Giant primary angiosarcoma of the small intestine showing severe sepsis

Primary malignant tumors of the small intestine are rare,comprising less than 2%of all gastrointestinal tumors.An 85-year-old woman was admitted with fever of 40℃ and marked abdominal distension.Her medical history was unremarkable,but blood examination showed elevated inflammatory markers.Abdominal computed tomography showed a giant tumor with central necrosis,extending from the epigastrium to the pelvic cavity.Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected.Because no improvement occurred in response to antibiotics,surgery was performed.Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity.The giant tumor was a solid tumor weighing 3490 g,measuring24 cm×17.5 cm×18 cm and showing marked necrosis.Histologically,the tumor comprised spindle-shaped cells with anaplastic large nuclei.Immunohistochemical studies showed tumor cells positive for vimentin,CD31,and factorⅧ-related antigen,but negative for c-kit and CD34.Angiosarcoma was diagnosed.Although no postoperative complications occurred,the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively.The prognosis of small intestinal angiosarcoma is very poor,even after volume-reducing palliative surgery.

Clinical impact of chemotherapy to improve tumor microenvironment of pancreatic cancer

A perioperative multimodal strategy including combination chemotherapy and radiotherapy, in addition to surgical resection, has been acknowledged to improve patient prognosis. However chemotherapy has not been actively applied as an immunomodulating modality because of concerns about various immunosuppressive effects. It has recently been shown that certain chemotherapeutic agents could modify tumor microenvironment and host immune responses through several underlying mechanisms such as immunogenic cell death, local T-cell infiltration and also the eradication of immune-suppressing regulatory cells such as regulatory T cells(Tregs) and myeloid-derived suppressor cells. With the better understanding of the cell components in the tumor microenvironment and the effect of chemotherapy to improve tumor microenvironment, it has been gradually clear that the chemotherapeutic agents is two-edged sword to have both immune promoting and suppressing effects. The cellular components of the tumor microenvironment include infiltrating T lymphocytes, dendritic cells, regulatory T cells, tumor associated macrophages, myeloid derived suppressor cells and cancer associated fibroblasts. Based on the better understanding of tumor microenvironment following chemotherapy, the treatment protocol could be modified as personalized medicine and the prognosis of pancreas cancer would be more improved utilizing multimodal chemotherapy. Here we review the recent advances of chemotherapy to improve tumor microenvironment of pancreatic cancer, introducing the unique feature of tumor microenvironment of pancreatic cancer, interaction between anti-cancer reagents and these constituting cells and future prospects.

Hilar cholangiocarcinoma with intratumoral calcification: A case report

This report describes a rare case of hilar cholangiocarcinoma with intratumoral calcification that mimicked hepatolithiasis. A 73-year-old man presented to a local hospital with a calcified lesion in the hepatic hilum. At first,hepatolithiasis was diagnosed,and he underwent endoscopic stone extraction via the transpapillary route. This treatment strategy failed due to biliary stricture. He was referred to our hospital,and further examination suggested the existence of cholangiocarcinoma. He underwent left hepatectomy with caudate lobectomy and extrahepatic bile duct resection. Pathological examination revealed hilar cholangiocarcinoma with intratumoral calcification,while no stones were found. To the best of our knowledge,only one case of calcified hilar cholangiocarcinoma has been previously reported in the literature. Here,we report a rare case of calcified hilar cholangiocarcinoma and reveal its clinicopathologic features.

Ganglioneuroblastoma arising within a retroperitoneal mature cystic teratoma

We discuss an extremely rare case of ganglioneuroblastoma arising within a retroperitoneal mature cystic teratoma. Radiological examinations showed a cystic tumor sandwiched between the pancreas and left kidney. Surgery was scheduled because the tumor seemed to have originated from the pancreas. En-block resection of the tumor with distal pancreatectomy, splenectomy, and left adrenalectomy was performed. In terms of macroscopic appearance, the tumor mainly consisted of a unilocular cystic mass, but the presence of a smaller, solid mass was also noted within the tumor. Histopathologic examination confirmed that the cystic mass was consistent with a mature cystic teratoma of the retroperitoneum, and in addition, a ganglioneuroblastoma was evident in the solid component. Histopathologically, the ganglioneuroblastomatous area was intimately associated with dermoid tissue of the mature cystic teratoma, thus this case was diagnosedto be a mature cystic teratoma with malignant transformation. To best of our knowledge, this is the first reported case of ganglioneuroblastoma arising in a mature cystic teratoma.

