Obstructive jaundice due to a rare periampullary tumor

Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes.A 92-year-old female presented with obstructive jaundice.On exam she had pale conjunctiva and icteric sclera.Abdominal examination revealed tenderness in the upper abdomen.Laboratory data was consistent with obstructive jaundice.Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct(CBD)with no evidence of liver lesions or pancreatic head mass.Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area,dilated CBD(9 mm),a prominent pancreatic duct(4.1 mm)and a hydropic gall bladder with no stones.Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection.Histopathology analyses with immunohistochemical stains were positive for cytokeratin,synaptophysin,S-100 protein,neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma.Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel.Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice.Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.     

Pancreatic cancer. Assessment of prognosis by clinical presentation

Three hundred ninety-three patients who were entered into pancreatic carcinoma treatment protocols of the Gastrointestinal Tumor Study Group (GITSG) were analyzed as to significant differences in clinical presentation and factors influencing survival. Patients were grouped according to the stage of the disease. Group I (21 cases) included those patients who had a potentially curative resection. Group II (182 cases) patients had a locally unresectable tumor less then 400 cm2 (surgically proven) and no distant metastases, and Group III (190 cases) had advanced disease. Group I patients had the smallest lesions (median area, 9 cm2), located in head of the gland in 90% and painless jaundice was the most frequent clinical presentation (52%). In Group II, 83% were located in the head of the gland but the median area was much larger (36 cm2). Pain was present in 80% of cases, and jaundice in 62% with 48% having jaundice and pain. In Group III patients, lesions of body and tail were over four-fold as frequent as in Group I and almost three-fold greater than in Group II. The median area of the lesion was large (30 cm2). Pain was present in 85% and jaundice in only 31%. Median survival in Group I patients was longer than Group III (73 versus 10 weeks; P less than 0.001). Ambulatory status, sex, race, abdominal pain, and histologic type influenced survival in one or more groups whereas age, jaundice, location of the tumor, degree of cellular differentiation, back pain, and nutritional status did not influence survival in any group. In all groups, those with a good performance status (Eastern Cooperative Oncology Group [ECOG] 0 and 1) survived longer than those with poor status (ECOG 2 and 3; P less than 0.05). The best potential prognosis is in those who are fully productive and present with painless jaundice, and who have resection of the tumor.     

Diagnosis of Small Carcinoma of the Pancreas: Importance of Ultrasound Scanning and Endoscopic Retrograde Cholangiopancreatography (ERCP)

Diagnostic aspects of 23 patients with carcinoma of the pancreaswere analyzed and four cases of small cancer (less than 2 cm)were presented. These tumors were resected between January 1975and Dcember 1982 in the National Cancer Center Hospital, Tokyo. Initial signs and symptoms were generally vague and none ofthem were specific for pancreatic cancer. The most importantclues to the detection of the tumor were jaundice in the casesof pancreas head cancer, and extragastric compression demonstratedby x-ray examination in cases of cancer of the pancreas bodyand tail. In those cases without jaundice, elevated levels ofamylase and glucose in the serum and urine were important findings.In regard to imaging diagnosis, endoscopic retrograde cholangiopancreatographyand ultra-sonography were the most helpful means of diagnosisof small pancreatic carcinoma.     

体部伽玛刀治疗晚期胰腺癌的临床分析

目的:探讨体部伽玛刀治疗晚期胰腺癌的安全性和有效性。方法:采用SGS-Ⅰ型立体定向伽玛射线体部治疗系统(体部伽玛刀)治疗79例晚期胰腺癌患者。伽玛刀治疗采用三维立体定向治疗计划系统给予患者精确定位。在临床靶区(CTV)边缘外放5mm-10mm形成计划靶区(PTV);等剂量线为50%-60%,肿瘤≤5cm的单次周边剂量3.5-4.5Gy,肿瘤>5 cm的单次周边剂量3.0-4.0Gy;治疗总剂量为35-48Gy;治疗次数9-11次,大多数患者为每周5次治疗。观察病人的有效率、生存时间和临床受益率(CBR)。结果:伽玛刀治疗过程中88.9%患者上腹部及腰背部疼痛明显减轻;66.7%患者黄疸指数下降。CBR 69.6%。伽玛刀治疗3个月复查CT,病变部位达到CR 1例,PR 25例;总有效率(CR+PR)32.9%。主要不良反应为:恶心,呕吐,骨髓抑制。结论:伽玛刀是治疗晚期胰腺癌的安全、有效的治疗方法。为临床肿瘤医师提供了一个新的治疗手段。     

