Small cell carcinoma of the ampulla of vater.

Primary small cell carcinoma of the periampullary region is rare. Only four patients have been reported previously. The authors examined a patient with ampullary small cell carcinoma using a range of immunocytochemical stains. This tumor shows morphologic and neuroendocrine features similar to its pulmonary and extrapulmonary counterparts.     

Embryonal rhabdomyosarcoma of the ampulla of vater

The first case to appear in the world literature is described of an embryonal rhabdomyosarcoma in a child arising from the region of the ampulla of Vater and projecting into the lumen of the duodenum.     

Small non-cleaved follicular cell lymphoma

A retrospective clinical and histologic analysis of data on 60 cases of lymphoma of small non-cleaved follicular center cells (Lukes-Collins classification) was carried out. 19 cases were classified as Burkitt's lymphoma and 41 as non-Burkitt's. Non-Burkitt's lymphomas showed more diverse cellularity. Age distribution was bimodal in both subgroups. Half the patients irrespective of histology had stage III or IV disease. GI tract involvement was similarly frequent in both groups. Lymphadenopathy was slightly more frequent in patients with non-Burkitt's lymphoma. Median survival time was as low as 9.2 months for Burkitt's and 14.8 months for non-Burkitt's lymphoma (difference not statistically significant).     

ras-family gene mutations in neoplasia of the ampulla of vater

Mutations in the first and second exons of Ha-, Ki- and N-ras oncogenes were investigated in 17 epithelial tumors of the ampulla of Vater by single-strand conformation polymorphism analysis and direct sequencing of DNA fragments amplified by polymerase chain reaction. The panel included 12 intestinaltype adenocarcinomas, 3 villous adenomas, I papillary carcinoma and I neuroendocrine carcinoma. Six cases (35%) contained ras mutations, affecting codon 12 of Ki-ras in 2 adenomas and 3 carcinomas, and of N-ras in I adenoma. All mutations were found in adenomas and among cancers with adenomatous areas, whereas none of the cases lacking adenomatous areas contained mutations. This suggested that ampullary cancers represent heterogeneous diseases with respect to the presence or absence of adenomatous areas and, among those with adenomatous areas, with respect to the presence of activated ras genes. Ki-ras mutated cases included 3 of 4 tumors which mainly involved the intraduodenal bile duct, thus suggesting that a proportion of Ki-ras -mutated ampullary cancers might correspond to those originating from the epithelium of the bile duct component of the ampulla.     

Prognostic factors of ampulla of vater carcinoma after radical surgery

OBJECTIVE Ampullary carcinoma is a rare disease with better prognosis than other periampullary neoplasms. This study investigated the association between clinicopathologic factors and prognosis after radical resection of ampulla of Vater carcinoma.
METHODS Clinical data from 105 patients who underwent radical pancreaticoduodenectomy from January 1990 to December 2005 were retrospectively analyzed by the Kaplan-Meier method, log-rank test, and the Cox proportional hazard model.
RESULTS The in-hospital mortality rate was 8.6%, the lymph node metastasis rate was 37.1%, and the five-year survival rate was 42.8%. Pancreatic involvement （P = 0.027）, tumor diameter （P = 0.008）, T stage （P = 0.003）, TNM stage （P 〈 0.001）, and number of metastatic lymph nodes （P 〈 0.001） were associated with prognosis when the univariate analysis was used. Multivariate analysis showed that the number of lymph node metastases （P 〈 0.001; OR： 1.923; CI： 1.367-2.705） and tumor diameter （P = 0.03; OR： 1.432; CI： 1.035-1.981） were the independent prognostic factors.
CONCLUSION The number of metastatic lymph nodes and tumor diameter are important pathologic factors predicting prognosis of ampulla of Vater carcinoma after radical resection, and lymph node dissection during the radical surgery effectively improves the survival rate.     

Role of adjuvant chemoradiation therapy in adenocarcinomas of the ampulla of vater

PURPOSE: The role of adjuvant chemoradiation therapy (CRT) in the treatment of ampullary cancers remains undefined. We retrospectively compared treatment outcomes in patients treated with pancreaticoduodenectomy alone versus those who received additional adjuvant CRT. METHODS AND MATERIALS: Between May 1990 and January 2006, 54 of 96 patients with ampullary adenocarcinoma who underwent potentially curative pancreaticoduodenectomy also received adjuvant CRT. The median preoperative radiation dose was 45 Gy (range, 30-50.4 Gy) and median postoperative dose was 50.4 Gy (range, 45-55.8 Gy). Concurrent chemotherapy included primarily 5-fluorouracil (52%) and capecitabine (43%). Median follow-up was 31 months. Univariate and multivariate statistical methodologies were used to determine significant prognostic factors for local control (LC), distant control (DC), and overall survival (OS). RESULTS: Actuarial 5-year LC, DC, and OS were 77%, 69%, and 64%, respectively. On univariate analysis, age, gender, race/ethnicity, tumor grade, use of adjuvant treatment, and sequencing of adjuvant therapy were not significantly associated with LC, DC, or OS. However, on univariate analysis, T3/T4 tumor stage was prognostic for poorer LC and OS (p = 0.02 and p < 0.001, respectively); node-positive disease was prognostic for poorer LC (p = 0.03). On multivariate analysis, T3/T4 tumor stage was independently prognostic for decreased OS (p = 0.002). Among these patients (n = 34), those who received adjuvant CRT had a trend toward improved OS (median, 35.2 vs. 16.5 months; p = 0.06). CONCLUSIONS: Ampullary cancers have a distinctly better treatment outcome than pancreatic adenocarcinomas. Higher primary tumor stage (T3/T4), an independent adverse risk factor for poorer treatment outcomes, may warrant the addition of adjuvant CRT to pancreaticoduodenectomy.     

