Granular Cell Tumor - An Important Entity to Consider on Upper Gastrointestinal Endoscopy.

Granular cell tumors (GCT) are benign, submucosal tumors derived from neural or Schwann cells. They are commonly found in the oral cavity, skin, breast and tongue. Less commonly, they can also occur in the gastrointestinal tract, usually in the esophagus. We describe two cases of esophageal GCT which were discovered by upper endoscopy (EGD). Case one underwent endoscopic ultrasound (EUS) guided fine needle aspiration and biopsy and case two underwent biopsy of a submucosal nodule. Both biopsies were histologically diagnosed as GCT with supportive S100 positive immunostaining. GCTs are an important diagnosis for physicians to consider in their differential of esophageal nodule or lesion. Though benign, they may rarely show malignant transformation. Therefore, it is essential to have surveillance with EGD/EUS every two to three years if they are not surgically resected.

Extramedullary Myeloid Sarcoma with Symptoms of Acute Pancreatitis Mimicking Pancreatic Carcinoma.

Extramedullary myeloid sarcomas are extramedullary tumors of immature myeloid cells, often associated with acute myeloid leukemia (AML), chronic myeloid leukemia (CML), or other myeloproliferative diseases. They are usually found in connective/soft tissues, skin, breast and gastrointestinal system. Pancreas is an uncommon site to be involved. We present a case of pancreatic myeloid sarcoma presenting with symptoms of acute pancreatitis including epigastric pain associated with nausea and vomiting, with imaging findings (abdominal CT) suggestive of pancreatic carcinoma, both presenting as hypoattenuating mass. It is important to consider myeloid sarcoma as a differential for pancreatic mass, especially given the history of AML as in our patient, which would lead to timely diagnosis and initiation of chemotherapy.

Diagnostic Challenge With Auto-Immune Pancreatitis and IgG4-Related Disease.

IgG4-related disease (IgG4RD) is a chronic immune mediated condition primarily affecting the hepato-pancreatico-biliary system. We report a case of IgG4RD with extensive pancreatic and hepatic involvement masquerading as metastatic pancreatic malignancy posing a diagnostic and therapeutic dilemma.

Utility of Endoscopic Ultrasound in the Work Up for Solitary Hepatobiliary Neuroendocrine Lesions.

A 43-year-old woman, with an unremarkable past medical history, presented with a three-week history of generalized itching, jaundice, and abdominal pain. Initial workup showed amorphous, regionally invasive, and obstructing soft tissue mass in the region of the hepatic hilum. The middle third of the main bile duct was subsequently found to harbor a polypoid mass on endoscopic retrograde cholangiopancreatograph. Biopsy revealed nests of neoplastic cells that was subsequently identified as well-differentiated neuroendocrine tumor. A search for a possible primary neuroendocrine tumor was performed and included imaging of the chest, abdomen, and pelvis, a colonoscopy, capsule endoscopy, and an octreotide scan; however, no primary tumor outside of the liver was identified. Surgical debulking was performed, during which intraoperative exploration and ultrasound failed to reveal any extra-hepatic tumor sanctuaries. A few months later, patient underwent endoscopic ultrasound (EUS) for evaluation of recurrent abdominal pain which revealed a small lesion in the pancreas. It was unclear, however, whether it was primary or a metastatic lesion. This case represents a diagnostic challenge and emphasizes the potential utility of EUS in the preoperative work up for any presumable primary hepatobiliary neuroendocrine tumor.

Undifferentiated carcinoma of pancreas: a rare entity with aggressive behavior and possible genetic link.

UNLABELLED: An aggressive undifferentiated carcinoma of pancreas is a rare tumor, especially with a strong personal and family history of malignant melanoma. Limited literature review and few case reports described a genetic association between distinct types of pancreatic cancer and malignant melanoma. CASE REPORT: We present an uncommon case of an aggressive undifferentiated carcinoma of the pancreas in a 50-year-old Caucasian female. Initially, she presented with intermittent epigastric postprandial pain and mild nausea. A computed tomography scan of the abdomen showed a 5 cm heterogenous pancreatic tail mass, which on biopsy was found to be a poorly differentiated non-small cell carcinoma. Past medical history included malignant melanoma, with extensive family history of pancreatic cancer and malignant melanoma. However, not genetically confirmed, a hereditary pancreatic linkage was highly suspected. A week later, repeat computed tomography demonstrated tremendous enlargement of the pancreatic tail mass. Within a few weeks, the patient developed aggressive metastasis in various organ systems, followed by multiple surgeries. An emergent first round of chemotherapy was started, followed by an intensive care unit stay, and she eventually died. CONCLUSION: Our case exposes the aggressive behavior of undifferentiated carcinoma of pancreas, along with possible hereditary link between pancreatic cancer and malignant melanoma.

