Single-Institution Experience with Irreversible Electroporation for T4 Pancreatic Cancer: First 50 Patients.

BACKGROUND: Irreversible electroporation (IRE) for treatment of locally advanced pancreatic tumors is garnering increasing attention. This study was conducted to determine perioperative morbidity and mortality for locally advanced pancreatic cancer. METHODS: Prospective data of 50 consecutive patients receiving IRE for T4 lesions at a single tertiary center were analyzed. The primary end point was Clavien-Dindo complications at 90 days, and the secondary outcomes were survival and recurrence. RESULTS: A total of 50 patients underwent 53 IRE procedures for primary treatment (n = 29) or margin extension (n = 24), and 47 patients had adenocarcinoma. Six patients died within 90 days after the procedure (5 in the primary control group). Mortality occurred a median of 26 days (range, 8-42 days) after the procedure. Five patients in both the margin-extension and primary control groups experienced grade 3 or 4 morbidity (p = 0.739). The incidences of grades 3 to 5 complications did not differ significantly based on the adjustable parameters of IRE, tumor size, or primary treatment versus margin extension. After a median follow-up period of 8.69 months [interquartile range (IQR), 0.26-16.26 months], the median overall survival period for the primary control group was 7.71 months [95 % confidence interval (CI), 6.03-12.0 months) and was not reached in the margin-extension group (p = 0.01, log-rank). CONCLUSIONS: At the authors' center, the mortality rate after IRE was higher than reported in other series, with the majority occurring in the primary control group. Major morbidity trended around upper gastrointestinal bleeding, visceral ulcerations/perforations, and portal vein thromboses. This favors further investigation of the safety and efficacy of IRE.

Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011).

BACKGROUND/AIMS: Primary chordomas, rare cancers arising from the notochord remnants, are extremely rare in the pediatric population. This study examined a large cohort of primary chordoma patients to determine factors impacting prognosis and survival. METHODS: Demographic and clinical data on 1,358 primary chordoma patients (86 pediatric patients ≤19 years of age and 1,272 adult patients ≥20 years of age) were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database (1973-2011). RESULTS: Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite the majority of primary chordomas presenting with locoregional involvement (90.4%), pediatric patients had more distant disease (14.8 vs. 9.2%, p < 0.05). Survival among pediatric patients having surgery only was significantly longer than for adults (22.5 vs. 14.3 years, p < 0.001). Overall survival was longer (17.2 vs. 12.6 years) and overall mortality was lower in pediatric patients (38.4 vs. 49.8%), but cancer-specific mortality was higher (37.2 vs. 28.6%, p < 0.005). CONCLUSIONS: Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite having a higher rate of metastasis, they have prolonged survival compared to adults. Surgical resection significantly improves survival in pediatric primary chordoma patients, and should be considered as first-line therapy in all eligible children.

Reoperative completion pancreatectomy for suspected malignant disease of the pancreas.

BACKGROUND: Completion pancreatectomy (CP) is a reoperative procedure to excise remnant pancreatic tissue after a prior pancreatic resection. In this study, we document our institution's experience with CP for recurrent malignant disease of the pancreas, describing indications for surgery, procedures performed, and patient outcomes. METHODS: We performed a retrospective review of 861 patients from the pancreatic surgery database in the Department of Surgery of Thomas Jefferson University from October 2005 to December 2010 to identify all cases of CP performed for suspected malignant disease. RESULTS: Eleven patients underwent reoperative CP at our institution from 2005 to 2010. The median time interval between the initial operation and CP was 32 mo. A combination of clinical symptoms, elevated tumor markers, and imaging studies were used for diagnosis of recurrent disease. Pancreatic ductal adenocarcinoma was the most common pathology, found in six patients. The postoperative complication rate was 18% and the median postoperative hospital length of stay was 6 d. There were no 30-d readmissions and no perioperative deaths. The 1-y survival rate following CP was 71% with an overall median survival of 17.5 mo. CONCLUSIONS: CP is a safe and effective option for a highly selected group of patients with suspected recurrent malignant disease of the remnant pancreas. Morbidity and mortality rates are within acceptable limits and similar to initial pancreatic resection. Eligibility depends heavily upon the absence of distant metastatic disease, technical factors for resection, and patient performance status.

Clinical considerations in the management of non-alcoholic steatohepatitis cirrhosis pre- and post-transplant: A multi-system challenge.

