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A Persistent Solid Pseudopapillary Tumor of the Pancreas: Case Report and Brief Literature Review. 胰腺持续性实性假乳头状瘤1例报告及简要文献复习。
Pub Date : 2015-11-01 eCollection Date: 2015-01-01 DOI: 10.1089/crpc.2015.29006.jbz
Jin Bao Zhang, Dong Shang, Theresa P Yeo, Shawnna Cannaday, Warren R Maley, Charles J Yeo

Background: Solid pseudopapillary tumors (SPTs) of the pancreas are uncommon neoplasms, first reported in 1934, well described by Frantz in 1959, and later further characterized by Hamoudi in 1970. Ninety percent of these tumors occur in young females in their second to third decade of life. An interesting case of a persistent solid pseudopapillary neoplasm is described in this report. Case presentation: A 24-year-old woman from a Middle Eastern country presented with an 8.2 × 7.6 cm heterogeneous-enhancing lesion growing within the uncinate process of the pancreas. She had first experienced symptoms at the age of 12 years. Imaging studies showed that the mass closely abutted the superior mesenteric vein as well as the superior mesenteric artery (SMA). The patient underwent an open cholecystectomy and a classic pancreaticoduodenectomy. During the resection, the SMA was transected due to tumor adherence. The vessel was subsequently reapproximated in an end-to-end manner. On the first postoperative day, thrombosis of the SMA occurred and a bile leak developed. The patient returned to the operating room for SMA embolectomy and for repair of a hepaticojejunostomy leak, with redo of the biliary-enteric anastomosis. Histopathological examination showed solid pseudopapillary-arranged cells and cystic areas, showing strong cellular immunoreactivity for CD56, CD10, vimentin, and β-catenin, and weak diffuse staining for synaptophysin. The tumor stained negative for chromogranin A, trypsin, AE1/AE3, and E-cadherin. Molecular genetic analysis was negative for the MYB gene deletion. At nearly 1 year of follow-up, the patient is well with no evidence of tumor recurrence. Conclusion: SPTs of the pancreas should be included in the differential diagnosis of pancreatic tumors, especially in young women.

背景:胰腺的实性假乳头状肿瘤是一种罕见的肿瘤,1934年首次报道,1959年由Frantz详细描述,1970年由Hamoudi进一步描述。90%的此类肿瘤发生在年轻女性的第二到第三个十年。本报告描述了一个有趣的持续性实性假乳头状肿瘤病例。病例介绍:一名来自中东国家的24岁女性,表现为胰腺钩突内生长的8.2 × 7.6 cm的非均匀增强病变。她在12岁时首次出现症状。影像学检查显示肿块紧靠肠系膜上静脉和肠系膜上动脉。患者接受了开放胆囊切除术和经典胰十二指肠切除术。在切除过程中,由于肿瘤粘附,横切SMA。随后,以端到端方式重新逼近了该船。术后第一天,SMA发生血栓形成并发生胆漏。患者返回手术室进行SMA栓塞切除术,修复肝空肠吻合术泄漏,并重新进行胆肠吻合术。组织病理学检查显示实性假乳头状排列细胞和囊性区,对CD56、CD10、vimentin和β-catenin表现出较强的细胞免疫反应性,对synaptophysin表现出较弱的弥漫性染色。肿瘤嗜铬粒蛋白A、胰蛋白酶、AE1/AE3、e -钙粘蛋白均呈阴性。分子遗传分析未发现MYB基因缺失。随访近1年,患者情况良好,无肿瘤复发迹象。结论:胰腺SPTs应作为胰腺肿瘤的鉴别诊断指标,尤其是年轻女性。
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引用次数: 2
Introduction to Case Reports in Pancreatic Cancer. 胰腺癌病例报告简介。
Pub Date : 2015-11-01 eCollection Date: 2015-01-01 DOI: 10.1089/crpc.2015.29005.cjy
Charles J Yeo
I would like to welcome the readers of this new publication—Case Reports in Pancreatic Cancer. It is critical at this point in history that we focus our attention on pancreatic cancer. Pancreatic cancer is currently the fourth leading cause of cancer deaths in the United States; however, it is projected to rise in both numbers and rankings. A recent executive summary from the Pancreatic Cancer Action Network (PanCAN) predicts that pancreatic cancer will move from the fourth leading cause of cancer deaths in the United States to become the second leading cause of cancer deaths in the United States by perhaps 2020 or 2025. Importantly, it is notable that pancreatic cancer is alone among the top five U.S. cancer killers (currently lung, colorectal, breast, pancreas, and prostrate in that order) in that the incidence rate and the death rate are increasing only for pancreatic cancer. Shockingly, but important to repeat, pancreatic cancer is poised to be the number two cause of cancer death in the United States in the next decade. Many of us have been privileged to work in the field of pancreatic cancer for almost three decades. Progress has been made. However, sadly there currently are no universally applicable early detection tools or ‘‘major breakthroughs’’ that have arisen in the field of pancreatic cancer. The pancreatic cancer research community is small, although growing, and has had some successes. The pancreatic cancer research community has sadly been underfunded by the National Cancer Institute (NCI). Fortunately, the U.S. Congress has passed the Recalcitrant Cancer Research Act, which has asked the NCI to formulate a comprehensive and long-term strategic plan to bring the infrastructure, intellectual energy, and resources into play so that we can more efficiently attack this urgent cancer diagnosis. I was honored to have been asked to serve as the editor-in-chief of this new journal in the field of pancreatic cancer. There are many journals that publish on pancreatic cancer: journals in the cancer field, surgical field, medical oncology field, pathology field, and so on. However, informative case reports can be difficult to publish, although each of them can serve an important educational mission. We have put together an outstanding group of associate editors, from our Jefferson Pancreas and Biliary Related Cancer Center. I am extremely pleased to welcome the following individuals who will join me as associate editors: Voichita Bar-Ad, MD Jonathan R. Brody, PhD Thomas Kowalski, MD Harish Lavu, MD Ashwin R. Sama, MD Jordan M. Winter, MD Theresa P. Yeo, PhD
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引用次数: 0
HER-2-Positive Ampullary Adenocarcinoma: A Case Report. her -2阳性壶腹腺癌1例报告。
Pub Date : 2015-11-01 eCollection Date: 2015-01-01 DOI: 10.1089/crpc.2015.29004.koh
Kevin O'Hayer, John Farber, Charles J Yeo, Ashwin R Sama