Educational climate of a pathology residency program at a tertiary care hospital.

Evaluating educational climate (EC) is imperative for ensuring postgraduate trainees' competencies and quality in residency training programs. This study assessed the EC experiences of pathology postgraduate residents (PGRs) during their postgraduate training in pathology residency programs-a cross-sectional study design assigned EC scores in the pathology residency program at a prestigious institution in Islamabad, which were measured using the Dutch Residency Educational Climate Test (D-RECT) questionnaire. Scores from the D-RECT were employed to conduct descriptive statistics and comparison of means across groups to evaluate EC scores by years of training and compared to assess where the differences were located. Among FCPS-II pathology residents, most of whom were females (94.4%), the mean age was 28.11±2.91 years. A mean positive score was observed among all pathology residents (M≥3.6) for all D-RECT subscales except for the feedback subscale: the average score for feedback was below the average mean score of 3.6 (M = 3.19). A significant difference p = 0.016 was observed in EC scores across different groups through the Analysis of Variances (ANOVA) test. The most significant difference was between less than two and greater than two groups p = 0.027, followed by the difference between equal to two groups and greater than two groups p = 0.052. Overall, positive scores for EC in the pathology residency program were observed. Thus, targeted interventions are needed to increase feedback scores and address observed differences in EC scores by years of training.

Comparing Patient Comfort During Bronchoscopy Under Conscious Sedation and Monitored Anesthesia Care: A Prospective, Observational, Controlled Study.

BACKGROUND: Flexible bronchoscopy (FB) often involves sedation, with the choice left to the bronchoscopist's discretion. Prior research on sedation in gastroscopic endoscopies yields conflicting information regarding the preferred method for FB. This study compares patient comfort levels during bronchoscopy with mindful sedation using fentanyl, nalbuphine, and midazolam versus monitored anesthesia care (MAC) using propofol, midazolam, and ketamine. METHODS: This prospective observational study assessed 83 patients undergoing bronchoscopy under either conscious sedation (CS) (n=40) or MAC (n=43). Patient comfort, sedation levels, emotional state, recovery time, safety, and the impact of smoking history and comorbidities were evaluated. Data collection included direct patient questioning and observation using the Modified Observed Assessment of Alertness and Sedation (MOAA/S) form. RESULTS: Comfort levels were similar between groups, with mean scores of 3.6±0.89 for CS and 3.3±0.54 for MAC. MAC induced deeper sedation (mean scores: 4.37±0.66 vs. 3.8±0.98). Recovery time and complications were comparable. Emotional states and medical history did not significantly differ between groups. CONCLUSION: CS is not inferior to MAC for bronchoscopy, providing comparable comfort and safety with less intense sedation and lower cost. These findings support the use of CS for bronchoscopy procedures, offering a cost-effective alternative without compromising patient comfort or safety.

Impact of Sarcopenia on Post-Liver Transplant Hospitalization: Insights From a South Asian Cohort.

BACKGROUND: Sarcopenia's impact on post-liver transplant outcomes remains a subject of debate, with limited data from South Asia on its association with post-liver transplant hospital stays. This study aims to investigate sarcopenia's influence on post-transplant hospitalization duration in South Asians. METHODS: In this retrospective study, patients with liver cirrhosis who underwent living-donor liver transplantation (LDLT) at Shifa International Hospital in Islamabad, Pakistan, between January 2022 and January 2023 were included. Computed tomography (CT) images were used to assess the skeletal muscle index (SMI). The areas of the psoas, erector spinae, multifidus, quadratus lumborum, rectus abdominis, transverse abdominis, and internal/external oblique muscles were quantified at the level of L3. The data were analyzed using SPSS version 29.0 (IBM). RESULTS: There was a total of 84 patients. Mean age was 47.4 ± 12.0 years. There were 62 (73.8%) male patients and 22 (26.2%) female patients. Hepatitis C was noted in 36 (42.9%) patients. Twenty-two (26.2%) patients had hepatocellular carcinoma. Sarcopenia was identified in 58 (69.0%) patients. No significant association was observed between sarcopenia and intensive care unit (ICU) or general floor stays. Regression analysis identified pre-transplant model for end-stage liver disease-sodium (MELD-Na) score as the sole significant factor associated with both ICU and total length of stay (P value .002; P value .009). CONCLUSION: In our population, sarcopenia did not correlate with post-transplant ICU or overall hospital stay. The pre-transplant MELD-Na score emerged as the most influential predictor of length of stay. Therefore, delaying liver transplant procedures based on muscle mass estimations may not be a practical clinical approach for South Asian patients.

