Marjan Khan

Purpose Pancreatic acinar cell carcinoma accounts for 1–2% of pancreatic tumors, with increasing frequency in recent years, and still represents a poor prognosis. This study aims to expand and update existing literature by analyzing national data gathered over almost two decades. Methods Data from 488 patients diagnosed with PACC in the Surveillance, Epidemiology, and End Results database were analyzed. This study employed the Cox regression method to compute hazard ratios and identify independent factors influencing survival. Additionally, Kaplan–Meier survival curves were utilized alongside the log-rank test. Results The median age was 64.7 years with male predilection (70.5%). “Poorly differentiated carcinoma” was the most common subtype (45.8%). The liver was the most common site of metastases (31.3%). The 5-year observed overall survival (OS) rate was 19.2% (95% CI, 14.9–23.8). The 5-year cause-specific survival (CSS) rate was 22.4% (95% CI, 17.7–27.5). Male gender has a 5-year OS of 19.2% (95% CI, 14.0–25.1) compared to female OS of 30.2% (95% CI, 20.7–40.1). Patients treated with multimodal therapy (surgery with chemoradiation) over only surgery or chemotherapy had better 5-year OS, 53.5% (95% CI, 31.8–71.0). Age > 60 and distant stage were independent factors associated with increased mortality. Conclusion Pancreatic acinar cell carcinoma is a rare, aggressive form of pancreatic cancer that primarily affects older adults. Our findings offer valuable insights to guide future clinical guidelines and tailored treatment strategies.

Background Hepatic angiosarcoma (HA) is a rare malignant vascular neoplasm. Currently, there are no standardized protocols for treating HA. This study aims to understand clinicopathologic analysis, prognostic factors, and treatment outcomes comprehensively. Methods The data retrieved from the SEER database was reviewed for hepatic angiosarcoma cases between 2000 and 2021. Results A total of 389 patients with hepatic angiosarcoma were identified with a mean age of 63.9 years (SD +/- 16). Most patients were male (64%), and per US census data, non-Hispanic Asians or Pacific Islanders were the most common race (17%). In known cases of tumor stage (61%), the most common tumor stage was distant (22%), and most were grade III (18%) tumors. Overall, the 3-year survival rate was 6.7% with a 95% confidence interval (95% CI 0.044-0.100), disease-specific survival at a 1-year survival rate was 4.43% (95% CI 0.023-0.083), and no patients survived by 3 years. The best overall survival rate was the 1-year rate for surgical resection, 18.20% (95% CI 0.075-0.441). Chemotherapy had a 1-year survival rate of 11% (95% CI 0.057-0.211), and radiation therapy had no survival significance (p = 0.2). Multivariate analysis shows age above 70 years (H.R. 1.67 (95% CI 1.181-2.381), p < 0.05), no surgical intervention (H.R. 2.29 (95% CI 1.585-3.336) p < 0.001), and distant stage (H.R. 2.54 (95% CI 1.696-3.805) p < 0.001) are negative prognostic factors, whereas female sex (H.R. 0.68 (95% CI 0.536-0.875) p < 0.05) is a positive prognostic factor. Conclusion Increasing age (> 70 years), male sex, and distant stage were found to be strong predictors of poor survival outcomes. Patients had better outcomes when surgical resection and chemotherapy were included in their treatment. These results can provide continued evidence in the future management of patients with hepatic angiosarcoma.

Background: This retrospective study aims to examine the patient demographics, survival rates, and treatment methods for small-cell neuroendocrine carcinoma (SCNEC) and large-cell neuroendocrine carcinoma (LCNEC) of prostate origin while also identifying the main differences between common types of prostate cancer with comparative analysis for survival. Methods: Our analysis utilized the Surveillance, Epidemiology, and End Results database (SEER), and data was collected from 2000–2020. Cox proportional hazards and chi-squared analysis were used for statistical analysis. Results: A total of 718 cases of prostate small and large neuroendocrine carcinoma were identified. The median age was 71.5 years, and the median follow-up was 11.0 years (95% confidence interval (95% CI) = 9.2–12.8). Most patients were over the age of 80 years (33.8%) and Caucasian (74.4%). The overall 5-year survival was 8.0% (95% CI = 6.8–9.2). The 5-year OS for Caucasians was 7.3% (95% C.I. 6.0–8.3). For Black Americans, the 5-year OS was 11.9% (95% C.I. 7.3–16.5). For Hispanics, the 5-year OS was 12.2% (95% C.I. 7.7–16.7). The 5-year cause-specific survival (CSS) was 16.2% (95% CI = 14.3–18.1). For treatment modality, the five-year survival for each were as follows: chemotherapy, 3.5% (95% CI = 2.1–4.9); surgery, 18.2% (95% CI = 13.6–22.8); multimodality therapy (surgery and chemotherapy), 4.8% (95% CI = 1.7–7.9); and combination (chemoradiation with surgery), 5.0% (95% CI = 1.0–9.0). The prognostic nomogram created to predict patient survivability matched the findings from the statistical analysis with a statistical difference found in race, income, housing, stage, and nodal status. The nomogram also indicated a slight increase in mortality with tumors of greater size. This analysis showed a slight increase in mortality for patients of Asian race. In addition, there was a significant increase in death for patients with stage 3 tumors, as well as patients who underwent surgery and radiation. Furthermore, we performed propensity score matching for survival differences, and no survival difference was found between SCNEC and LCNEC. Conclusions: Asian patients, larger tumor size, and distant disease were associated with worse long-term clinical outcomes. By leveraging insights from registry-based studies, clinicians can better strategize treatment options, improving patient outcomes in this challenging oncology arena.