体部伽玛刀治疗晚期胰腺癌的临床分析

目的：探讨体部伽玛刀治疗晚期胰腺癌的安全性和有效性。方法：采用SGS-Ⅰ型立体定向伽玛射线体部治疗系统（体部伽玛刀）治疗79例晚期胰腺癌患者。伽玛刀治疗采用三维立体定向治疗计划系统给予患者精确定位。在临床靶区（CTV）边缘外放5mm-10mm形成计划靶区（PTV）;等剂量线为50%-60%,肿瘤≤5cm的单次周边剂量3.5-4.5Gy,肿瘤〉5 cm的单次周边剂量3.0-4.0Gy;治疗总剂量为35-48Gy;治疗次数9-11次,大多数患者为每周5次治疗。观察病人的有效率、生存时间和临床受益率（CBR）。结果：伽玛刀治疗过程中88.9%患者上腹部及腰背部疼痛明显减轻;66.7%患者黄疸指数下降。CBR 69.6%。伽玛刀治疗3个月复查CT,病变部位达到CR 1例,PR 25例;总有效率（CR＋PR）32.9%。主要不良反应为：恶心,呕吐,骨髓抑制。结论：伽玛刀是治疗晚期胰腺癌的安全、有效的治疗方法。为临床肿瘤医师提供了一个新的治疗手段。     

胰腺癌早期诊断及外科治疗探讨

目的:探讨胰腺癌早期诊断和提高手术疗效的临床措施。方法:回顾性分析我院自1974年-2001年间连续收治的340例胰腺癌的诊断及外科治疗。结果:本组19.1%(65/340)的胰腺癌病例施行了根治性手术,手术死亡率7.7%(5/65),80.9%(275/340)行姑息性手术及探查术。行根治性手术的胰腺癌病例,首发症状多为上腹部隐痛、胀满不适、纳差、乏力和消瘦等非特异性症状,仅16.9%为无痛性黄疸。影像学检查间接征象检出率分别为胆道扩张97.1%,胆囊肿大88.8%,胰管狭窄、中断、移位和增粗93.8%。对有首发症状的高危人群,联用影像学技术检查直接及间接征象,并及早手术探查,有助于胰腺癌早期诊断。Child改良式胰十二指肠切除术后并发症和手术死亡率明显降低。结论:联用影像技术、及早手术探查,早识别,早诊断,早治疗,是提高胰腺癌早期诊断改善疗效的有效措施。根治性手术切除(Child改良术式)为主的综合性治疗仍是治疗本病的主要治疗措施。     

Obstructive jaundice in a metastatic tumor of the pancreas from breast cancer: a case report

Metastatic pancreas tumors from breast cancer are comparatively uncommon and patients with this tumor usually remain asymptomatic during their life. A 55-year-old woman presented with obstructive jaundice following mastectomy for invasive ductal carcinoma. We diagnosed obstructive jaundice due to a pancreatic tumor demonstrated on computed tomography and performed percutaneous transhepatic cholangio-drainage. Although the patient recovered from the jaundice, she had exacerbation of pneumonia from which she died. At autopsy, invasive ductal carcinoma was found in the pancreas tumor. Immunohistochemical staining was performed to confirm whether the pancreatic tumor was primary or secondary. Human milk fat globules 1 and 2 and gross cystic disease fluid protein-15, which characteristically exist in normal breast tissue or breast carcinoma, were expressed both in the primary breast tumor and the pancreatic tumor. In contrast, both the anti-estrogen receptor and anti-progesterone receptor antibodies stained positively in the primary breast cancer; however, neither of them was positive in the metastatic pancreatic tumor. We report a rare case of a patient who presented with obstructive jaundice from a pancreatic tumor metastasizing from breast cancer and in whom immunohistochemical staining using the antibodies unique to the mammary gland was effective for the diagnosis of this secondary tumor.     