Background: Ampullary adenocarcinomas are a rare subset of periampullary tumors with an overall poor prognosis. Treatment decisions are generally extrapolated from pancreatic chemotherapy protocols and consist mainly of traditional chemotherapy drugs. There are no known targets for therapeutic intervention in ampullary adenocarcinoma at this time. Next generation sequencing and other novel molecular profiling of tumors, including circulating tumor DNA (ctDNA), have recently made it possible to better understand tumor biology and elucidate driver mutations which are amenable to targeted therapy. This case describes the use of novel DNA sequencing technology to provide a targeted treatment option, HER-2 inhibition, in a patient with HER-2 overexpressing ampullary adenocarcinoma. This is the first time this has been described in the literature. Case presentation: The patient is a 63-year-old Caucasian man who initially presented with symptoms of obstructive jaundice and was found to have a periampullary tumor. He underwent resection of his tumor and pathology confirmed a stage IIB ampullary adenocarcinoma. He unfortunately developed a recurrence in the liver and lung two years later. Next generation sequencing of his tumor at the time of resection as well as ctDNA analysis demonstrated a HER-2 overexpressing tumor. Following first line therapy with FOLFOX he had progression and was treated with trastuzumab and pertuzumab with stabilization of his disease prior to his ultimate demise from multifocal pneumonia. Conclusion: The use of next generation sequencing as well as ctDNA technology generated a novel therapeutic intervention in our patient. As these techniques become more widespread, it is likely more targeted therapies will be used in these difficult to treat diseases.

