scholarly journals Pancreatic Origin Hepatoid Adenocarcinoma with Liver Metastasis

2021 ◽  
Vol 8 (10) ◽  
Author(s):  
Salehi M ◽  
◽  
Nwachukwu N ◽  
Grijalva V ◽  
Li LZ ◽  
...  
Keyword(s):  
Pancreatic Adenocarcinoma ◽  
Epigastric Pain ◽  
Pancreatic Head ◽  
Fine Needle Biopsy ◽  
Ampulla Of Vater ◽  
Hepatoid Adenocarcinoma ◽  
Outpatient Chemotherapy ◽  
Poorly Differentiated ◽  
Specific Symptoms ◽  
Pancreatic Origin

Hepatoid Adenocarcinoma (HAC) is a rare form of aggressive extrahepatic neoplasm with similar morphologic features to Hepatocellular Carcinoma (HCC). HAC with pancreatic origin is rare and the exact incidence is unknown. Given shared morphological and Immunohistochemical (IHC) characteristics, it may be difficult to distinguish metastatic HAC with liver involvement from primary HCC. We present a rare case of ductal type pancreatic hepatoid adenocarcinoma involving the pancreatic head, ampulla of Vater, and liver, and illustrate strategies for diagnosis and treatment. A 65-year-old woman presented with epigastric pain, vomiting, melena, and weight loss. There was direct hyperbilirubinemia with elevated hepatic markers. Imaging displayed a pancreatic head mass with moderate biliary obstruction and a hepatic lobe lesion. Fine needle biopsy of the liver mass initially was consistent with HCC, and biopsies of the pancreatic mass and ampulla walls showed pancreatic adenocarcinoma. Due to the unusual finding of co-existing HCC and pancreatic adenocarcinoma, the hepatic mass biopsy was sent for external evaluation, which revealed poorly differentiated adenocarcinoma, consistent with HAC of pancreatic origin. The tumor was positive for mucicarmine stain, HepPar1, CEA, and CK7. It was negative for MOC-31, Arginase, ALBISH, MGB, ER, TTF-1, GCDFP15, CK-20 and CDX2, thus confirming HAC. The patient was referred to outpatient chemotherapy with gemcitabine and paclitaxel however demonstrated progression and expired following recurrent bilateral pulmonary emboli. HAC may present with non-specific symptoms, is highly aggressive, and may be difficult to distinguish from primary HCC from histopathologic characteristics alone. Accurate diagnosis requires clinicohistopathologic and IHC analysis.

Radical Resection in Signet Ring Carcinoma of the Ampulla of Vater: Report of an 11-Year Survivor

2006 ◽  
Vol 72 (2) ◽  
pp. 193-195 ◽  
Author(s):  
Mark Bloomston ◽  
Michael Walker ◽  
Wendy L. Frankel
Keyword(s):  
Radical Resection ◽  
Ampulla Of Vater ◽  
Signet Ring Cell ◽  
Gastrointestinal Malignancies ◽  
Dismal Prognosis ◽  
Signet Ring ◽  
Painless Jaundice ◽  
Poor Differentiation ◽  
Poorly Differentiated

Carcinomas of the ampulla of Vater are uncommon tumors but have a better prognosis than typical pancreatic cancers. They tend to be well or moderately differentiated while the poorly differentiated variants are still quite aggressive. Signet ring features associated with poor differentiation traditionally confer a dismal prognosis in other gastrointestinal malignancies, particularly gastric cancer. Signet cell ring morphology has only been described in a few ampullary carcinomas with all cases reporting short follow-up. We describe a 58-year-old woman who presented with painless jaundice and a prominent ampulla of Vater by endoscopy. She underwent pancreaticoduodenectomy and was found to have a 1.0 x 0.8 cm poorly differentiated carcinoma with prominent signet ring cell features arising from the ampulla of Vater and invading into the periampullary duodenum but sparing the pancreatic parenchyma. No distant or nodal metastases were seen (pT2N0M0). No adjuvant therapy was given, and the patient remains free of disease with 134 months of follow-up.

scholarly journals Factors affecting overall survival in ductal adenocarcinoma of the pancreatic head. Experience of one center

