritoneal lavage cytology and curative resection at our institution. We analyzed the impact of positive peritoneal lavage cytology on clinicopathological factors and on the prognosis of pancreatic body and tail cancer. RESULTS: Malignant cells were detected in 14 patients (14.4%) using peritoneal lavage cytology. In these patients, the tumor diameter was significantly larger (p < 0.001) and anterior serosal invasion (p = 0.034), splenic artery invasion (p = 0.013), lympho-vessel invasion (p = 0.025), and perineural invasion (p = 0.008) were significantly more frequent. The R1 resection rate was also significantly higher in patients with positive peritoneal lavage cytology than in negative patients (p = 0.015). Positive peritoneal lavage cytology had a significantly poor impact on overall survival (p = 0.001) and recurrence-free survival (p < 0.001). This cytology was also an independent poor prognostic factor for recurrence (p = 0.022) and was associated with peritoneal dissemination and liver metastasis. CONCLUSIONS: Positive peritoneal lavage cytology is considered to be indicative of more systemic disease in patients with resectable pancreatic body and tail cancer than in patients with negative peritoneal lavage cytology. Early detection of pancreatic cancer before it develops micrometastases is important to improve prognosis, and CY+ patients require more intensive multimodality treatment than standard treatment for resectable pancreatic cancer.

Indications for resection of recurrent lesions in patients with distal cholangiocarcinoma based on prognostic factors: a single-institute retrospective study and brief literature review.

BACKGROUND: To evaluate the effectiveness of surgery for recurrent distal cholangiocarcinoma and determine surgical indications based on prognostic factors for the recurrence of distal cholangiocarcinoma. METHODS: We analysed the outcomes of 101 patients who underwent surgical resection for distal cholangiocarcinoma between 2000 and 2018. The clinicopathological factors and prognosis of primary and recurrent distal cholangiocarcinoma were investigated. RESULTS: Of the 101 patients with resected distal cholangiocarcinoma, 52 (51.5%) had relapsed. Seven (13.5%) and 45 patients (86.5%) underwent resection of recurrent lesions and palliative therapy, respectively. There were no major complications requiring therapeutic intervention after metastasectomy. The median overall survival in patients with and without surgery for recurrent lesions was 83.0 (0.0-185.6) and 34 months (19.0-49.0), respectively. Therefore, patients who had undergone surgery for recurrent lesions had a significantly better prognosis (p = 0.022). Multivariate analyses of recurrent distal cholangiocarcinoma revealed that recurrence within one year was an independent predictor of poor survival. Resection of recurrent lesions improved prognosis. CONCLUSIONS: Radical resection in recurrent distal cholangiocarcinoma may improve the prognosis in selected patients. Although time to recurrence is considered an important factor, the small number of cases of recurrence and resection of recurrent lesions in this study makes it difficult to conclude which patients are best suited for resection of recurrent lesions. This issue requires clarification in a multicentre prospective study, considering patients' background, such as the recurrence site and number of metastases.

True Carcinosarcoma of the Gallbladder: A Case Report and Brief Review of the Literature.

INTRODUCTION: Carcinosarcoma of the gallbladder (CSGB) is very rare, accounting for less than 1% of gallbladder malignancies. Therefore, the biological behavior is not well known. We report the case of a patient with CSGB who showed long-term survival after treatment with surgery and postoperative adjuvant chemotherapy. CASE PRESENTATION: A 79-year-old man was referred to our department with suspected gallbladder cancer after undergoing positron emission tomography-computed tomography (PET-CT) scan for preoperative examination of lung cancer, which showed strong accumulation in the gallbladder. Abdominal contrast-enhanced computed tomography (CT) demonstrated a heterogeneous enhanced, 25-mm mass in the anterior wall of the gallbladder fundus. Cholecystectomy and hepatoduodenal mesenteric lymph node sampling revealed a polypoid tumor. Histopathological findings showed a mixture of adenocarcinoma and sarcoma with spindle-shaped cells. Immunohistochemical s taining of the s arcoma s howed negative results for the epithelial markers and positive results for the mesenchymal markers, leading to a diagnosis of true CSGB. We administered S-1 as postoperative adjuvant chemotherapy and was reported to be alive 45 months after surgery without recurrence. CONCLUSION: CSGB has a poor prognosis, but if radical resection can be performed, there is a possibility of long-term survival. Further case studies and treatment options are needed to help understand this disease.