经皮肝穿刺胆道造影对恶性梗阻性黄疸病因诊断价值的探讨

本文报告２４例恶性梗阻性黄疸，其中肝门区癌２例，胰头癌１１例，胆管癌７例，壶腹癌３例．原发性十二指肠腺癌１例。在术前作Ｂ超和ＰＴＣ检查，对梗阻的部位、范围、程度、性质和病因诊断等临床资料进行对照分析，Ｂ超和ＰＴＣ诊断与手术和病理学诊断的符合率分别为５４％和９１．６％。结果表明，ＰＴＣ对恶性梗阻性黄疸术前病因诊断的正确率明显高于Ｂ超，其操作方法简便安全，更适合于基层医院应用。     

超声检查在胆道下段阻塞性黄疸恶性病变中的应用价值

目的：探讨二维、彩色多普勒及超声造影技术在胆道下段阻塞性黄疸恶性病变中的声像图特征。方法：应用二维、彩色多普勒及超声造影技术对经病理证实的88例胆道下段阻塞性黄疸恶性病变即胰腺癌、胆管癌、壶腹癌患者观察其胆总管梗阻的程度、胰管增宽情况及肿瘤位置、大小形态、包膜边界、内部结构、血流状况等图像特征。胰腺癌中6例胰头癌患者进行超声造影。结果：88例恶性肿瘤患者中胰腺癌39例,胆管癌18例,壶腹癌31例。声像图特征：胆道下段阻塞性黄疸恶性病变均有肝内外胆管扩张、胆囊正常或增大、胰管扩张。胰腺癌包括胰头癌35例、胰体癌2例及胰尾癌2例,可有大小不等肿瘤,周围血流包绕。超声造影胰腺癌肿瘤本身与周围胰腺组织相比,表现出略低于正常胰腺的增强强度模式,时相呈典型低增强,造影剂充填分布不均,廓清时间早于正常胰腺组织,肿瘤病灶的大小和边界以及与胰腺周围动静脉的关系显示更清晰。胆管癌18例中,截断型2例,肿块型5例,漏斗型11例。壶腹癌31例。误诊8例：胰腺癌4例漏诊,胆管癌2例误诊,壶腹癌2例漏诊,诊断符合率为90．9％（80／88）。全部经病理证实。结论：引起胆道下段阻塞性黄疸之恶性肿瘤二维及彩色多普勒声像图根据其特征可作出诊断,对不典型图像还可采用超声造影技术进行确诊,有利于临床治疗方案的制定和术后疗效的评估。     

A case of pancreatic acinar cell carcinoma with a giant liver metastasis successfully treated with combination of gemcitabine and peroral S-1

Pancreatic acinar cell carcinoma is rare, and its incidence is less than 1% of all the malignant pancreatic tumors. Little is reported on effectiveness of chemotherapy. We report a 64-year-old male patient with pancreatic acinar cell carcinoma and a giant metastatic liver tumor, which responded to combination chemotherapy with gemcitabine(GEM)and peroral S-1 administration. The patient had upper abdominal pain and hypervascular tumors in liver(15 cm in diameter)and pancreas tail (3 cm in diameter), which were detected by an enhanced abdominal computed tomography(CT)scan, and was admitted for further examination. Abdominal angiography, FDG-positron emission tomography(PET), and liver tumor biopsy led to a diagnosis of pancreatic acinar cell carcinoma in the pancreas tail with liver metastasis. The patient was then treated with combination chemotherapy, which consisted of intravenous infusion of GEM and peroral administration of S-1, and the metastatic liver tumor was markedly reduced(partial response in RECIST). Although the prognosis of patients with unresectable pancreatic acinar cell cancers is generally unfavorable, it is suggested that the GEM/S- 1 combination chemotherapy is effective for these patients' treatment.     