2021 ◽  
Vol 11 (1) ◽  
pp. 20-28
Author(s):  
D.  M. Kuchin ◽  
Ya.  I. Kolesnik ◽  
H.  G. Torgomyan ◽  
V.  E. Zagainov
Keyword(s):  
Overall Survival ◽  
Adjuvant Chemotherapy ◽  
Vascular Invasion ◽  
Survival Rates ◽  
Pancreatic Head ◽  
Ductal Adenocarcinoma ◽  
Factors Affecting ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Major Factors

Purpose. To identify major factors affecting the overall survival (OS). To select the cohort of patients with the best prognosis.Materials and methods. A retrospective analysis included data of 268 patients, 128 men and 140 women, with median age of 59±10,53 (30 to 83) years. For multivariate analysis of survival, patients were selected who underwent pancreaticoduodenectomy (PD) for ductal adenocarcinoma of the pancreatic head.Results. Our study demonstrated that histologically verified vascular invasion (detected only in 30 % of patients who underwent PD with resection of the major vessels) statistically significantly affected the OS. The increased CA19-9 level over 500 U / L (detected in 32,3 % of cases) is the factor that significantly worsens the OS. Patients with high grade adenocarcinoma have significantly better survival rates compared with patients who have moderately or poorly differentiated adenocarcinoma (p = 0.014; median 26 months, 95 % CI 4.4–47.6 versus median 17 months, 95 % CI 15–19, an median: 13 months, 95 % CI 5–21, respectively). Also, the use of adjuvant chemotherapy has a positive effect on long-term outcomes (p = 0.0001; median 26 months, 95 % CI 21.7–30.3 versus median 13 months, 95 % CI 11.3–14.7).Conclusion. A well-differentiated tumor and the use of adjuvant chemotherapy significantly increase the OS of patients. Poorly differentiated tumor, CA19-9 level over 500 U / mL and the histologically confirmed vascular invasion significantly worsen the prognosis of these patients.

scholarly journals Well differentiated endocrine carcinomas of the pancreas

2011 ◽  
Vol 139 (9-10) ◽  
pp. 625-630
Author(s):  
Radoje Colovic ◽  
Nikica Grubor ◽  
Marjan Micev ◽  
Vladimir Dugalic ◽  
Dejan Stojakov ◽  
...  
Keyword(s):  
Distal Pancreatectomy ◽  
Pancreatic Head ◽  
Distant Metastases ◽  
Massive Bleeding ◽  
R0 Resection ◽  
Gastric Bleeding ◽  
Poorly Differentiated ◽  
The Difference ◽  
Well Differentiated ◽  
Endocrine Carcinomas

Introduction. For the difference from poorly differentiated, well differentiated endocrine carcinomas of the pancreas are the tumours in whom with aggressive surgery and chemotherapy fair results can be achieved. Objective. The aim of the study was to point out the importance of such treatment. Methods. Over a 6-year period eight patients (seven female and one male) of average age 51 years (ranging from 23 to 71 years) were operated on for well differentiated endocrine carcinoma: six of the head and two of the tail of the pancreas. There were two functional and six nonfunctional tumours. Pain in the upper part of the abdomen in seven, mild loss in weight in two, strong heartburn in two, obstructive jaundice in three, diarrhoea in one, sudden massive bleeding from gastric varicosities due to prehepatic portal hypertension caused by pancreatic head tumour in one, and bruise in one patient were registered preoperatively. US and CT in all, angiography in one, octreoscan in two and PET scan in one patient were performed. Whipple?s procedure was performed in six and distal pancreatectomy in two patients, as well as systemic lymphadenectomy in all and excision of liver secondary tumours in two patients. In the patient with massive gastric bleeding a total gastrectomy was performed first, followed by Whipple?s procedure a month later. Results. R0 resection was achieved in all patients. Lymph nodes metastases were found in six patients. Six patients were given chemotherapy. One patient died 3 years after surgery, seven are still alive, on average 2.5 years. A local recurrence after distal pancreatectomy that occurred 5 years after surgery was successfully reresected and the patient is on peptide-receptor radiotherapy. In other six patients there were no local recurence or distant metastases. Conclusion. With aggressive surgery and chemotherapy fair results can be achieved in well differentiated endocrine carcinomas of the pancreas.