Non-alcoholic steatohepatitis (NASH) is the most common chronic liver disease worldwide, and the fastest growing indication for liver transplantation in the United States. NASH is now the leading etiology for liver transplantation in women, the second leading indication for men, and the most common cause amongst recipients aged 65 years and older. Patients with end-stage liver disease related to NASH represent a unique and challenging patient population due the high incidence of associated comorbid diseases, including obesity, type 2 diabetes (T2D), and hypertension. These challenges manifest in the pre-liver transplantation period with increased waitlist times and waitlist mortality. Furthermore, these patients carry considerable risk of morbidity and mortality both before after liver transplantation, with high rates of T2D, cardiovascular disease, chronic kidney disease, poor nutrition, and disease recurrence. Successful transplantation for these patients requires identification and management of their comorbidities in the face of liver failure. Multidisciplinary evaluations include a thorough pre-transplant workup with a complete cardiac evaluation, control of diabetes, nutritional support, and even, potentially, consultation with a bariatric surgeon. This article provides a comprehensive review of the conditions and challenges facing patients with NASH cirrhosis undergoing liver transplantation and provides recommendations for evaluation and management to optimize them before liver transplantation to produce successful outcomes.

Determining 30-day readmission risk for heart failure patients: the Readmission After Heart Failure scale.

BACKGROUND: Chronic heart failure (CHF), which affects >5 million Americans, accounts for >1 million hospitalizations annually. As a part of the Hospital Readmission Reduction Program, the Affordable Care Act requires that the Centers for Medicare and Medicaid Services reduce payments to hospitals with excess readmissions. This study sought to develop a scale that reliably predicts readmission rates among patients with CHF. METHODS: The State Inpatient Database (2006-2011) was utilized, and discharge data including demographic and clinical characteristics on 642,448 patients with CHF from California and New York (derivation cohort) and 365,359 patients with CHF from Florida and Washington (validation cohort) were extracted. The Readmission After Heart Failure (RAHF) scale was developed to predict readmission risk. RESULTS: The 30-day readmission rates were 9.42 and 9.17% (derivation and validation cohorts, respectively). Age <65 years, male gender, first income quartile, African American race, race other than African American or Caucasian, Medicare, Medicaid, self-pay/no insurance, drug abuse, renal failure, chronic pulmonary disorder, diabetes, depression, and fluid and electrolyte disorder were associated with higher readmission risk after hospitalization for CHF. The RAHF scale was created and explained the 95% of readmission variability within the validation cohort. The RAHF scale was then used to define the following three levels of risk for readmission: low (RAHF score <12; 7.58% readmission rate), moderate (RAHF score 12-15; 9.78% readmission rate), and high (RAHF score >15; 12.04% readmission rate). The relative risk of readmission was 1.67 for the high-risk group compared with the low-risk group. CONCLUSION: The RAHF scale reliably predicts a patient's 30-day CHF readmission risk based on demographic and clinical factors present upon initial admission. By risk-stratifying patients, using models such as the RAHF scale, strategies tailored to each patient can be implemented to improve patient outcomes and reduce health care costs.

Oligodendrogliomas in pediatric and adult patients: an outcome-based study from the Surveillance, Epidemiology, and End Result database.

INTRODUCTION: Oligodendrogliomas (OGs) account for <20% of all intracranial tumors and 25% of gliomas. Despite improvements in imaging techniques allowing for earlier diagnosis, OG is rare among the pediatric population. This study examines a large cohort of OG patients in an effort to define the demographic, clinical, and pathologic factors associated with clinical and survival outcomes. METHODS: Data on 7,001 OG patients were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database (1973-2013). Pediatric patients were defined as ≤19 years old, and adult patients were defined as age ≥20 years. RESULTS: Among 7,001 OG patients, 6.5% were pediatric (mean age 12 ± 6 years), and 93.5% were adult (mean age 46 ± 15 years). Overall, OGs were more common among males, with a male-to-female ratio of 1.28:1. Overall, OGs were more common among Caucasians (76.9%) and also among the African American (10.8% pediatric vs. 4.0% adult) and Hispanic (12.8% pediatric vs. 11.8% adult). OGs occurred most commonly in the temporal lobe of pediatric patients and the frontal lobes of adults. Surgical resection was the primary treatment modality for both pediatric and adult populations (70.6% and 40.5%), followed by combined surgery and radiation (19.7% and 41.2%). Surgical resection was associated with significantly improved survival in both groups. Pediatric patients had a lower overall mortality (19.8% vs. 48.5%) and lower cancer-specific mortality (17.6% vs. 36.8%). CONCLUSION: OGs most often present in Caucasian males in their fifth decade of life with tumors >4 cm in size. Children typically present with tumors in the temporal lobe, while adults present with tumors in the frontal lobe. Surgical resection confers a survival advantage among all patients, especially pediatric patients. Overall survival (OS) and cancer-specific survival are higher in the pediatric population. Further studies on novel treatment techniques, including bevacizumab and immunotherapy approaches, are required.