Prediction of non-functioning pancreatic neuroendocrine tumor grades with fractal analysis of preoperative contrast-enhanced computed tomography images.

PURPOSE: To assess intra-tumoral heterogeneity (ITH) via fractal analysis of preoperative contrast-enhanced computed tomography (CT) images to predict pathological grades in non-functioning pancreatic neuroendocrine tumors (NF-PNETs) and verify its impact on patient survival. METHODS: This retrospective study enrolled 40 patients with NF-PNET resected in our institution during a period from July 2005 to December 2018, except functioning tumors, unidentified tumors in CT, patients without preoperative contrast-enhanced CT. CT images were analyzed using plugin software for calculating fractal dimension (FD), and the maximum value was denoted as "FDmax," and compared with pathological grades and patient survival between G1 and G2/3 group separating according to two different Ki-67 index thresholds (3% and 5%). All CT images were acquired in three-phases and arterial phase images were examined. RESULTS: Ki-67 index and FDmax showed a direct correlation with significance (p < 0.01). The mean FDmax of the G2/3 tumor group was significantly higher than that of the G1 tumor group (p < 0.01 in both 3% and 5% thresholds). In the ROC analysis, FDmax showed 0.773 of AUC, and cut-off value of 1.036 reported 62.5 % sensitivity, 90.0 % specificity, 86.2 % PPV, and 70.6 % NPV to distinguish G2/3 patients. The high-FD (≥1.036) group showed a significantly shorter disease-free survival (DFS) than the low-FD group (p = 0.0128). In multivariate analysis of prognostic factors, high FD was the only significant factor for DFS (HR, 5.793; 95 % CI: 1.213-27.664; p = 0.028). CONCLUSIONS: The tumor's FDmax using CE-CT analysis might be a potential biomarker for preoperative prediction of G2/3 tumors, and predicting recurrence.

Significance of pulmonary resection in patients with metachronous pulmonary metastasis from pancreatic ductal adenocarcinoma: a retrospective cohort study.

BACKGROUND: Pulmonary metastases from pancreatic ductal adenocarcinoma (PDAC) are relatively rare. Systemic chemotherapy is the first choice of treatment in patients with distant metastases, and the role of metastasectomy is controversial. The aim of the present study was to evaluate the outcome of patients with pulmonary metastases after resection of PDAC and the indications for metastasectomy. METHODS: We retrospectively analysed patients with pulmonary metastases as the first recurrence after resection of primary PDAC between January 2006 and December 2018. Clinical data were obtained from the patients' medical records. Relapse-free survival (RFS) and overall survival (OS) were analysed using the Kaplan-Meier method, and statistical significance was evaluated by the log-rank test. RESULTS: Of the 417 patients with resected PDACs, 24 (7.9%) had pulmonary metastases. Six patients (25.0%) underwent pulmonary resection and 18 (75.0%) received systemic chemotherapy and best supportive care. There were no major complications requiring therapeutic intervention after pulmonary resection. The median RFS was 24.0 months (95% CI 10.8-37.2), and the 1-, 3-, and 5-year RFS rates were 66.7%, 33.3%, and 4.2%, respectively. The median OS was 50.0 months (95% CI 15.9-84.1), and the 1-, 3-, and 5-year OS rates were 95.8%, 70.3%, and 46.4%, respectively. All patients with resected pulmonary metastases were alive at the end of the study, whereas the median OS of the patients who did not undergo resection was 37.0 months (95% CI 34.4-39.6). Therefore, patients with resected pulmonary metastases had a significantly better prognosis (p = 0.008). CONCLUSIONS: Pulmonary resection may improve the prognosis in selected patients with pulmonary metastases from PDAC. However, the present study is based on a small number of patients and may include a selection bias; therefore, a multi-institutional prospective study is needed to clarify the indications for pulmonary resection.