Ampullary neuroendocrine tumors: A window into a rare tumor using a national database.

2021 ◽  
Vol 39 (3_suppl) ◽  
pp. 373-373
Author(s):  
Samantha M Ruff ◽  
Gary B Deutsch ◽  
Matthew John Weiss ◽  
Danielle Deperalta
Keyword(s):  
Overall Survival ◽  
Neuroendocrine Tumors ◽  
Cox Regression ◽  
Case Series ◽  
Pancreatic Head ◽  
National Database ◽  
Rare Tumor ◽  
Cox Regression Analysis ◽  
Significant Difference ◽  
Poorly Differentiated

373 Background: Ampullary neuroendocrine tumors (NET) make up < 1% of all gastrointestinal NETs. Information about their behavior and prognosis is reliant on small case series. This study set out to describe the population of patients who are diagnosed with ampullary NETs and compare them to patients with duodenal and pancreatic head NETs. Methods: The National Cancer Database (2004 – 2016) was queried for patients with ampullary, duodenal, and pancreatic head NETs. Clinicopathologic and treatment characteristics were compared. Subset analysis was performed on patients who underwent surgery. Kaplan Meier (KM) analysis and Cox regression were used to analyze the survival of patients with ampullary NETs. Results: Overall, 872 patients were identified with ampullary NET, 9692 with duodenal NET, and 6562 with pancreatic head NET. Patients with ampullary NET had an average age of 60.9 +/- 14.5 years, were evenly split among men and women (N = 437, 50.1% vs N = 435, 49.9%, respectively), and primarily Caucasian (N = 663, 76.0%). 72.1% underwent local tumor destruction or surgery (N = 629). Most did not receive radiation (N = 832, 95.4%), chemotherapy (N = 627, 71.9%), or hormone therapy (N = 788, 90.4%). Patients with ampullary NETs had more poorly differentiated tumors (N = 119, 13.6%) than patients with duodenal (N = 159, 1.6%) or pancreatic head (N = 602, 9.2%) NETs. Patients with ampullary NETs had more positive lymph nodes (N = 288, 33%) than patients with duodenal (N = 915, 9.4%) or pancreatic head (N = 1381, 21%) NETs. At five years, the overall survival for patients with ampullary, duodenal, and pancreatic head NETs was 57%, 68%, and 46%, respectively. Within the surgical population, five-year survival for patients with ampullary (N = 367), duodenal (N = 991), and pancreatic head (N = 1961) NETs was 60%, 74%, and 72%, respectively. When compared, there was a statistically significant difference between the mean overall survival of patients with ampullary (98 +/- 4.7 months), duodenal (112 +/- 2.5 months), and pancreatic head (108 +/- 1.7 months) NETs (p < 0.001). In the cox regression analysis, sex, Charlson-Deyo score, lymph node positivity, lymph-vascular invasion, mitotic rate, chromogranin A level, 5-HIAA level, and tumor size did not correlate with survival. Increasing age (HR 1.04, CI 1.01 – 1.07, p = 0.008) and worse tumor differentiation (poorly differentiated HR 3.33, CI 1.38 – 8.04, p = 0.008 and undifferentiated HR 8.31, CI 2.77 – 24.92, p < 0.001 compared to well differentiated) were associated with increased mortality. Conclusions: This study sheds light on a rare tumor histology. When compared to patients who underwent surgical resection for duodenal or pancreatic head NETs, patients with ampullary NETs had a significantly worse prognosis. Identifying prognostic factors allows us to create more concrete treatment recommendations and provide patients with improved prognostic information.