Gallbladder Carcinoma in the United States: A Population Based Clinical Outcomes Study Involving 22,343 Patients from the Surveillance, Epidemiology, and End Result Database (1973-2013).

INTRODUCTION: Gallbladder carcinoma (GBC) is the most common malignancy of the biliary tract and the third most common gastrointestinal tract malignancy. This study examines a large cohort of GBC patients in the United States in an effort to define demographics, clinical, and pathologic features impacting clinical outcomes. METHODS: Demographic and clinical data on 22,343 GBC patients was abstracted from the SEER database (1973-2013). RESULTS: GBC was presented most often among Caucasian (63.9%) females (70.7%) as poorly or moderately differentiated (42.5% and 38.2%) tumors, with lymph node involvement (88.2%). Surgery alone was the most common treatment modality for GBC patients (55.0%). Combination surgery and radiation (10.6%) achieved significantly longer survival rates compared to surgery alone (4.0 ± 0.2 versus 3.7 ± 0.1 years, p = 0.004). Overall mortality was 87.0% and cancer-specific mortality was 75.4%. CONCLUSIONS: GBC is an uncommon malignancy that presents most often among females in their 8th decade of life, with over a third of cases presenting with distant metastasis. The incidence of GBC has doubled in the last decade concurrent with increases in cholecystectomy rates attributable in part to improved histopathological detection, as well as laparoscopic advances and enhanced endoscopic techniques. Surgical resection confers significant survival benefit in GBC patients.

Ocular melanoma-when you have seen one, you have not seen them all: a clinical outcome study from the Surveillance, Epidemiology and End Results (SEER) database (1973-2012).

BACKGROUND: Ocular melanoma (OM) comprises <5% of all melanomas. Uveal melanoma (UM) is the most common subtype of OM, while conjunctival melanoma (CM) is rare and differs significantly from UM. The purpose of this study is to evaluate a large cohort of OM patients to differentiate demographic, pathologic, and clinical factors between these two neoplasms, which may affect treatment and outcomes. METHODS: The Surveillance, Epidemiology, and End Results database (1973-2012) was used to extract demographic and clinical data on 8,165 OM patients (92.1% UM and 7.9% CM). RESULTS: Both CM and UM were most prevalent among Caucasian males in the seventh decade of life. UM patients presented more often with localized disease (90.9% vs 81.2, P<0.01). Surgery (42.8%), radiation (43.0%), or combined surgery and radiation (7.0%) were used in the treatment of UM, while CM was treated almost exclusively with surgery (88.7%). Mean overall survival was longer (15.4 vs 14.6 years; P<0.01) and mortality rates were lower in patients (38.8% vs 46.1%; P<0.01) with CM. CONCLUSION: Despite presenting with more advanced disease than UM, CM is associated with an increased overall survival. Surgery is the primary therapy for CM, whereas radiotherapy is the primary therapy for UM and is associated with prolonged survival.

Malignant Melanoma in African-Americans: A Population-Based Clinical Outcomes Study Involving 1106 African-American Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1988-2011).

Malignant melanoma accounts for 75% of all skin cancer deaths and is potentially curable if identified early. Although melanoma is rare in African-Americans (AA), it is associated with a worse prognosis than in Caucasians. This study examines the demographic, pathologic, and clinical factors impacting AA melanoma outcomes.Data for 1106 AA and 212,721 Caucasian cutaneous melanoma patients were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database (1988-2011). Data were grouped on the basis of histological subtypes: "Superficial Spreading" (SS), "Nodular" (NM), "Lentigo Maligna" (LM), "Acral Lentiginous" (AL), and "Not otherwise specified" (NOS).Cutaneous malignant melanoma occurs most commonly in the sixth and seventh decade of life. Caucasian patients presented most commonly with trunk melanomas (34.5%), while lower extremity melanomas were more common in AAs (56.1%), P < 0.001. AAs presented with deeper tumors, more advanced stage of disease, and higher rates of ulceration and lymph node positivity than Caucasians. Cancer-specific mortality was significantly higher, while 5-year cancer-specific survival was significantly lower among AAs for NM and AL subtypes. Multivariate analysis identified male gender, regional and distant stage, NM and AL subtypes as independently associated with increased mortality among both ethnic groups.AAs present most often with AL melanoma on the lower extremities, and with deeper and more advanced stage lesions. AAs have higher cancer-specific mortality for NM and LM than Caucasians. Melanoma education for AA patients and health care providers is needed to increase disease awareness, facilitate early detection, and promote access to effective treatment.