[A case of simultaneous multicentric cancer in the gallbladder duct and the common bile duct without pancreaticobiliary maljunction].

A 68-year-old man who underwent cholecystectomy for acute cholecystitis and was diagnosed with gallbladder duct carcinoma was referred to our hospital. Postoperative computed tomography showed thickening of the middle to lower bile duct without any tumorous lesions. Endoscopic retrograde cholangiopancreatography and intraductal ultrasonography revealed irregular wall thickening of the lower bile duct and apparent infiltration of gallbladder duct tumor to the common bile duct without pancreaticobiliary maljunction. Subtotal stomach-preserving pancreaticoduodenectomy was performed. Pathological examination showed papillary adenocarcinoma and tubular adenocarcinoma in the gallbladder duct and BilIN-3 lesion in the distal bile duct. The final diagnosis was biliary simultaneous multicentric cancer without pancreaticobiliary maljunction.

Combined primary hepatic neuroendocrine carcinoma and hepatocellular carcinoma: case report and literature review.

BACKGROUND: Hepatocellular carcinoma (HCC) can grow in a mosaic pattern, often combined with various non-hepatocellular cells. However, HCC combined with a neuroendocrine carcinoma (NEC) component is rarely reported, and its clinical features, origin, diagnosis, and behavior have not been established. In the literature, mixed HCC-NEC tumors are categorized as either collision type or combined type, depending on their microscopic features. Here, we report a patient with a combined-type HCC-NEC tumor. CASE PRESENTATION: An asymptomatic 84-year-old woman was found to have a solid mass in the right lobe of the liver. Laboratory and radiologic examinations showed typical findings of HCC, including arterial-phase enhancement, and portal- and delay-phase washout. She was treated by partial laparoscopic hepatectomy of segment 5. Pathological examination showed that the tumor was predominantly HCC, partly admixed with an NEC component. A transitional zone between the HCC and NEC tissues was also observed. The tumor was finally diagnosed as a combined-type primary mixed NEC-HCC tumor. After the preoperative diagnosis, the patient underwent somatostatin receptor scintigraphy to detect the primary NEC lesion, but no accumulation was found in any other part of her body. She has been free of recurrence for 9 months since the surgery. CONCLUSION: Mixed HCC-NEC tumors are extremely rare, and correct diagnosis requires multidisciplinary collaboration. The accumulation of further cases is needed to help understand the exact pathology, diagnosis, and treatment of this disease.

Adult Ileo-ileal Intussusception Due to Inflammatory Fibroid Polyp: A Case Report.

INTRODUCTION: Intussusception occurs when one part of the intestines slides into the adjacent intestine resulting in bowel obstruction. It is a rare condition in adults, accounting for only 5% of all intussusceptions. It has multiple causes, with inflammatory fibroid polyps (IFPs) very infrequently being the cause. We present a rare case of intussusception in an adult due to an IFP. CASE PRESENTATION: A 72-year-old woman visited our hospital complaining of abdominal pain. Abdominal contrast-enhanced computed tomography (CT) demonstrated an ileo-ileal intussusception due to a round mass. An emergency surgery involving a partial ileal resection with laparoscopic assistance was performed. Pathological findings of the tumor showed proliferation of spindle-shaped cells, edematous stroma, dilation of lymphatic vessels, and infiltration of inflammatory cells, which were mainly eosinophils. Immunohistochemistry was positive for vimentin and SMA and negative for CD117, CD34, S-100, and desmin. Based on these findings, the tumor was diagnosed as an IFP. CONCLUSION: Bowel obstruction in adults due to intussusception is rare, and those due to IFPs are even more rare. Preoperative diagnosis of IFP is difficult, but surgeons must keep in mind that it can be a cause of adult intussusception.

Intussusception due to hematogenous metastasis of hepatocellular carcinoma to the small intestine: A case report.