背景:壶腹腺癌是壶腹周围肿瘤中一种罕见的亚型,总体预后较差。治疗决定通常是从胰腺化疗方案推断出来的,主要由传统的化疗药物组成。目前还没有已知的壶腹腺癌治疗干预的靶点。下一代测序和其他新的肿瘤分子图谱,包括循环肿瘤DNA (ctDNA),最近使得更好地理解肿瘤生物学和阐明可用于靶向治疗的驱动突变成为可能。本病例描述了使用新的DNA测序技术,为HER-2过表达壶腹腺癌患者提供靶向治疗选择,HER-2抑制。这是第一次在文献中描述这种情况。病例介绍:患者是一名63岁的白人男性,最初以梗阻性黄疸的症状出现,后来发现壶腹周围有肿瘤。他接受了肿瘤切除术,病理证实为IIB期壶腹腺癌。不幸的是,两年后他的肝脏和肺部又复发了。切除时肿瘤的下一代测序以及ctDNA分析显示HER-2过表达肿瘤。在FOLFOX一线治疗后,患者病情出现进展,并在多灶性肺炎最终死亡前接受曲妥珠单抗和帕妥珠单抗治疗,病情趋于稳定。结论:下一代测序和ctDNA技术的使用为我们的患者带来了一种新的治疗干预。随着这些技术的普及,更有针对性的治疗方法可能会用于这些难以治疗的疾病。
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引用次数: 5
Cholangio-Conundrum: A Case Series of Painless Jaundice. 胆道难题:无痛性黄疸系列病例。
Pub Date : 2015-11-01 eCollection Date: 2015-01-01 DOI: 10.1089/crpc.2015.29002.apj
Adam P Johnson, Deviney A Rattigan, Michael J Pucci, Jeffrey P Baliff, Jordan M Winter, Charles J Yeo, Harish Lavu

Background: Correct preoperative diagnosis of hepatobiliary disease can be challenging-even with current advances in radiographical imaging, laboratory testing, and endoscopic evaluation. Case presentation 1: A 65-year-old female with painless jaundice and weight loss was found to have cholelithiasis complicated by the Mirizzi syndrome. Case presentation 2: A 71-year-old female with new-onset painless jaundice and impacted stone in the gallbladder neck was found to have a cholangiocarcinoma. Case presentation 3: A 70-year-old male with progressive painless jaundice and weight loss was found to have a pancreatic adenocarcinoma. Conclusion: Proper diagnosis and management of patients with painless jaundice can be difficult in the preoperative setting and may require surgical exploration to obtain a definitive diagnosis.

背景:肝胆疾病的正确术前诊断是具有挑战性的,即使在放射成像、实验室检查和内窥镜评估方面取得了进展。病例报告1:65岁女性,无痛性黄疸,体重下降,发现胆石症合并Mirizzi综合征。病例报告2:一名71岁女性,新发无痛性黄疸及胆囊颈部嵌塞结石被发现为胆管癌。病例报告3:一位70岁男性,患有进行性无痛性黄疸和体重下降,被发现患有胰腺腺癌。结论:术前对无痛性黄疸患者的正确诊断和处理是困难的,可能需要手术探查才能得到明确的诊断。
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引用次数: 1
Hepatoid Carcinoma of the Pancreas: A Case Report and Review of the Literature. 胰脏类肝癌1例报告及文献复习。
Pub Date : 2015-11-01 eCollection Date: 2015-01-01 DOI: 10.1089/crpc.2015.29001.nlw
Noelle L Williams, Joshua D Palmer, Voichita Bar-Ad, Pramila Rani Anné, Ashwin R Sama, Jonathan C Weinstein, Miguel L Rufail, Charles J Yeo, Mark D Hurwitz

Background: Hepatoid carcinoma (HC) is a rare extrahepatic malignancy that shares many morphological and serological features with hepatocellular carcinoma. HC has been reported to arise from several organs that are derived from the foregut endoderm, including the stomach, gallbladder, and pancreas. We present a case of an elderly man with hepatoid adenocarcinoma of the pancreatic head with duodenal invasion, presenting with pancreatitis and a gastrointestinal bleed. With only 23 reported cases at the time of our literature search, we discuss the presentation, histopathology, and management of such a rare disease. Case presentation: A 71-year-old man presented initially with abdominal pain and was treated conservatively for pancreatitis. Four months later, he presented with melena and anemia. His examination was noncontributory. Esophagogastroduodenoscopy revealed a friable ampulla of Vater, and a CT scan of the abdomen showed a 4.5 cm pancreatic head mass. Fine needle aspirate revealed an epithelioid neoplasm with hepatoid morphology. Serum α-fetoprotein was normal. Surgical resection confirmed hepatoid adenocarcinoma of the pancreas with positive lymphadenopathy and negative margins. There was no radiographical or gross evidence of distant spread. Observation and adjuvant gemcitabine were discussed as possible options. The patient elected to receive care closer to home and will continue surveillance imaging. Conclusion: With only 23 reported cases, pancreatic HC represents a rare entity within gastrointestinal oncology. There is no clear postoperative adjuvant standard therapy for this likely heterogeneous group of tumors. Although surgical resection is the mainstay of upfront treatment, metastatic disease to the lymph nodes or liver portends a poor prognosis and may warrant treatment such as transarterial embolization, chemotherapy, or radiotherapy.