Patient Selection for Living Donor Liver Transplantation in Acute-on-chronic Liver Failure.

Background and objectives: Acute-on-chronic liver failure (ACLF) is associated with high short-term mortality without liver transplantation (LT). The selection criteria for LT in these patients are not well defined. The objective of this study was to determine factors associated with post-transplant survival in ACLF. Methods: This was a single-center retrospective study of patients who underwent living donor liver transplantation (LDLT) for ACLF between 2012 and 2022. Out of 1093 transplants, 110 patients had underlying ACLF, based on the European Association for the Study of the Liver-Chronic Liver Failure Consortium (EASL-CLIF) criteria. We looked at factors associated with 1-year posttransplant survival. Results: The median model for end-stage liver disease (MELD) score was 33.5 (31-38), and the 1-year posttransplant survival was 72%. Six risk factors were associated with posttransplant survival, namely, body mass index > 30 kg/m2 [HR, 4.4; 95% CI, 1.8-10.7], platelet count < 66,000/µl [HR, 2.91; CI,1.2-6.6], poor response to medical treatment [HR, 2.6; CI, 1.1-5.7], drug-resistant bacterial or fungal cultures [HR, 4.2; CI, 1.4-12.4], serum creatinine > 2.5 mg/dl [HR, 3.4; CI, 1.5-7.7], and graft-to-recipient weight ratio < 0.7 [HR, 4.8; CI, 1.4-16.3]. The 1-year post-transplant survival was 84% in patients with 0-2 risk factors (n = 89) and was 6% with 3 risk factors (n = 15) (P < 0.001). For 1-year posttransplant survival, the area under curve (AUC) for the current model was 0.8 (0.69-0.9). The AUC for CLIF-ACLF, Chronic Liver Failure-Sequential Organ Failure Assessment (CLIF-SOFA), and EASL-CLIF ACLF grades was < 0.5. Conclusion: In LT for ACLF, acceptable survival can be achieved when less than three high-risk factors are present.

EUS-FNA for pancreatic neuroendocrine tumors: a tertiary cancer center experience.

OBJECTIVES: Pancreatic neuroendocrine tumors (PNET) are fairly uncommon. Recent data highlight the importance of EUS in diagnosis of PNET. With this background, we decided to review our experience from a tertiary cancer center with regard to the presentation and clinical features of PNET and the diagnostic utility of EUS-FNA in this scenario. METHODS: We identified patients who underwent EUS at our institution between January 1st 2001 and December 31st 2009 for a suspected PNET. Data on clinical features, cross-sectional imaging findings, EUS findings, and cytology results were collected. RESULTS: A total of 81 patients were referred for EUS-FNA for a suspected PNET. Mean age was 58.1 years. There were 41 (50.6%) males. PNET was found incidentally in 38 (46.9%) patients. Computed tomography scanning identified a pancreatic mass in 72 out of 79 (91.1%) cases. Mean diameter of the largest lesion seen on EUS was 27.5 mm (range: 6.9-80 mm). The most common site (34; 42%) was the head of the pancreas. EUS-FNA correctly confirmed a PNET in 73 out of 81 cases with diagnostic accuracy of 90.1%. Seven (8.6%) out of 81 patients had functional lesions, including three gastrinomas and four insulinomas. Liver metastases were found in 31 out of 81 (38.3%) cases. Of the 31 patients with liver metastasis, the mean diameter of lesions on EUS was 33.9 mm compared with 23.5 mm in patients without liver metastasis (P = 0.005). CONCLUSION: EUS-FNA is a reliable modality for further characterization of suspected lesions and for establishing a tissue diagnosis. The occurrence of complications of EUS-FNA in this setting is low. Non-functional PNET are more frequently encountered than functional PNET.

Metastatic tumors to the pancreas: Balancing clinical impression with cytology findings.