BACKGROUND: The commonest sites of extrahepatic metastases from hepatocellular carcinoma (HCC) are the lungs, bones, adrenal glands, and regional lymph nodes. Hematogenous metastasis to the gastrointestinal (GI) tract is a rare condition in patients with HCC, and the prognosis is usually poor. We report, herein, an extremely rare case of a patient with intussusception due to hematogenous metastasis of HCC to the ileum and his long-term survival with multidisciplinary therapy. CASE SUMMARY: The patient was a 71-year-old man with a history of chronic hepatitis B, who had undergone three surgeries for HCC. He was treated with sorafenib for peritoneal metastases of HCC. He was admitted to our hospital with chief complaints of abdominal pain and vomiting. Abdominal contrast-enhanced computed tomography imaging revealed a small intestinal tumor, presenting with intussusception and small bowel obstruction. Conservative treatment was started, but due to repeated exacerbation of symptoms, surgery was planned on the 28th d of hospitalization. Partial ileal resection without reducing the intussusception and end-to-end anastomosis was performed. On histological examination, tumor cells were not observed on the serosal surface, but intravascular invasion of tumor cells was seen. Immunohistochemistry was positive for immunohistochemical markers, and a diagnosis of hematogenous metastasis of HCC to the ileum was made. He remains alive 82 mo after the first surgery. CONCLUSION: Prognosis of HCC patients with GI tract metastasis is usually poor, but in some cases, multidisciplinary therapy may prolong survival.

[Multidisciplinary Therapy for Unresectable Pancreatic Cancer].

The treatment outcomes of unresectable pancreatic cancer(URPC)have improved due to the advent of gemcitabine with nab-paclitaxel(GnP)and FOLFIRINOX as first-line therapy. There have been increasing reports of URPC responding to chemotherapy or chemoradiation and that conversion surgery(CS)can help to achieve long-term survival. This study aims to assess the treatment outcomes of URPC in our department and consider CS adaptation. Thirty-six patients with URPC who were treated with GnP or FOLFIRINOX between 2015 and 2018 were included in this retrospective analysis. Thirty-five patients had GnP, while 1 patient had FOLFIRINOX. The median age of the patients was 68.0 years and included 17 males and 19 females. Twenty-eight of the tumors were located in the pancreas head and 8 in the body-tail. Twenty-one cases were locally advanced(UR-LA), and 15 cases had distant metastases(UR-M). CS was performed in 9 cases(25.0%). The 2-year survival rate for patients that underwent CS was 53.3%, and 34.1% for patients that did not undergo CS. The prognosis of patients who underwent CS tended to be better, but there was no significant difference(p=0.141). In the patients that underwent CS, there were cases of early recurrence in which the period of preoperative chemotherapy was short, and the tumor markers were not normalized. Therefore, it is thought that prolonging preoperative treatment could help to select more suitable patients for CS.

Lung metastases from intraductal papillary neoplasm of the bile duct: a case report.

BACKGROUND: Intraductal papillary neoplasm of the bile duct (IPNB) is considered a pre-cancerous biliary lesion and/or an early cancer lesion, although its classification remains unclear. The 2019 revised edition of the World Health Organization Classification of Tumors of the Digestive System proposed type 1 and type 2 as new classification categories, and meta-analyses and/or multi-center cohort studies are beginning to be reported. However, treatment for IPNB recurrence and metastasis remains unclear. CASE PRESENTATION: A 60-year-old man who was referred to our hospital after a suspected liver tumor was diagnosed using abdominal ultrasonography. Imaging findings revealed an irregularly shaped tumor in segment 5 (S5) of the liver (size 20 mm). The S5 lesion was suspected as IPNB, and segmentectomy was performed. The pathological findings revealed invasive carcinoma derived from IPNB, and immunohistochemistry revealed positive expression of MUC1, MUC5AC, and MUC6, but negative expression of CDX2 and MUC2. At 9 months after the surgery, computed tomography revealed a tumor in the right bile duct, which was diagnosed as liver recurrence of IPNB, and right hepatectomy was performed. The histopathological findings were the same as for the first resected specimen (i.e., IPNB). At 45 months after the second surgery, computed tomography revealed nodules in both lungs, which were diagnosed as lung metastases from IPNB and resected in two separate procedures. The pathological findings were metastatic carcinoma from IPNB for both lung lesions. The patient is currently alive and undergoing adjuvant chemotherapy (S-1), which was initiated 64 months after the first resection and 12 months after resection of the lung metastases. CONCLUSION: We encountered a rare case of lung metastases from IPNB, which were diagnosed immunohistologically. Because IPNB is generally a slow-growing tumor, resection may be feasible for IPNB recurrence and/or metastasis, which may be detected during long-term follow-up. Thus, even if resection is performed for primary IPNB, additional surgical treatment may be feasible in this setting.