Acute mental disorders and short and long term morbidity in patients with third degree flame burn: A population-based outcome study of 96,451 patients from the Nationwide Inpatient Sample (NIS) database (2001-2011).

BACKGROUND: Although burn patients with preexisting mood disorders have been shown to have diminished clinical recovery, acute mental disorders (AMD) are often unrecognized despite a link with post-traumatic stress disorder and social maladjustment later on. This study assessed the clinical profile of a large cohort of burn patients who developed AMD compared to those with chronic mental illness (CMI) and those without mental health problems to assess the impact of AMI on burn outcomes. METHODS: Admission data on 96,451 patients with third degree flame burns was abstracted from the Nationwide Inpatient Sample (NIS) Database from 2001 to 2011. AMD was defined as adjustment disorder (ICD-10 codes F43.2-F43.29) and acute stress disorder (F43.0), while CMI was defined as major depressive disorder (F33.0-F33.9) and bipolar disorder (F31.0-F31.9). Data was compared across three subgroups: AMD, CMI, and patients without any mood disorders. Categorical variables were compared using the Chi-square test, and continuous variables were compared using Student t-test and analysis of variance (ANOVA). Multivariate analysis using the "backward Wald" method was performed to calculate odds ratios (OR) and determine independent factors which increased the risk of developing AMD after burn. RESULTS: 979 (1%) burn patients were diagnosed with AMD, compared to 5971 (6.2%) with CMI and 89,501 (92.8%) without mood disorders at the time of the burn. Patients with AMD were significantly younger, predominantly male and Caucasian. Patients with AMD had a higher frequency of multiple third-degree burn sites. AMD patients had a significantly longer length of hospitalization and shorter actuarial survival. More AMD patients lacked social or family support, suffered from alcoholism or illicit drug abuse, and had a history of psychological trauma or self-inflicted injuries compared to other groups. After burn, 4.9% of AMD patients developed burn wound infections, 5.0% had nutritional deficiencies, 1.7% had skin graft failure, 0.7% had acute psychosis, and 3.7% had suicidal ideation, p<0.05. Multivariate analysis identified age 10-39 (OR 4.6), alcoholism (OR 1.4), drug abuse (OR 1.9), psychoses (OR 1.4), and total body surface are (TBSA) 10-39% third degree burns (OR 1.8) as independently associated with developing AMD, p<0.005. CONCLUSION: The development of AMD in burn patients is associated with poor clinical outcomes including longer hospitalization, decreased survival, and increased complication rates. Premorbid psychopathology, substance abuse and age were associated with a higher incidence AMD after burn than was the severity of the burn wound itself. Routine screening for psychological symptoms of AMD should be performed in all burn patients to avoid increased rates of short and long-term morbidity and mortality.

Trends in incidence, survival, and management of uveal melanoma: a population-based study of 7,516 patients from the Surveillance, Epidemiology, and End Results database (1973-2012).