Cervical Castleman's disease mimicking lymph node metastasis of esophageal carcinoma

Castleman's disease(CD) is an uncommon benign lymphoproliferative disorder of unknown etiology. A rare case of cervical CD diagnosed at lymph node dissection for esophageal carcinoma is reported. An esophageal tumor was identified in a 67-year-old man during a follow-up examination after surgery for oral carcinoma. Esophagoscopy revealed a type 1 tumor in the cervical esophagus. Histology of esophagoscopic biopsies indicated squamous cell carcinoma. Contrastenhanced computed tomography revealed swollen lymph nodes of the right cervical region. No distant metastasis was detected. Esophageal carcinoma, T2N2M0, Stage ⅢA was diagnosed. Neoadjuvant chemotherapy was recommended, but the patient rejected the chemotherapy. The patient underwent laparoscopic-assisted transhiatal esophagectomy. The histopathological diagnosis was moderately differentiated squamous cell carcinoma with pT1bN0M0, Stage ⅠA. On histology, the swollen lymph nodes of the right cervical region revealed CD. The patient's postoperative course was relatively good.

Totally Laparoscopic Total Gastrectomy with D2 Lymphadenectomy for Advanced Gastric Cancer

Introductions: Gastrectomy, which is the standard surgical procedure for gastric cancer, has gradually come to be performed laparoscopically. Laparoscopic distal gastrectomy (LDG) has been adopted gradually and performed for advanced gastric cancer. However, laparoscopic total gastrectomy (LTG) has not been as widely accepted as LDG due to technical difficulties, especially with reconstruction and proper D2 lymphadenectomy. The purpose of the current study was to determine the utility of TLTG with concomitant splenectomy and D2 lymphadenectomy (TLTGS) for advanced gastric cancer (AGC). Materials and Methods: Between January 2006 and May 2014, 10 consecutive patients who underwent TLTGS for AGC and 76 patients who underwent TLTG with D1 lymphadenectomy were included in this study. These two groups were compared in terms of perioperative results, with assessment of intraoperative and postoperative outcomes. Results: There were no significant differences in patients’ characteristics between the two groups. Operative time was longer in the TLTGS group than in the TLTG group. However, the rate of patients with postoperative complications including major complications was not different between the groups, and no patient in the TLTGS group had anastomotic leakage or pancreatic fistula. Conclusions: In the short-term, TLTGS had good postoperative outcomes and was useful and acceptable for AGC.

胆道肿瘤临床实践指南(英文第三版)

日本肝胆胰外科学会(JSHBPS)于2007年出版第一版胆道肿瘤(胆管癌、胆囊癌及壶腹癌)临床实践指南,于2014年更新第二版,2021年英文第三版胆道肿瘤临床实践指南围绕6个主题提出若干临床问题,基于相关循证医学证据并组织专家讨论后,最终确定推荐意见、推荐强度以及推荐说明。根据证据推荐分级的评估、制订与评价(GRADE)系统,推荐强度被分为1级(强)或者2级(弱)。英文第三版胆道肿瘤临床实践指南中提出的31个临床问题涵盖:(1)预防性治疗;(2)诊断;(3)胆道引流;(4)外科治疗;(5)化疗;(6)放疗。31个临床问题中,14个问题给予推荐强度为强的推荐意见,14个问题给予推荐强度为弱的推荐意见,剩余的3个问题未给予推荐意见。每条推荐意见都进行推荐强度说明。最新版指南基于循证医学证据,为临床提供了重要建议。未来与癌症登记数据库协作将是评估指南和建立新证据的关键。

Validation study of postoperative liver failure and mortality risk scores after liver resection for perihilar cholangiocarcinoma

Background:Surgery for perihilar cholangiocarcinoma(PHCC)remains a challenging procedure with high morbidity and mortality.The Academic Medical Center(Amsterdam UMC)and Memorial Sloan Kettering Cancer Center proposed a postoperative mortality risk score(POMRS)and post-hepatectomy liver failure score(PHLFS)to predict patient outcomes.This study aimed to validate the POMRS and PHLFS for PHCC patients at Hokkaido University.Methods:Medical records of 260 consecutive PHCC patients who had undergone major hepatectomy with extrahepatic bile duct resection without pancreaticoduodenectomy at Hokkaido University between March 2001 and November 2018 were evaluated to validate the PHLFS and POMRS.Results:The observed risks for PHLF were 13.7%,24.5%,and 39.8%for the low-risk,intermediate-risk,and high-risk groups,respectively,in the study cohort.A receiver-operator characteristic(ROC)analysis revealed that the PHLFS had moderate predictive value,with an analysis under the curve(AUC)value of 0.62.Mortality rates based on the POMRS were 1.7%,5%,and 5.1%for the low-risk,intermediate-risk,and high-risk groups,respectively.The ROC analysis demonstrated an AUC value of 0.58.Conclusions:This external validation study showed that for PHLFS the threshold for discrimination in an Eastern cohort was reached(AUC>0.6),but it would require optimization of the model before use in clinical practice is acceptable.The POMRS were not applicable in the eastern cohort.Further external validation is recommended.