Role of cholangitis in predicting survival in patients with carcinoma of the ampulla of vater

BackgroundThere have been strong suggestions that acute inflammation promotes tumour growth, and has a tendency for increased invasiveness, leading to poor survival after surgery. When cholangitis coexists with ampulla of vater cancer, the patient's prognosis may be influenced by the acute inflammatory status of the bile duct. We evaluated the relationship between the severity of acute cholangitis, preoperative CRP levels and survival.MethodsWe retrospectively analysed 154 patients who underwent surgical resection for AOV cancer between January 2003 and November 2018. Cholangitis was graded according to Tokyo Guidelines 2018. Patients were categorised into two groups based on their CRP levels: CRP > 1 mg/L and CRP ≤ 1 mg/L. Relationship of cholangitis grade and CRP >1 mg/L with survival was assessed.ResultsThe mean age of the patients was 65.8 years. Preoperative cholangitis was present in 40 (25.9%) patients, of which 28 (18.2%) had mild cholangitis, 11 (7.1%) had moderate cholangitis, and one (0.6%) had severe cholangitis. CRP was elevated preoperatively in 56 (36.4%) patients. The median follow-up period was 33.5 months. Severity of cholangitis significantly affected the overall survival (P < 0.001) and disease-free survival (P < 0.001). Patients with mild cholangitis had a median overall survival of 43 months compared to 14 months in those with moderate cholangitis. A preoperative CRP level of >1 mg/L was significantly associated with poor overall survival (P = 0.023) but not with disease-free survival (P = 0.128), although it was associated with a trend towards poorer survival. The survival rate of patients with CRP ≤1 mg/L was 77.4%, whereas that of patients with CRP >1 mg/L was 56.7%. In multivariable analysis, age >65 years (P = 0.015), cholangitis grade (P = 0.050), CRP > 1 mg/L (P = 0.045), venous invasion (P = 0.005), and perineural invasion (P = 0.034) were independent prognostic factors for overall survival while cholangitis grade (P = 0.049) and perineural invasion (P = 0.004) were independent prognostic factors for disease-free survival.ConclusionAcute inflammation when associated with cancer can have a negative effect on patient's survival. Patients with higher grades of cholangitis should be adequately treated to improve the inflammatory status.     

DNA ploidy as a major prognostic factor in resectable ampulla of vater cancers

The archival paraffin-embedded specimens from 63 ampulla of Vater cancers after pancreaticoduodenectomy between 1965 and 1991 were analyzed by flow cytometry. Of the 63 cancers, 31 (49.2%) were diploid DNA cancers and 32 (50.8%) were aneuploid. Patients with diploid DNA cancer had a median survival time of 159.0 months, and patients with aneuploid DNA cancer had 24.0 months. This difference is statistically significant (P = 0.0257). The aneuploid group did have a poorer prognosis than the diploid group. The multivariate analysis demonstrated that DNA ploidy was an independent and very important prognostic factor, even stronger than the stage and lymph node status. There was a tendency toward higher values of S-phase fraction, proliferative index, and total aneuploid DNA fraction in the shorter survival groups, but they were of no statistical significance. These data suggest that DNA ploidy appears to be the most important and the only pre-operative predictor of prognosis in resectable ampulla of Vater cancers since endoscopic biopsy is feasible. © 1993 Wiley-Liss, Inc.     

Large cell neuroendocrine carcinoma of the ampulla of vater with adenocarcinoma and squamous cell carcinoma components

A 73-year-old woman visited our hospital complaining of general fatigue and jaundice. Laboratory tests revealed an elevated total bilirubin, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and γ-glutamyltransferase. Computed tomography and magnetic resonance imaging demonstrated a mass lesion at the ampulla of Vater with dilatation of the common bile duct and main pancreatic duct. Percutaneous transhepatic cholangiography revealed dilatation of the bile duct and a negative filling defect due to the tumor. Pancreatoduodenectomy was performed. The specimen included an ulcerated firm tumor of the papilla Vater. The surface of the ampulla consisted of well-differentiated papillary adenocarcinoma, whereas the deep layer, such as submucosal or muscular layer, contained large cell neuroendocrine carcinoma and squamous cell carcinoma. Immunohistochemistry revealed that the large cell neuroendocrine carcinoma component was positive for chromogranin A, synaptophysin and CD56. The patient died from multiple liver and bone metastases 13 months after surgery. This is a very rare case of a large cell neuroendocrine carcinoma accompanied by adenocarcinoma and squamous cell carcinoma components.     

