Current status of gastrointestinal carcinoids

Gastrointestinal (GI) carcinoids are ill-understood, enigmatic malignancies, which, although slow growing compared with adenocarcinomas, can behave aggressively. Carcinoids are classified based on organ site and cell of origin and occur most frequently in the GI (67%) where they are most common in small intestine (25%), appendix (12%), and rectum (14%). Local manifestations--mass, bleeding, obstruction, or perforation--reflect invasion or tumor-induced fibrosis and often result in incidental detection at emergency surgery. Symptoms are protean (flushing, sweating, diarrhea, bronchospasm), usually misdiagnosed, and reflect secretion of diverse amines and peptides. Biochemical diagnosis is established by elevation of plasma chromogranin A (CgA), serotonin, or urinary 5-hydroxyindoleacetic acid (5-HIAA), while topographic localization is by Octreoscan, computerized axial tomography (CAT) scan, or endoscopy/ultrasound. Histological identification is confirmed by CgA and synaptophysin immunohistochemistry. Primary therapy is surgical excision to avert local manifestations and decrease hormone secretion. Hepatic metastases may be amenable to cytoreduction, radiofrequency ablation, embolization alone, or with cytotoxics. Hepatic transplantation may rarely be beneficial. Chemotherapy and radiotherapy have minimal efficacy and substantially decrease quality of life. Intravenously administered receptor-targeted radiolabeled somatostatin analogs are of use in disseminated disease. Local endoscopic excision for gastric (type I and II) and rectal carcinoids may be adequate. Somatostatin analogues provide the most effective symptomatic therapy, although interferon has some utility. Overall 5-year survival for carcinoids of the appendix is 98%, gastric (types I/II) is 81%, rectum is 87%, small intestinal is 60%, colonic carcinoids is 62%, and gastric type III/IV is 33%.     

卵巢原发性乳头状甲状腺癌的CDFI分析及探讨

目的 探讨卵巢原发性乳头状甲状腺癌的二维及彩色多普勒血流显像特点.方法 对2例经病理学检查确诊的卵巢原发性乳头状甲状腺癌进行二维及彩色多普勒血流显像分析,结合文献对其声像图和血流特点进行探讨.结果 超声显示盆腔或下腹部囊实性包块,多数包块内部以实性回声为主,伴有多房小囊;少数以囊性成分为主伴有分隔及实性结节,彩色多普勒显示实性部分有血供较丰富的高速低阻血流.2例均没有临床恶变及转移的证据.结论 卵巢原发性乳头状甲状腺癌的声像图缺乏明显特征性,必须结合彩色多普勒及其他影像学和实验室检查与其他卵巢肿瘤相鉴别;肿瘤以多房囊实性或多房囊性内有实性成分多见,同时伴有实性部分的低阻动脉血流信号.因此,在超声疑为畸胎瘤的瘤体内测到有明显血流供应的实质或厚分隔成分时,结合临床应高度怀疑卵巢原发性乳头状甲状腺癌.     

甲状腺乳头状癌中ret癌基因的表达

目的：观察人甲状腺乳头状癌中ret癌基因的表达。方法：应用RT－PCR技术研究甲状腺乳头状癌新鲜组织中ret癌基因活化形式PTC基因的表达。结果：25例甲状腺乳头状癌中有6例（24％）PTC基因表达阳性,主要分布于Ⅱ级以上肿瘤中;而甲状腺滤泡型癌、甲状腺瘤和正常甲状腺组织吕PTC均为阴性。结论：PTC基因是ret癌基因新的活化形式,ret基因的活化仅限于甲状腺头状癌类型中,可能是乳头状癌的特殊遗传事件,而无PTC基因表达的乳头状癌,其分子发生机制可能与其他基因的遗传性改变有关。因此检测PTC基因可作为判断甲状腺乳头状癌生物学行为的有效参考指标。     

Results of percutaneous transhepatic cholecystostomy for high surgical risk patients with acute cholecystitis

Aim: To assess the efficacy and safety of percutaneous transhepatic cholecystostomy (PTC) in treatment for acute cholecystitis in high surgical risk patients. Patients and methods: A retrospective review was carried out from January 1999 to June 2007 on 23 patients, 11 males and 12 females, who underwent PTC for the management of acute cholecystitis at the Department of Surgery, Queen Mary Hospital, Hong Kong, China. The mean age of the patients was 83. They all had either clinical or radiological evidence of acute cholecystitis and had significant pre‐morbid diseases. The median follow‐up period on them was 35 months. Results: All the PTCs performed were technically successful. One patient died from procedure‐related haemoperitoneum, while 87% (n= 20) of all the patients had clinical resolution of sepsis by 20 h after PTC. Eight patients underwent elective cholecystectomy afterwards (62.5% with the laparoscopic approach). Eight patients had dislodgement of the PTC catheter and one of them developed recurrent acute cholecystitis 3 months after PTC. That patient was treated conservatively. Four patients died from their pre‐morbid conditions during the follow‐up period. Conclusion: PTC was a safe and effective alternative for treating acute cholecystitis in this group of patients. Thirteen of them without elective cholecystectomy performed did not have recurrent acute cholecystitis after a single session of PTC. It may be considered as a definitive treatment for this group of patients.     