Background: Neuroendocrine carcinomas (NECs) of the tubular gastrointestinal tract (GI-NECs) are rare and associated with worse clinical outcomes. This population-based study aims to highlight key demographics, clinicopathological factors, and survival outcomes in the US population. Methods: Data from 10,387 patients with GI-NECs were extracted from the Surveillance, Epidemiology, and End Result (SEER) database from 2000 to 2020. Results: Most patients were >40 years old at the time of presentation with a median age of 63 years old, with almost equal ethnic distribution per US population data. The most common primary tumor site was the small intestine (33.6%). The metastatic spread was localized in 34.8%, regional in 27.8%, and distant in 37.3% of cases, and the liver was the most common site of metastasis (19.9%) in known cases of metastases. Most NEC patients underwent surgery, presenting the highest 5-year overall survival of 73.2% with a 95% confidence interval (CI) (95% CI 72.0–74.4%), while chemotherapy alone had the lowest 5-year survival of 8.0% (95% CI 6.4–10.0%). Compared to men, women had a superior 5-year survival rate of 59.0% (95% CI 57.6–60.5%). On multivariate analysis, age > 65 (HR 2.49, 95% CI 2.36–2.54%, p ≤ 0.001), distant metastasis (HR 2.57, 95% CI 2.52–2.62%, p ≤ 0.001), tumor size > 4 mm (HR 1.98, 95%, CI 1.70–2.31%, p ≤ 0.001), esophageal (HR 1.49, 95% CI 0.86–2.58%, p ≤ 0.001), transverse colon (HR 1.95, 95% CI 1.15–3.33%, p ≤ 0.01), descending colon (HR 2.12, 95% CI 1.12, 3.97%, p = 0.02) anorectal sites, and liver or lung metastases were associated with worse survival. Surgical intervention and tumors located in the small intestine or appendix showed a better prognosis. Conclusion: GI-NECs are a group of rare malignancies associated with a poor prognosis. Therefore, epidemiological studies analyzing national databases may be the best alternative to have a more comprehensive understanding of this condition, assess the impact of current practices, and generate prognosis tools.

Introduction Pancreatic ductal adenocarcinoma (PDAC) is a highly aggressive malignancy about 50% of PDAC are metastatic at presentation. In this study, we evaluated PDAC demographics, annual trend analysis, racial disparities, survival rate, and the role of different treatment modalities in localized and metastatic disease. Methods A total of 144,824 cases of PDAC were obtained from the SEER database from 2000 to 2018. Results The median age was 69 years, with a slightly higher incidence in males (52%) and 80% of all cases were white. Among cases with available data, 43% were grade III tumors and 57% were metastatic. The most common site of metastasis was the liver (15.7%). The annual incidence has increased steadily from 2000 to 2018. The overall observed (OS) 5-year survival rate was 4.4% (95% CI 4.3–4.6%), and 5 years cause-specific survival (CSS) was 5% (95% CI 5.1–5.4%). The 5-year survival with multimodal therapy (chemotherapy, surgery, and radiation) was 22% (95% CI 20.5–22.8%). 5-year CSS for the blacks was lower at 4.7% (95% CI 4.2–5.1%) compared to the whites at 5.3% (95% CI 5.1–5.4%). Multivariate analysis found male gender and black race associated with worse prognosis. Kaplan–Meier survival analysis found multimodal therapy to have the best outcomes in all three stages. Conclusion PDAC is an aggressive malignancy with male gender and black race are associated with a poor prognosis. Surgery with chemoradiation was associated with the best overall survival. With steadily increasing rates of PDAC, improved treatment modalities are paramount to improving survival in these patients.

Background Large cell neuroendocrine carcinoma (LCNEC) of the lung is a rare, aggressive cancer most commonly found in the lungs but not exclusively, with a worse prognosis than non-small cell lung carcinomas. Currently, LCNEC patients are treated using small cell and non-small cell protocols. This study aims to use the SEER database to identify demographic, clinical, pathological, and therapeutic factors affecting the prognosis and survival of patients with LCNEC of the lung. Methods Demographic, clinical, and management data of patients with lung LCNEC were extracted from the SEER database for the period 2000–2018. Results In the USA, LCNEC has a higher incidence in elderly white men: M:F ratio = 1.2:1, Caucasian: 83.3%, mean age: 67 ± 10.2 years. The most common treatment modality was chemotherapy only: 29.2%, followed by surgery: 21.5% (but in this group the statuses of chemotherapy were unknown), and combination surgery/chemotherapy: 8.8%. The overall and cause-specific 5-year survival was 17.5% (95% CI 16.3–18.8) and 21.9% (95% CI 20.5–23.4), respectively. By treatment, the best 5-year survival was for surgery alone (48%), followed by multimodality therapy (chemo + surgery + radiation) at 35% (95% CI 27–43). Age > 60 years, male gender, size > 7 cm, and nodal and liver metastasis were independent risk factors associated with increased mortality. Conclusion Lung LCNEC is an aggressive neoplasm most common in older white males that presents at an advanced stage despite small primary tumors. Most patients die within 2 years. The best predictor of survival is surgery with chemotherapy. Given its dismal prognosis, new treatment guidelines are needed for this aggressive cancer.