高龄vater壶腹癌行局部切除的价值探讨

目的:探讨对高龄vater壶腹癌患者进行局部切除术的价值。方法:回顾性总结24例高龄vater壶腹癌患者行局部切除术后临床情况及生存状况。结果:24例高龄vater壶腹癌患者行局部切除术后均无并发症及围手术期死亡,恢复快。1、2、3年生存率为分别为91.6%、75.0%和70.8%,与胰十二指肠切除术相比无明显差异。结论:对高龄vater壶腹癌患者行局部切除是一种较为合理的选择。     

A case of lung metastases of carcinoma of the ampulla of vater effectively treated with S-1

A 70-year-old female patient underwent pylorus-preserving pancreaticoduodenectomy for carcinoma of the ampulla of Vater in March 2007. In April 2009, multiple lung metastases were detected by CT scanning. The patient was treated with S-1 (80mg/day, day 1-28, followed by 2-weeks withdrawal)from April 2009. The shrinkage of lung metastases was diagnosed as a complete response based on the Response Evaluation Criteria in Solid Tumors(RECIST). No severe toxicities were observed. S-1 is an effective and safe anti-cancer agent available for lung metastases of carcinoma of the ampulla of Vater.     

Synchronous primary carcinomas of the ampulla of vater and ascending colon in a patient with multiple flat adenomas

Multiple primary cancers occurring in the same patients have been reported to represent 1.8–3.9% of all cancers. The majority of all patients reported to have had a combination of simultaneous neoplastic changes in the ampulla of Vater and the colon showed familial adenomatous polyposis (FAP) syndrome. Variants of familial adenomatous polyposis coli are: attenuated adenomatous polyposis coli (AAPC, previously also known as flat adenoma syndrome) and multiple adenoma coli. AAPC is characterized clinically by many, but usually fewer than 100, colonic lesions that are characteristically slightly elevated and plaque-like, with a reddish surface and sometimes central depression. Genetically it represents an extremely rare variant of FAP. Another group of individuals, so-called multiple adenoma patients, have a phenotype similar to AAPC, but most have no demonstrable germ-line adenomatous polyposis coli mutation, as do patients with FAP or AAPC. However, there have been only a few reports that discussed concurrent neoplastic changes in the ampulla of Vater and colon in patients with multiple colonic flat adenomas, but without the florid phenotype of classical FAP. We present rare clinical course of a patient with multiple (more than 60) flat adenomas in the proximal colon and two primary cancers: of the ampulla of Vater and of the ascending colon. This patient and his family history did not show polyposis compatible with FAP or hereditary nonpolyposis colorectal cancer (HNPCC) syndrome.     

滤泡性淋巴瘤研究进展

滤泡性淋巴瘤(FL)起源于生发中心B细胞,是我国最常见的惰性B细胞非霍奇金淋巴瘤(NHL),目前仍是一种不可治愈的疾病.在第59届美国血液学会(ASH)年会上,FL为NHL领域报道最多的亚型.目前FL的研究热点为诱导后维持治疗、新药联合诱导治疗及维持治疗、去化疗治疗FL以及寻找新的预后生物学标志.     

Radiotherapy of relapse-refractory follicular lymphoma

Purpose

To assess the efficacy of treatment and outcomes of patients with relapsed or refractory follicular lymphoma treated with external beam irradiation.

滤泡性淋巴瘤预后因素的研究

滤泡性淋巴瘤(follicular lymphoma,FL)是一种起源于滤泡生发中心B细胞的淋巴瘤,其发病率在美国和西欧最高,而在亚洲和发展中国家的发病率相对较低。FL主要累及淋巴结,通常表现为无痛性多发淋巴结肿大。原发于淋巴结外的FL常见于胃肠道、软组织、乳腺及眼眶附属器。FL患者多表现为惰性临床过程,总生存率较高,但多数患者仍会复发或进展。回顾FL的预后因素,为指导患者的临床治疗提供理论依据。     

Cytogenetic evolution of follicular lymphoma

Follicular lymphoma (FL) is closely associated with the chromosomal translocation t(14;18)(q32;q21), which results in an overexpression of the anti-apoptotic bcl-2 gene product leading to a survival advantage of B-lymphocytes. However, in animal models, this genomic aberration is not sufficient for the initiation of the malignant phenotype. Additional genomic rearrangements are required for disease progression. In this review, the most important additional aberrations and possible candidate genes in the respective genomic regions are discussed. In addition, relevant data regarding their role in disease progression as well as the association with clinical presentation and clinical course are presented.     

Spontaneous remission of follicular lymphoma

Follicular lymphoma is an indolent B cells proliferative disorder that represents approximately 35% of all non‐Hodgkin lymphomas. Although spontaneous remission is uncommon in patients with low grade non‐Hodgkin lymphomas, some cases have been reported. We present a case of follicular lymphoma for which we have documented a spontaneous remission both with serial instrumental investigations and through histological biopsy of the bone marrow. The patient is still in remission after 2 years of follow‐up. The causes for a spontaneous remission are not known, and we can only hypothesize a possible reawakening of the host's immune response against the tumour.