胰腺腺鳞癌24例临床诊治分析

目的:探讨胰腺腺鳞癌的临床病理特征及诊治经验。方法:回顾性分析我院2006年12月至2018年11月收治的24例胰腺腺鳞癌患者的临床资料。并进行随访,截止到2019年3月31日,失访2例,失访病人采用截尾数据处理。结果:24例患者中男性16例,女性8例,平均年龄为（60.2±9.2）岁。15例（62.5%）患者因上腹痛、腹胀腹泻就诊,4例（16.7%）患者因皮肤巩膜黄染、皮肤瘙痒就诊,2例（8.3%）患者因腹胀伴腰痛就诊,1例（4.2%）患者因腰背痛就诊,1例（4.2%）患者因黑便乏力就诊,1例（4.2%）患者因体检发现胰腺占位就诊。所有患者术前检查均证实为胰腺占位。超声提示胰腺内见肿物,轮廓不规则,边界欠清晰,肿瘤向周围组织呈蟹足样浸润,内呈低回声不均匀,肿瘤坏死液化呈现无回声区。CT提示胰腺可见不规则低密度灶,边界不清,增强扫描病灶呈弱强化,其强化程度明显低于周围胰腺实质。磁共振提示胰腺可见稍长T1、稍长T2信号肿块,边界模糊,增强扫描弱强化,强化欠均匀。术中明确肿瘤位于胰头及钩突部14例（58.3%）,胰体尾部10例（41.7%）。肿瘤长径平均为5.1 cm（2.3~10 cm）。TNM分期:Ⅰb期8例（33.3%）,Ⅱa期12例（50.0%）,Ⅱb期1例（4.2%）,Ⅲ期3例（12.5%）。所有患者均行手术治疗,行胰十二指肠切除术14例,胰体尾、脾切除术10例。术后随访22例,失访2例,平均生存时间为21.3个月（1~58个月）,随访截止时有8例患者存活。结论:胰腺腺鳞癌是一种罕见的恶性肿瘤,临床表现缺乏特异性,术前影像学表现提示恶性可能,确诊依赖病理学诊断,其具有嗜神经性,手术后结合化疗、放疗仍是目前主要的治疗手段。     

Small carcinoma of the pancreas. Clinical and pathologic evaluation of 17 patients

The clinical and pathologic characteristics of 17 small carcinomas (less than 2 cm in diameter) of the pancreas are reviewed in this article. All the tumors were located in the head of the pancreas, and the clue to the diagnosis was jaundice in ten patients and abdominal pain in seven. Carcinoembryonic antigen (CEA) and CA 19-9 were not reliable markers for detecting small carcinomas of the pancreas. Ultrasonography (US), computerized tomography (CT), percutaneous transhepatic cholangiography (PTC), and endoscopic retrograde cholangiopancreatography (ERCP) were useful diagnostic tools. Lymph node metastases were found in 41% of affected patients, capsular invasion in 24%, retroperitoneal invasion in 24%, and portal system involvement in 29%. In five patients the carcinoma was Stage I; in eight patients, Stage II; in two patients, Stage III, and in two patients, Stage IV. Fifteen patients with Stages I to III and one patient with Stage IV underwent curative pancreaticoduodenectomy or total pancreatectomy, and one patient with liver metastasis and Stage IV underwent noncurative pancreaticoduodenectomy. The cumulative 4-year survival rate was 37%. Although four patients with Stage I disease lived for more than 48 months, the survival period of the 12 patients with Stages II to IV disease was less than 25 months. Thus, small carcinoma of the pancreas is not always curable; however, a small, localized lesion without any extratumoral extension can be resected with a chance of cure.     