[Radical Resection of a Locally Advanced Unresectable Pancreatic Adenosquamous Carcinoma after Treatment with Systemic Chemotherapy].

A 64-year-old man visited our hospital because of weight loss and after being detected with a tumor in the pancreatic body and tail on abdominal ultrasonography at a nearby hospital. Abdominal contrast-enhanced CT revealed a 45 mm tumor with celiac artery invasion in the pancreatic body and tail. This case was diagnosed as unresectable pancreatic cancer. After 2 regimens of systemic chemotherapy(5 courses of gemcitabine plus nab-paclitaxel and 2 courses of S-1), CT revealed a partial response(ie, reduction in the tumor size). Therefore, distal pancreatectomy was performed with en bloc celiac axis resection, portal vein resection, and left adrenalectomy, as well as lymph node dissection. The pathological diagnosis was adenosquamous carcinoma, and R0 resection was achieved. The histological response was Grade 1b. However, the patient developed lung metastasis 16 months after surgery and liver metastasis 18 months after surgery; he died 33 months after starting treatment.

Conversion surgery for an initially unresectable, locally advanced pancreatic cancer after induction chemotherapy and carbon-ion radiotherapy: a case report.

BACKGROUND: Pancreatic cancer has a very high mortality rate worldwide, and about 30-40% of all patients have extensive vascular involvement at initial diagnosis that precludes surgical intervention. Here, we describe our experience in a patient with locally advanced pancreatic cancer (LAPC) who underwent R0 conversion surgery after undergoing a combination of chemotherapy and carbon-ion radiotherapy (CIRT), which led to long-term relapse-free survival (23 months). CASE PRESENTATION: A 41-year-old woman presented a month ago with epigastralgia referred to our facility and was subsequently diagnosed with pancreatic cancer cStage III (Ph, TS2 (35 mm), cT4, cCH1, cDU1, cS1, cRP1, cPL1, cVsm0, cAsm1, cN0, cM0) that was also categorized as an unresectable LAPC. She immediately underwent 3 cycles of induction chemotherapy (gemcitabine + nanoparticle albumin-bound (nab-) paclitaxel) followed by CIRT with concurrent gemcitabine. Although significant shrinkage of the primary tumor occurred, frequent cholangitis due to duodenal stenosis of unknown etiology prevented continued chemotherapy, and 9 months after the first visit, she underwent a radical, subtotal, stomach-preserving, pancreaticoduodenectomy (SSPPD). Histopathologic examination of the resected tissue revealed a R0 resection with a histological response of Evans grade IIB. She was administered an almost full dose of S-1 as adjuvant chemotherapy for 6 months and has shown no signs of recurrence in 23 months. CONCLUSIONS: We report a first case of successful conversion surgery for an initially unresectable LAPC after rapid induction GEM + nab-PTX chemotherapy followed by CIRT. Rapid induction GEM + nab-PTX chemotherapy followed by CIRT for LAPC might be a safe and effective treatment option.

Rectal Neuroendocrine Tumor with Synchronous Pancreatic Metastasis: A Case Report.