Background: Salivary gland neoplasms are uncommon in both pediatric and adult populations. Mucoepidermoid carcinoma (MEC) is one of the most common salivary gland tumors and usually presents with atypical clinical features. This study sought to evaluate the demographic and clinical factors affecting outcomes in adults and pediatric populations with MEC that could be used to risk stratification for treatment selection and clinical trial enrollment. Methods: Data on 4507 MEC patients were extracted from the Surveillance Epidemiology and End Result (SEER) database (2000–2018). Patients aged ≤ 18 years were classified into the pediatric population, and those older than 18 years were placed in the adult group. Kaplan–Meier survival curves were created to analyze survival probabilities for various independent factors. Results: The pediatric population comprised 3.7% of the entire cohort, with a predominance of females (51.5%), while the adult population constituted 96.3% of the cohort, with a predominance of female patients (52.2%). Caucasians were the predominant race overall (75.3%), while more African Americans were seen in the pediatric group. In tumor size of p < 0.05). Surgical resection was the most common treatment option (53.9%), making up 63.6% of the pediatric and 53.5% of the adult groups. A combination of surgical resection and radiation was used in 29.8% of the entire cohort while a combination of surgical resection, radiation, and chemotherapy made up only 3.2%. The pediatric group had a lower overall mortality rate (5.5%) than the adult group (28.6%). Females had a higher 5-year survival rate in comparison to males (86.5%, and 73.7%, respectively). Surgical resection led to a more prolonged overall survival and 5-year cancer-specific survival (98.4% (C.I, 93.7–99.6) in the pediatric group and 88.8% (C.I, 87.5–90.0) in the adult group), respectively. Metastasis to the lung, bone, brain, and/or liver was found to have significantly lower survival rates. Multivariate analysis demonstrated that adults (hazard ratio [HR] = 7.4), Asian or Pacific Islander (HR = 0.5), male (HR = 0.8), poorly differentiated histology (HR = 3.8), undifferentiated histology (HR = 4.5), regional spread (HR = 2.1), and distant spread (HR = 3.2) were associated with increased mortality (p < 0.05). Conclusions: Mucoepidermoid carcinoma of the salivary glands primarily affects Whites and is more aggressive in adults than in the pediatric population. Even with surgical resection, the overall survival is poor in the adult population as compared to its pediatric counterparts. Advanced age, larger tumor size, male sex, and lymph node invasion are associated with increased mortality.

Programmed death-ligand (PD-L) 1 and 2 are ligands of programmed cell death 1 (PD-1) receptor. They are members of the B7/CD28 ligand-receptor family and the most investigated inhibitory immune checkpoints at present. PD-L1 is the main effector in PD-1-reliant immunosuppression, as the PD-1/PD-L pathway is a key regulator for T-cell activation. Activation of T-cells warrants the upregulation of PD-1 and production of cytokines which also upregulate PD-L1 expression, creating a positive feedback mechanism that has an important role in the prevention of tissue destruction and development of autoimmunity. In the context of inadequate immune response, the prolonged antigen stimulation leads to chronic PD-1 upregulation and T-cell exhaustion. In lung cancer patients, PD-L1 expression levels have been of special interest since patients with non-small cell lung cancer (NSCLC) demonstrate higher levels of expression and tend to respond more favorably to the evolving PD-1 and PD-L1 inhibitors. The Food and Drug Administration (FDA) has approved the PD-1 inhibitor, pembrolizumab, alone as front-line single-agent therapy instead of chemotherapy in patients with NSCLC and PD-L1 ≥1% expression and chemoimmunotherapy regimens are available for lower stage disease. The National Comprehensive Cancer Network (NCCN) guidelines also delineate treatment by low and high expression of PD-L1 in NSCLC. Thus, studying PD-L1 overexpression levels in the different histological subtypes of lung cancer can affect our approach to treating these patients. There is an evolving role of immunotherapy in the other sub-types of lung cancer, especially small cell lung cancer (SCLC). In addition, within the NSCLC category, squamous cell carcinomas and non-G12C KRAS mutant NSCLC have no specific targetable therapies to date. Therefore, assessment of the PD-L1 expression level among these subtypes of lung cancer is required, since lung cancer is one of the few malignances wherein PD-L1 expression levels is so crucial in determining the role of immunotherapy. In this study, we compared PD-L1 expression in lung cancer according to the histological subtype of the tumor.