BACKGROUNDS/AIMS: Metastatic lesions of the pancreas (PMET) account for 1%-5% of all malignant solid pancreatic lesions (SPL). In this study we evaluated the utility of endoscopic ultrasonography with fine needle aspiration (EUS-FNA) in diagnosing PMET. METHODS: Patients who underwent EUS-FNA at a community referral center between 2011-2017 for SPL were identified. Clinical, radiologic, and EUS-FNA features of those with PMET were compared to those with primary solid tumors of the pancreas: pancreatic adenocarcinoma (PDAC) and neuroendocrine tumors (PNET). RESULTS: A total of 191 patients were diagnosed with solid pancreatic malignancy using EUS-FNA: 156 PDAC, 27 PNET, and eight (4.2%) had PMET. Patients with PMET were less likely to have abdominal pain (25.0% vs. 76.3% vs. 48.2%; p < 0.01) or obstructive jaundice (37.5% vs. 58.3% vs. 0%; p < 0.01) compared to PDAC and PNET. Those with PMET were more likely to have mass lesions with/without biliary or pancreatic ductal dilatations (100% vs. 86.5% vs. 85.2%; p < 0.01) and lower CA19-9 (82.5 ± 43.21 U/mL vs. 4,639.30 ± 11,489.68 U/mL vs. 10.50 ± 10.89 U/mL; p < 0.01) compared to PDAC and PNET. Endosonographic features were similar among all groups. Seven (87.5%) patients with PMET had a personal history of malignancy prior to PMET diagnosis. The primary malignancy was renal cell carcinoma in five PMET. CONCLUSIONS: PMET are exceedingly rare, comprising less than 5% of SLP. Patients with PMET are less likely to present with symptoms and mostly identified by surveillance imaging for the primary malignancy.

Giant gastric hyperplastic polyp: not always a benign lesion.

Giant gastric hyperplastic polyps constitute of around 76% of all gastric polyps found. They are often found incidentally on upper GI endoscopy. They often present with occult GI bleeding causing iron deficiency anemia or partial gastric outlet obstruction. Although mostly benign, they do have potential for malignant transformation and hence must be excised endoscopically or surgically, whichever may be feasible. We hereby present a couple of cases of gastric hyperplastic polyps in an attempt to add to the current literature on this rather rare entity.

Spatial Epidemiology of Pancreatic Cancer in South Dakota.

BACKGROUND: Pancreatic cancer is the fourth leading cause of cancer-related mortality in the United States. Descriptive epidemiology of pancreatic cancer in South Dakota has not been studied before. MATERIALS AND METHODS: All cases of pancreatic cancer reported to the SD Cancer Registry between January 2005 and December 2014 were included in the study. Variables collected included demographics, geographical location including county of residence and zip codes, date of diagnosis, date of last contact or follow-up, size and location of the tumor, grade of the tumor, diagnostic modality as well as therapeutic interventions. Log rank test was used to compare survival curves. Kaplan-Meier product limit estimates were provided. Data was analyzed using SAS 9.1.4 software. RESULTS: One thousand sixty-four cases of pancreatic cancer were reported. Median age was 73 years. Cumulative age-adjusted incidence rate for pancreatic cancer for 2005-2014 was 11.1. Cumulative age-adjusted mortality rate for pancreatic cancer for 2005-2014 was 10.2. Almost half of these patients had distant metastasis at the time of presentation (n = 536; 50.4%). Overall, median survival was 5 months. Median survival for patients under the age of 60 years was 9.5 months as opposed to median survival for patients 60 years or older which was 3.9 months. Median survival of patients with well-differentiated, moderately differentiated, and poorly differentiated tumors, was 20.8 months, 8.2 months and 6.3 months respectively (p value = 0.0017). CONCLUSION: Incidence of pancreatic cancer in South Dakota is similar to the national trends in the United States. Age at presentation, location of tumor in pancreas, and biological behavior of tumor were all predictors of survival in patients with pancreatic cancer.

Distinct Clinical Characteristics in Young-Onset Pancreatic Neuroendocrine Tumor.