背景:肝样癌(HC)是一种罕见的肝外恶性肿瘤,与肝细胞癌具有许多形态学和血清学特征。据报道,HC起源于前肠内胚层的几个器官,包括胃、胆囊和胰腺。我们报告一位老年男性胰脏头部肝样腺癌伴十二指肠侵犯,表现为胰腺炎及胃肠道出血。在我们的文献检索中,只有23例报告病例,我们讨论了这种罕见疾病的表现、组织病理学和治疗。病例介绍:一名71岁男性,最初表现为腹痛,保守治疗胰腺炎。四个月后,他出现黑黑和贫血。他的检查没有结果。食管胃十二指肠镜显示脆弱的壶腹,腹部CT扫描显示4.5厘米的胰头肿块。细针抽吸显示为肝样上皮样肿瘤。血清α-胎蛋白正常。手术切除证实胰腺肝样腺癌伴淋巴结病变阳性和边缘阴性。没有放射学或肉眼证据表明远处扩散。观察和辅助治疗吉西他滨是可能的选择。患者选择在离家较近的地方接受治疗,并将继续进行监测成像。结论:胰腺HC在胃肠道肿瘤中是一种罕见的肿瘤,仅有23例报告。对于这种可能异质性的肿瘤,尚无明确的术后辅助标准治疗方法。虽然手术切除是前期治疗的主要方法,但转移到淋巴结或肝脏的疾病预示着预后不良,可能需要经动脉栓塞、化疗或放疗等治疗。
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引用次数: 11
Adenosquamous Carcinoma of the Pancreas in a Patient with BRCA2 Mutation: A Case Report. 1例BRCA2突变患者的胰腺腺鳞癌:1例报告
Pub Date : 2015-11-01 eCollection Date: 2015-01-01 DOI: 10.1089/crpc.2015.29003.vye
Vincent Yeung, Joshua D Palmer, Noelle Williams, Jonathan C Weinstein, Danielle Fortuna, Ashwin Sama, Jordan Winter, Voichita Bar-Ad

Background: Pancreatic adenosquamous carcinoma (ASC) is an uncommon subtype of pancreatic neoplasm, representing 1-4% of all pancreatic cancers. Given the rarity of this tumor, there is no well-established standard of care regarding treatment. We present the case of a BRCA2-deficient patient who responded tremendously well to a combination of gemcitabine and cisplatin therapy. Case presentation: A 66-year-old Caucasian man presented with a 2-week duration of progressively worsening clay-colored stools, tea-colored urine, and jaundice. Computed tomography scan of the abdomen revealed a 4-cm mass at the head of the pancreas. Preoperative carbohydrate antigen (CA) 19-9 was 255 U/mL (normal <37 U/mL). The patient underwent an uncomplicated pylorus-preserving pancreaticoduodenectomy with pathology revealing 11/12 positive lymph nodes, positive resection margins, perineural invasion, lymphovascular invasion, and positive disease in two distant perihepatic lymph nodes. The patient received one cycle of combination of gemcitabine and abraxane, was subsequently found to be BRCA2 deficient, and completed five cycles of gemcitabine and cisplatin thereafter. CA 19-9 before chemotherapy was 203 U/mL. Postchemotherapy CA 19-9 was 13 U/mL. As of today, the patient continues to do well 22 months postresection without radiographical or gross evidence of disease. Conclusion: Gemcitabine in combination with a platinum agent shows promise in the treatment of pancreatic ASC, particularly in setting of BRCA2 deficiency.

背景:胰腺腺鳞癌(ASC)是一种罕见的胰腺肿瘤亚型,占所有胰腺癌的1-4%。鉴于这种肿瘤的罕见性,目前还没有完善的治疗标准。我们提出了一例brca2缺陷患者,他对吉西他滨和顺铂联合治疗反应非常好。病例介绍:一名66岁的白人男性,表现为持续2周的逐渐恶化的泥色大便,茶色尿液和黄疸。腹部计算机断层扫描显示胰腺头部有一个4厘米的肿块。术前碳水化合物抗原(CA) 19-9为255 U/mL(正常)结论:吉西他滨联合铂类药物治疗胰腺ASC有希望,特别是在BRCA2缺乏的情况下。
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引用次数: 2
期刊
Case reports in pancreatic cancer
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