原发性小肠肿瘤121例临床分析

目的 加强对原发性小肠肿瘤临床表现、诊断技术及病理类型的认识.方法 回顾性分析121例原发性小肠肿瘤患者的临床资料.结果 121例患者主要临床表现依次为腹痛、贫血、消化道出血、黄疸、消瘦、呕吐、腹部包块、发热.原发部位为十二指肠者74例(61.2％)、空肠28例(23.1％)、回肠19例(15.7％).确诊方法包括ERCP和外科手术.121例患者中,恶性肿瘤占85.1％( 103/121),以腺癌、恶性间质瘤常见;良性肿瘤占13.2％( 16/121),腺瘤常见;交界性间质瘤占1.7％ (2/121).结论 原发性小肠肿瘤最常见临床表现为腹痛、贫血,且大部分为恶性肿瘤,ERCP和外科手术可以确诊;加强对其认识有望提高早期诊断率.     

Diagnosis and treatment of carcinoids of the large intestine

A total of 1,279 cases of carcinoids reported in Japan included 882 cases (69.0%) in the digestive organs. Among the latter cases, carcinoids of the large intestine consisted of (A) 320 cases (36.3%) in the rectum and (B) 46 cases (5.2%) in the ileocecal region, cecum and colon excluding the rectum. The predominant difference of signs and symptoms between (A) and (B) was a high incidence of large palpable abdominal tumors and obstructive findings (ileus) in the latter and a high incidence of small-sized tumors and anorectal disturbances such as hemorrhoids, anal bleeding or anal pain in the former. The incidence of the carcinoid syndrome in (B) was 13.0%, considerably higher than the average of the Japanese series (4.1%) and the America-Europe series. Silver impregnation methods with a combination of Grimeluis' argyrophil and Masson-Fontana's argentaffin reactions were considered to be useful methods for diagnosis of carcinoids of the large intestine. The rate of metastasis in relation to tumor size in the Japanese series was 6.1% for lesions less than 1.0 cm in size and 25.7% for lesions between 1.0 and 2.0 cm. Because of a high incidence of metastasis in (B), 41.3%, greater than that for ordinary carcinoma in the identical sites, it was emphasized that surgical treatment of carcinoids of the large intestine should be principally in accordance with that for ordinary carcinomas.     

肾癌的早期诊断：附小肾癌与无症状肾癌18例分析

１９６６年至１９８９年，我科收治肾癌１２５例，其中小肾癌１０例，无症状肾癌８例，对于小肾癌的影像学检查，我们体会，应该用Ｂ超，ＩＶＵ及ＣＴ进行联合影像学诊断，１０例小肾癌中，６例有血尿或腰疼，说明血尿及腰疼并不是肾癌的晚期症状，而也可以是肾癌的早期临床表现。８例无症状肾癌中，３例为Ｂ超查体发现，我们建议推广对４０岁以上中老年人Ｂ超查体，本文还提出了肾癌的狭义早期诊断及广义早期认断的两个新概念。     

Conclusions concerning the symptomatic treatment of cancer of the pancreas]

Symptomatic treatment is the only possibility in the large majority of patients with pancreatic cancer since a curative surgical excision of the tumor is only possible in less than 5% of cases. In most cases, symptomatic treatment must take short survival rate into consideration. Symptoms managed, in the aim to improve the quality of remaining life include weight loss and anorexia, abdominal pain, jaundice secondary to biliary compression, and sometimes digestive consequences of pancreatic surgery. Weight loss can be treated with nutritional support (particularly enteral nutrition) but the indications of such a treatment are rare. Abdominal pain should be treated by potent analgesics (opiates) and sometimes by percutaneous block of the coeliac ganglion rather than abdominal radiotherapy. Jaundice can be treated by surgical biliary drainage or better still by palliative biliary endoprothesis. Finally, digestive consequences of pancreatic surgery require symptomatic treatment.     