Paneth Cell Carcinoma of the Ampulla of Vater

2004 ◽  
Vol 128 (8) ◽  
pp. 908-910
Author(s):  
Jorge I. Mora ◽  
Julieta E. Barroeta ◽  
David A. Bernstein ◽  
Juan Lechago
Keyword(s):  
Cell Carcinoma ◽  
Paneth Cell ◽  
Ampulla Of Vater ◽  
Paneth Cells ◽  
Biological Behavior ◽  
Neoplastic Cells ◽  
Poorly Differentiated Adenocarcinoma ◽  
Ca 19.9 ◽  
Positive Immunostaining ◽  
Poorly Differentiated

Abstract We describe a Paneth cell carcinoma arising within the ampulla of Vater in a 64-year-old man. The phenotype of virtually all neoplastic cells was consistent with that of Paneth cells, based on routine morphology and their strong positive immunostaining for lysozyme. Additional widespread positive immunostaining for carcinoembryonic antigen and CA 19.9 supports a totipotential cell as the origin of such neoplastic cells. This case, therefore, represents a true Paneth cell carcinoma, as opposed to inclusion of occasional neoplastic Paneth cells into a poorly differentiated adenocarcinoma. This pattern of differentiation is rare, and predictions regarding its ultimate biological behavior and malignant potential must be guarded.

scholarly journals Intraluminal Duodenal Diverticulum: A Rare Cause of Chronic Epigastric Pain

2020 ◽  
Vol 06 (02) ◽  
pp. e125-e127
Author(s):  
Shadi Nassar ◽  
Alaa El-Kheir ◽  
Charif Khaled ◽  
Anis Nassar ◽  
Joseph Boujaoude ◽  
...  
Keyword(s):  
Epigastric Pain ◽  
Case Reports ◽  
Duodenal Diverticulum ◽  
Rare Entity ◽  
Epigastric Discomfort ◽  
Asymptomatic Patients ◽  
Intraluminal Duodenal Diverticulum ◽  
Diagnostic Modalities ◽  
Specific Symptoms

AbstractDespite its first identification in 1885, intraluminal duodenal diverticulum remains a rare entity and only a few case reports are found in the literature. Its diagnosis is almost always delayed due to the lack of specific symptoms and to the very vague presentation consisting of mild epigastric discomfort. However, with the aid of new diagnostic modalities and imaging, it has become easier to diagnose this entity when its symptoms persist. Finally, it can remain undiagnosed in asymptomatic patients.

Neoadjuvant Therapy Followed by Resection Versus Upfront Resection for Resectable Pancreatic Cancer: A Propensity Score Matched Analysis

2017 ◽  
Vol 35 (5) ◽  
pp. 515-522 ◽  
Author(s):  
Ali A. Mokdad ◽  
Rebecca M. Minter ◽  
Hong Zhu ◽  
Mathew M. Augustine ◽  
Matthew R. Porembka ◽  
...  
Keyword(s):  
Overall Survival ◽  
Propensity Score ◽  
Adjuvant Therapy ◽  
Neoadjuvant Therapy ◽  
Pancreatic Adenocarcinoma ◽  
Early Stage ◽  
Clinical Stage ◽  
Pancreatic Head ◽  
Hazard Ratio ◽  
Stage I

Purpose To compare overall survival between patients who received neoadjuvant therapy (NAT) followed by resection and those who received upfront resection (UR)—as well as a subgroup of UR patients who also received adjuvant therapy—for early-stage resectable pancreatic adenocarcinoma. Patients and Methods Adult patients with resected, clinical stage I or II adenocarcinoma of the head of the pancreas were identified in the National Cancer Database from 2006 to 2012. Patients who underwent NAT followed by curative-intent resection were matched by propensity score with patients whose tumors were resected upfront. Overall survival was compared by using a Cox proportional hazards regression model. Early postoperative and oncologic outcomes were evaluated. Results We identified 15,237 patients with clinical stage I or II resected pancreatic head adenocarcinoma. From the NAT group, 2,005 patients (95%) were matched with 6,015 patients who underwent UR. The NAT group was associated with improved survival compared with UR (median survival, 26 months v 21 months, respectively; stratified log-rank P < .01; hazard ratio, 0.72; 95% CI, 0.68 to 0.78). Patients in the UR group had higher pathologic T stage (pT3 and T4: 86% v 73%; P < .01), higher positive lymph nodes (73% v 48%; P < .01), and higher positive resection margin (24% v 17%; P < .01). Compared with a subset of UR patients who received adjuvant therapy, NAT patients had a better survival (adjusted hazard ratio, 0.83; 95% CI, 0.73 to 0.89). Conclusion NAT followed by resection has a significant survival benefit compared with UR in early-stage, resected pancreatic head adenocarcinoma. These findings support the use of NAT, particularly as a patient selection tool, in the management of resectable pancreatic adenocarcinoma.