INTRODUCTION: Gastrointestinal neuroendocrine tumors (GI-NETs) often show hematogenous metastasis, with the liver being the most common metastatic site; however, metastasis to the pancreas is rare. CASE PRESENTATION: We report a rare case of rectal NETs with pancreatic metastases in a 75-year-old man who presented with a chief complaint of constipation. Imaging and endoscopic findings revealed a rectal submucosal tumor, a pancreatic hypovascular mass, and multiple liver masses. The rectal lesion and pancreatic lesions were diagnosed as neuroendocrine tumors using biopsy and endoscopic ultrasound fine-needle aspiration, respectively. Synchronous rectal NET and pancreatic NET (P-NET) with liver metastasis of either of these two were preoperatively diagnosed. A two-stage surgery was performed, comprising abdominoperineal resection and distal pancreatectomy. Pre-operative imaging findings showed a solitary mass in the pancreas, although the resected specimen contained multiple lesions. Immunohistochemical staining of the resected rectal and pancreatic lesions showed that both were synaptophysin positive and chromogranin A (CgA) negative. Generally, rectal NET cells are positive for synaptophysin and negative for CgA, while the majority of P-NETs are positive for both. The final diagnosis was rectal NETs with pancreatic and liver metastases. Till date, there have been no reports on the outcomes in patients with pancreatic metastasis of GI-NETs. CONCLUSIONS: More case reports on metastatic NETs are needed to arrive at a consensus for a standardized treatment regimen.

Laparoscopic splenopancreatectomy for an endocrine tumor with cystic changes: a case report.

The biological behavior of a cystic pancreatic endocrine neoplasm is less aggressive than that of pancreatic neuroendocrine neoplasms, and its prognosis is better. Limited surgery should be considered for cystic pancreatic endocrine neoplasms that are not accompanied preoperatively by lymph node or distant metastasis.

Undifferentiated carcinoma of the ampulla of Vater.

Undifferentiated carcinoma of the ampulla of Vater is a rare disease with unclear and clinical characteristics and prognosis. Here, we report the case of a 61-year-old man with undifferentiated carcinoma of the ampulla of Vater. He presented to our hospital with an increase in hepatobiliary system enzymes that was detected during a health check-up. Imaging and endoscopy demonstrated a tumor with ulcer in the ampulla of Vater, which was diagnosed as a carcinoma by biopsy. No distant metastasis was observed. Subtotal stomach-preserving pancreaticoduodenectomy was performed. Undifferentiated carcinoma was confirmed based on the presence of small round atypical cells with the formation of a solid alveolar lesion on histopathological examination and immunohistochemical staining that was positive for CAM 5.2 but negative for chromogranin A and synaptophysin. The tumor infiltrated the duodenum, but not the pancreas; no lymph node metastasis was observed. However, liver metastases were detected 2 months postoperatively. Chemotherapy was performed, and the tumor size temporality decreased; however, it grew in size again, and the patients subsequently died of the primary disease 15 months postoperatively. Undifferentiated carcinoma of the ampulla of Vater is a very rare histological type. More number of cases is necessary to clarify optimal treatment.

Carcinosarcoma of the ampulla of Vater: a case report and literature review.

BACKGROUND: Carcinosarcoma of the ampulla of Vater is extremely rare, and to the best of our knowledge, this is the third reported study. CASE PRESENTATION: The patient was a 73-year-old man, who presented with a chief complaint of dark urine. After a work-up, we suspected duodenal papillary cancer and performed a subtotal stomach-preserving pancreaticoduodenectomy with lymph node dissection. Immunohistochemically, the sarcomatous atypical cells were diffusely positive for cytokeratin AE1&3 and vimentin and focally positive for α-smooth muscle actin; these cells were also negative for desmin, CD34, DOG1, c-kit, and S100. From these findings, we diagnosed the patient with so-called carcinosarcoma. There was no lymph node metastasis. CONCLUSIONS: Carcinosarcoma of the ampulla of Vater has a poor prognosis, and lymph node metastases are often seen. For the complete cure of carcinosarcoma of the ampulla of Vater, resection with the dissection of the lymph nodes may be necessary.