Role of endoscopic ultrasound in the diagnosis of pancreatic cancer

Endoscopic ultrasonography (EUS) with or without fine needle aspiration has become the main technique for evaluating pancreatobiliary disorders and has proved to have a higher diagnostic yield than positron emission tomography, computed tomography (CT) and transabdominal ultrasound for recognising early pancreatic tumors. As a diagnostic modality for pancreatic cancer, EUS has proved rates higher than 90%, especially for lesions less than 2-3 cm in size in which it reaches a sensitivity rate of 99% vs 55% for CT. Besides, EUS has a very high negative predictive value and thus EUS can reliably exclude pancreatic cancer. The complication rate of EUS is as low as 1.1%-3.0%. New technical developments such as elastography and the use of contrast agents have recently been applied to EUS, improving its diagnostic capability. EUS has been found to be superior to the recent multidetector CT for T staging with less risk of overstaying in comparison to both CT and magnetic resonance imaging, so that patients are not being ruled out of a potentially beneficial resection. The accuracy for N staging with EUS is 64%-82%. In unresectable cancers, EUS also plays a therapeutic role by means of treating oncological pain through celiac plexus block, biliary drainage in obstructive jaundice in patients where endoscopic retrograde cholangiopancreatography is not affordable and aiding radiotherapy and chemotherapy.     

Recent advances in diagnosis of pancreatic cancer

The clinical course of patients with pancreatic cancer remains dismal. This may be because the pancreas is a retroperitoneal organ, the symptoms are non-specific, occur late, and pancreas cancer has an invasive nature. Screening for asymptomatic or symptomatic patients using serum tumor markers and ultrasonography is not cost-effective. However, the clinical course of small carcinomas less than 1 cm or 1 cm in size is favorable. Thus, the detection of small pancreatic carcinoma is essential to improve the clinical outcome. Possible discovery of pancreatic carcinoma as a diagnostic clue to diabetes mellitus, and intraductal papillary-mucinous neoplasm are introduced based on our clinical experience.     

胰腺实性假乳头状瘤:MDCT诊断及鉴别

目的 探讨胰腺实性假乳头状瘤的多排螺旋CT(multi-detector computer tomography,MDCT)诊断价值.方法 回顾性分析11例经手术病理证实的胰腺实性假乳头状瘤的临床资料及MDCT表现.结果 11例临床多表现为上腹部胀痛不适,所有病例均无黄疸.CT表现为胰腺单发囊实性类圆形肿块,最大直径约5.5～13.6 cm,平均8.6 cm;肿瘤位于胰头部3例,胰体部2例,胰尾部6例.平扫肿瘤呈不均匀的较低密度,边界较清,3例肿块内可见钙化;动态增强后肿瘤实性成分呈不均匀的轻至中度进行性强化,弱于正常胰腺组织强化,囊变坏死区无强化,10例见较完整包膜,7例可见肿瘤与胰腺间呈“喇叭口”样界面.所有病例均无胆管及胰管扩张,未见腹腔及腹膜后肿大淋巴结.结论 胰腺实性假乳头状瘤多见于较年轻女性,好发于胰头、尾部,MDCT对其有较大诊断价值.     

Obstructive jaundice caused by hepatocellular carcinoma

Hepatocellular carcinoma can be complicated by obstructive jaundice with tumor growing into the extrahepatic bile duct. This complication is an autopsy finding in most reported cases and, rarely, is recognized ante mortem. We report two patients with hepatocellular carcinoma who presented initially with obstructive jaundice. Clotted blood and tumor, which caused bile duct obstruction, was removed operatively and the biliary tract was drained in both patients. We conclude that blood clot and fleshy debris removed from the common bile duct at operation for obstructive jaundice suggests the possibility of hepatocellular carcinoma; the differential diagnosis of jaundice and fever in patients known to have hepatocellular carcinoma should include intrabiliary tumor causing obstruction and cholangitis; and the association of obstructive jaundice and hepatocellular carcinoma may occur more often than previously recognized.