Hilar cholangiocarcinoma: resectability and radicality after routine diagnostic imaging

Background/Purpose En-bloc resection has contributed to the improvement of long-term survival in patients with hilar cholangiocarcinoma. In addition, attenuation of intraoperative traumatization of the tumor may decrease tumor spread. The objective of this study was to assess the importance of a routine diagnostic workup for the surgical strategy, radicality, and results in patients with hilar cholangiocarcinoma.Methods Between September 1997 and December 2002, 82 patients with hilar cholangiocarcinoma were treated at our department. Preoperative diagnostic workup included endoscopic retrograde cholangiography (ERC), percutaneous transhepatic cholangiography (PTC), computed tomography (CT), and magnetic resonance imaging (MRI). The results of preoperative and retrospective (blinded) assessment of diagnostic data concerning the tumor growth along the bile ducts were compared with the results of surgery.Results The resection rate was 75%, and the hospital mortality, 7%. The prospective assessment of the resection to be performed was correct in 81% of cases. In ERC, magnetic resonance cholangiography (MRC), and PTC, tumor assessment was precise in 29%, 36%, and 53%, of cases, respectively. Overestimation occurred more frequently than underestimation. The 3-year survival of patients with formally curative or palliative en-bloc resection was 61% and 15%, respectively. For the 9 patients with hilar resection, the 3-year survival was 25%. Survival of patients was comparable, regardless of whether their tumor had been correctly assessed or over- or underestimated. In the multivariate analysis, R0 resection was the only significant prognostic factor (P = 0.011).Conclusions Our routine diagnostic approach led to high resection and survival rates. Obviously a sophisticated diagnostic workup is not an absolute prerequisite for adequate surgery.     

甲状腺乳头状癌中肿瘤相关巨噬细胞浸润及CD47表达的意义

目的 探讨肿瘤相关巨噬细胞浸润及CD47蛋白表达与甲状腺乳头状癌患者临床病理特征间的关系及意义.方法 收集大连市友谊医院2015—2019年间甲状腺乳头状癌手术切除标本65例,应用免疫组织化学方法检测所有标本中CD68+和CD163+肿瘤相关巨噬细胞浸润和CD47蛋白表达情况,分析肿瘤相关巨噬细胞浸润、CD47蛋白表达...     

分化型甲状腺癌76例^131I治疗临床分析

目的观察口服^131I治疗分化型甲状腺癌患者效果,旨在探讨^131I碘在甲状腺癌治疗中的作用和可行性。方法回顾分析我科2001—05—2012—04收治的甲状腺癌全切或次全切术后76例,给予口服^131I治疗,服^131I后6个月随访复查,通过监测^131I全身显像、甲状腺球蛋白（Tg）、甲状腺球蛋白抗体（TgAb）、游离三碘甲状腺激素（FT3）、游离甲状腺激素（FT4）、促甲状腺激素（TSH）、颈部超声检查决定患者服^131I情况。结果76例甲状腺癌患者,有32例未发生转移的甲状腺癌,均经口服1次^131I治愈,一次治愈率42．1％;有35例患者（5例无转移,30例有转移病灶）经过多次^131I碘治疗后治愈,总治愈率达88．2％（67／76）。5例合并转移的DTC经过服^131I治疗后甲状腺残留或转移病灶有所缩小但未消失,也没有发现新的转移灶及原有病灶扩散。总有效率94．7％。4例合并转移的DTC患者经多次^131I疗效果不明显。多次大量服^131I的患者也未出现严重不良反应。结论分化型甲状腺癌（DTC）术后应该服^131I治疗,以预防和减少复发;残留较多甲状腺组织或有转移的需多次^131I治疗;甲状腺癌术后^131I治疗是安全、有效的。     

Sudden death due to a novel nonsense mutation in Marfan syndrome

BackgroundMarfan syndrome is a hereditary connective tissue disease accompanied by autosomal dominant inheritance; that mainly arises from a mutation in the fibrillin-1 gene (FBN1). Aortic dissection and rupture are the common and lethal complications of MFS and may cause sudden unexpected death.MethodA man aged 34 was admitted to the hospital due to persistent pain in his abdomen 12 h post-drinking and suddenly died 10 h later. A forensic autopsy was performed to identify the underlying mechanism of death. Due to the high suspected of MFS, Sanger sequencing was performed, and a novel mutation was detected in the deceased. To clarify the underlying mechanism of this mutation, real-time quantitative polymerase chain reaction was conducted and Western blot analysis was performed in vitro.ResultsA novel PTC mutation c.933C > A in FBN1 was found. Through family history inspection and Sanger sequencing, other MFS patients in the present family were confirmed. The pathologic changes in the aorta in the present case showed media cystic degeneration, disordered arrangement of elastic fibers and a significant reduction in fibrillin 1 compared with the control. The mutation led to significant reduction inFBN1 mRNA and fibrillin-1 in cells in vitro, and overexpression of phospho-Smad2 was observed.ConclusionWe confirmed a novel pathogenic PTC mutation in the FBN1gene through Sanger sequencing, and the pathological changes and underlying mechanisms were also identified. The present work not only extends the pathogenic mutation spectrum of MFS, but also stresses the role of forensic autopsy, genetic analysis and functional validation of novel mutations in cases of sudden death associated with congenital diseases.