RTOG 9704 a phase III study of adjuvant pre and post chemoradiation (CRT) 5-FU vs. gemcitabine (G) for resected pancreatic adenocarcinoma

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 4007-4007 ◽  
Author(s):  
W. F. Regine ◽  
K. W. Winter ◽  
R. Abrams ◽  
H. Safran ◽  
J. P. Hoffman ◽  
...  
Keyword(s):  
Pancreatic Adenocarcinoma ◽  
Pancreatic Head ◽  
Phase Iii ◽  
Hematologic Toxicity ◽  
Tumor Diameter ◽  
Head Tumors ◽  
Post Surgery ◽  
Significant Difference ◽  
Phase Iii Study ◽  
Grade 3

4007 Background: RTOG 9704 was designed to determine if the addition of G to postoperative adjuvant 5-FU CRT improved survival for patients(pts) with resected pancreatic adenocarcinoma. Methods: In this Intergroup trial involving RTOG, ECOG and SWOG, pts post gross total resection of pancreatic adenocarcinoma (path stage T1 - 4, N0 - 1, M0) were randomized to receive pre and post CRT 5-FU vs pre and post CRT G. 5-FU = continuous (CI) at 250 mg/m2/day. G = 1000 mg/m2 IV weekly. Both were given over 3 weeks pre and 12 weeks post - CRT. CRT = 50.4 Gy 1.8 Gy/fx/day with CI 5-FU, 250 mg/m2/day during RT for all pts. Pts were stratified by nodal status (uninvolved vs involved), primary tumor diameter ( < 3 cm vs ≥ 3 cm) and surgical margins (negative vs positive vs unknown). Survival was the primary endpoint with an original targeted accrual of 330 pts. Rapid enrollment allowed study amendment for increased targeted accrual to add survival among pts with lesions of the pancreatic head as a primary, prospective endpoint. Results: From 7/98 - 7/02, 538 pts were entered; 442 were eligible and analyzable. Major reasons for patient ineligibility were serum not sent for CA-19–9 analysis (n=22) and treatment starting > 8 weeks post surgery (n=19). Treatment arms were well balanced except for T-stage (T3/4 > for G, p=0.013). Pts with pancreatic head tumors(n=380) experienced significantly improved survival, with median and 3-year survival of 18.8 months and 31% respectively for the G arm vs. 16.7 months and 21% for the 5-FU arm (p=0.047; HR=0.79, CI=0.63–0.99). When analysis was inclusive of pts with body/tail tumors(n=442) no significant difference in survival was found (p=0.20). No significant difference in non-hematologic grade ≥ 3 toxicity was seen. The grade 4 hematologic toxicity rate was 14% in the G arm and 2% in the 5-FU arm (p<0.0001) without difference in febrile neutropenia/infection. The ability to complete chemotherapy (86%, 5-FU vs. 90%, G) and RT (85%, 5-FU vs. 88%, G) as per study was similar. Conclusions: The addition of G to postoperative adjuvant 5-FU CRT significantly improves survival in pts with pancreatic head adenocarcinoma. No significant financial relationships to disclose.

scholarly journals Adult Burkitt’s lymphoma presenting as intussusception: first UK case report

2017 ◽  
Vol 99 (7) ◽  
pp. e206-e208 ◽  
Author(s):  
R Simson ◽  
A Planner ◽  
Roderick Alexander
Keyword(s):  
Epigastric Pain ◽  
Recurrent Abdominal Pain ◽  
Burkitt’S Lymphoma ◽  
Right Hemicolectomy ◽  
Burkitt's Lymphoma ◽  
Adult Intussusception ◽  
Lead Point ◽  
History Of ◽  
The Uk ◽  
Specific Symptoms

Colonic intussusception is rare in adults and can present with non-specific symptoms that can make diagnosis difficult and delayed. Unlike in children, it is commonly due to a pathological lead point that is often malignant. This case is the first reported case of adult intussusception due to Burkitt's lymphoma in the UK. We describe the case of a 22-year-old woman who presented with 4-week history of intermittent epigastric pain. On the third hospital admission, the diagnosis was made by abdominal computed tomography, which showed the ‘target’ sign, suggestive of intussusception. A right hemicolectomy was performed and histology later confirmed Burkitt’s lymphoma. This case demonstrates the difficulty in diagnosing intussusception in adults, which must be considered in recurrent abdominal pain when more common causes have been ruled out. The rare diagnosis of Burkitt’s lymphoma made early diagnosis and treatment important.