BACKGROUND: We aimed to study the effect of socioeconomic differences and molecular characteristics on survival in patients with young-onset pancreatic neuroendocrine tumors (YOPNET) and typical-onset PNET (TOPNET). METHODS: We identified the patients with YOPNET (<50 years) and TOPNET (≥50 years) who underwent definitive surgery diagnosed between 2004 and 2016 using the National Cancer Database. We evaluated overall survival (OS) using the Kaplan-Meier and Cox regression methods before and after propensity score matching. A publicly available genomic dataset was used to compare mutation frequencies among the two groups. RESULTS: A total of 6259 patients with PNET were included, of which 27% were YOPNET. Patients with YOPNET were more likely to be Black, Hispanic, female, and have private insurance versus patients with TOPNET (all p < 0.001). Patients with YOPNET had a lower comorbidity score, but higher stage and tumor size (all p < 0.001). YOPNET was associated with a greater improved OS than TOPNET before and after propensity score matching (p < 0.001). On multivariable analysis, this survival difference persisted for YOPNET as an independent prognostic factor (unmatched p = 0.008; matched p = 0.01). For genomic analysis, patients with YOPNET had a lower rate of multiple endocrine neoplasia type-1 (MEN-1) mutation than patients with TOPNET (26% vs. 56%, p < 0.001). CONCLUSIONS: YOPNET represents a disease with distinct clinical features. Patients with YOPNET who underwent definitive surgery had better OS than patients with TOPNET despite having higher stage and tumor size. YOPNET also had lower rate of MEN-1 mutation.

Finding a needle in a haystack: Endoscopic ultrasound-guided fine-needle aspiration for solid pancreatic masses in the setting of chronic pancreatitis.

BACKGROUND: The mainstay for the definitive diagnosis of pancreatic lesions is endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). However, there is evidence that EUS-FNA has low sensitivity in the setting of chronic pancreatitis (CP). This single-center retrospective study aimed to compare and analyze the diagnostic yield of EUS-FNA for solid pancreatic lesions in the presence and absence of CP, and to further investigate strategies for overcoming the low diagnostic yield in the setting of CP. METHODS: This study identified patients who underwent EUS-FNA at Sanford USD Medical Center (SD, USA) for a solid pancreatic lesion between July 15, 2011, and November 30, 2017. Data on demographics, clinical features, cross-sectional imaging findings, EUS findings, cytology/pathology, and clinical follow up were collected. RESULTS: The final diagnosis was adenocarcinoma in 156 patients (67%), neuroendocrine tumor in 27 (12%), lymphoma in 6 (3%), metastatic malignancy in 8 (4%), and benign etiologies in 35 (15%). CP was identified in 44/234 (19%) patients. The overall diagnostic sensitivity, specificity, positive predictive value, negative predictive value, and accuracy for EUS-FNA were 92.9%, 97.1%, 99.5%, 70.8%, and 93.5%, respectively. The sensitivity (80% vs. 95%, P=0.020) and accuracy (86% vs. 95%, P=0.043) were significantly lower in patients with CP compared to those without CP. CONCLUSION: CP can significantly affect the EUS-FNA diagnostic yield of solid pancreatic neoplasms. A high index of clinical suspicion is required in these cases to make a definitive diagnosis.

Hepatitis E virus antibodies in patients with chronic liver disease.

In the United States, the seroprevalence rate for hepatitis E virus (HEV) is approximately 20%. This study examined HEV seroprevalence in persons with and without chronic liver disease. Our data indicate that HEV seropositivity is high in patients with chronic liver disease and that HEV seroprevalence increases significantly with age.

Isolated tuberculosis of the pancreas: a case report.

CONTEXT: Pancreatic tuberculosis is a rare entity. Only a few cases have been reported in the medical literature. We hereby describe a case of pancreatic tuberculosis in an immunocompromized individual. CASE REPORT: A fifty-year-old African-American gentleman with history of HIV non-compliant on anti-retroviral therapy presented with epigastric pain for five weeks duration. CT scan of abdomen showed large necrotic node on the posterior aspect of the head of pancreas and multiple cystic masses adjacent to the pancreas. Acid fast bacilli were found on staining of CT guided biopsy of the node. Cultures grew Mycobacterium tuberculosis. Anti-tubercular therapy was initiated and resulted in gradual resolution of symptoms. CONCLUSION: Pancreatic tuberculosis is rare and is frequently confused with pancreatic cancer on clinical presentation as well as on imaging studies. Since it is a curable disease, accurate diagnosis is paramount CT or ultrasound guided biopsy is cornerstone of diagnosis. Endoscopic ultrasound is now increasingly being used for obtaining tissue for diagnosis. Anti-tubercular therapy is curative in majority of the cases.