ID: 33: ACUTE PANCREATITIS WITH NORMAL LIPASE AND AMYLASE ENZYMES

2016 ◽  
Vol 64 (4) ◽  
pp. 941.1-941
Author(s):  
H Alkhawam ◽  
C Catalano ◽  
F Zaiem ◽  
N Vyas ◽  
M Fabisevich ◽  
...  
Keyword(s):  
Acute Pancreatitis ◽  
Abdominal Pain ◽  
Epigastric Pain ◽  
Pancreatic Head ◽  
Uncinate Process ◽  
Oral Contrast ◽  
Significant Past Medical History ◽  
Laboratory Findings ◽  
Lipid Panel ◽  
Basic Metabolic Panel

Case ReportA 44 year-old Male with no significant past medical history presented to the Emergency Department complaining of nausea, vomiting, diarrhea, upper abdominal pain and fever. For the past one week prior to presentation, patient developed pressure-like epigastric pain, radiating to the back, worsened with lying down, and associated with non-bloody, non-bilious vomiting, followed by anorexia, nausea and fever to 102F. Patient had not eaten several days prior to arrival to the hospital; hence he was brought in by his family for evaluation. Notably, two months prior to presentation, patient was evaluated in an outside hospital for abdominal pain similar in quality, but not in intensity, and reportedly had normal blood tests and imaging.Physical examination: vital signs significant for hypertension of 150/90, tachycardia to 108 and fever of 101.5; abdomen notable for tenderness to palpation over epigastrium, with mild guarding, but no rebound or Murphy's sign; the rest of the exam, including cardiovascular, pulmonary, integumentary and neurological exam, unremarkable. Initial laboratory findings are: WBC of 10.1, with 81% neutrophils, amylase of 47 (N 28–100 U/L), lipase level of 14 (N 11–82 U/L), and unremarkable basic metabolic panel. Liver function tests notable for normal AST and ALT, elevated GGT to 277 (N <50 U/L), LDH: 681 (N 90–225 U/L), Total bilirubin: 0.9(N 0–1.5 mg/dl). Lipid panel: Total Cholesterol 201 (N<200 mg/dL), Triglycerides 80 (N<150 mg/dL), LDL 68 (<100 mg/dL). Chest X-ray showed a small left-sided pleural effusion.Patient was admitted to medicine service for treatment of gastroenteritis, and was started on intravenous fluids and symptomatic management. On day three of hospitalization, patient developed worsening abdominal pain, associated with inability to tolerate per oral intake secondary to vomiting of food contents, and due to worsening abdominal pain, underwent further workup. CBC revealed leukocytosis with a left shift, WBC count of 15.3, with 81.5% neutrophils. Basic metabolic panel notable for sodium of 124, potassium of 3.2, calcium of 7.4, magnesium of 1.7, phosphate of 1.9. Repeat lipase was 67(N 11–82 U/L). An abdominal CT scan (figure 1) with IV and oral contrast was performed, and showed extensive pancreatic edema, especially involving the pancreatic head and uncinate process, and peripancreatic stranding; these changes deemed consistent with acute pancreatitis; no calcifications or pseudocysts were observed on the CT. Abdominal ultrasound showed multiple gallbladder stones, however, common bile duct was of normal diameter (2.5 mm), and no intrabiliary duct dilatation was noted.Based on clinical presentation and radiological findings, the diagnosis of acute pancreatitis was made. The patient started on aggressive intravenous fluid hydration, pain management and bowel rest, with good improvement in symptoms. On day 5, patient was able to tolerate a regular diet, and noted an almost complete resolution of pain, and therefore was discharged home.Abstract ID: 33 Figure 1

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