INTRODUCTION: Uveal melanoma (UM) is the most common primary intraocular malignancy, despite comprising <5% of all melanomas. To date, relatively few case series of UM have been published. Moreover, the factors influencing survival remain largely unknown. This study sought to analyze the impact of demographics, histology, clinical presentation, and treatments on the clinical outcomes of UM in a large modern nationwide patient cohort. METHODS: Demographics and clinical data were abstracted on 277,120 histologically confirmed melanoma patients from the Surveillance, Epidemiology, and End Results database between 1973 and 2012. RESULTS: A total of 7,516 cases of UM represented 3.2% of all recorded cases of melanoma. The mean age-adjusted incidence was 5.1 per million (95% CI 4.2-6.1) and was higher in males (5.9, CI =4.4-7.6) compared to females (4.5, CI =3.3-5.8), P<0.001. UM occurred most commonly in the sixth decade of life (61.4±15) and among Caucasians (94.7%). A total of 52.3% of cases were reported in the Western US (35.7% in California). The initial diagnoses in 65.2% of cases were by histopathology, followed by clinical diagnosis (18.8%) and radiographic imaging (16.0%). The percentage of UM cases managed by surgery alone decreased by 69.4% between the 1973-1977 and 2006-2012 time periods, concomitant with a 62% increase in primary radiotherapy, P<0.001. The UM mean overall and cancer-specific 5-year relative survival rates were 79.8%±5.8% and 76%±5.3%, respectively. The mean 5-year cancer-specific survival rate (76%) remained stable during the study period between 1973 and 2012. The mean survival for patients treated with primary radiotherapy was significantly improved compared to those treated with surgery alone (15.4±0.4 vs 13.6±0.3, P<0.001). Multivariate analysis identified male sex (odds ratio [OR] 1.1, CI =1.0-1.3), age >50 years (OR 4.0, CI =3.4-4.6), distant metastases (OR 8.6, CI =4.7-15), and primary surgical treatment (OR 2.6, CI =2.0-3.3) as independently associated with increased mortality, P<0.005. Conversely, patients identified as Hispanic (OR 0.6, CI =0.5-0.8) and patients receiving radiation treatment (OR 0.5, CI =0.4-0.7) were independently associated with reduced mortality, P<0.005. CONCLUSION: UM remains a rare form of melanoma that occurs primarily in Caucasian patients older than 50 years. More than two-thirds of UM patients are curatively treated with primary radiotherapy as opposed to surgery, which has resulted in a significant improvement in both overall survival and cancer-specific survival. Despite this shift in management strategy, the mean 5-year cancer-specific survival rate remained relatively unchanged during the study period. Male sex, older age, distant disease, and primary surgical therapy rather than radiotherapy are associated with an increased risk of mortality.

Pediatric Burns: A Single Institution Retrospective Review of Incidence, Etiology, and Outcomes in 2273 Burn Patients (1995-2013).

Unintentional burn injury is the third most common cause of death in the U.S. for children age 5 to 9, and accounts for major morbidity in the pediatric population. Pediatric burn admission data from U.S. institutions has not been reported recently. This study assesses all pediatric burn admissions to a State wide Certified Burn Treatment Center to evaluate trends in demographics, burn incidence, and cause across different age groups. Demographic and clinical data were collected on 2273 pediatric burn patients during an 18-year period (1995-2013). Pediatric patients were stratified by age into "age 0 to 6," "age 7 to 12," and "age 13 to 18." Data were obtained from National Trauma Registry of the American College of Surgeons and analyzed using standard statistical methodology. A total of 2273 burn patients under age 18 were treated between 1995 and 2013. A total of 1663 (73.2%) patients were ages 0 to 6, 294 (12.9%) were 7 to 12, and 316 (13.9%) were age 13 to 18. A total of 1400 (61.6%) were male and 873 (38.4%) were female (male:female ratio of 1.6:1). Caucasians had the highest burn incidence across all age groups (40.9%), followed by African-Americans (33.6%), P < .001. Caucasian teenagers formed 62.1% of patients age 13-18, P < .001. A total of 66.3% of all pediatric burns occurred at home, P < .001. Mean TBSA burned was 8.9%, with lower extremity being the most common site (38.5%). Scald burns constituted the majority of cases (71.1%, n = 1617), with 53% attributable to hot liquids related to cooking, including coffee or tea, P < .001. In the teenage group, flame burns were the dominant cause (53.8%). Overall mean length of stay was 10.5 ± 10.8 days for all patients, and15.5 ± 12 for those admitted to the intensive care unit, P < .005. One hundred (4.4%) patients required ventilator support (P = .02), and average duration of mechanical ventilation was 11.9 ± 14.5 days. Skin grafting was performed for 520 (22.9%) patients, P < .001. Overall mortality was 0.9% (n = 20), with mean TBSA involved of 61.5%. The majority of pediatric burn injuries are scald burns that occur at home and primarily affect the lower extremities in Caucasian and African-American males. Among Caucasian teenagers flame burns predominate. Mean length of stay was 10 days, 23% of patients required skin grafting surgery, and mortality was 0.9%. The results of this study highlight the need for primary prevention programs focusing on avoiding home scald injuries in the very young, as well as fire safety training for teenagers.

Atypical teratoid rhabdoid tumors: a population-based clinical outcomes study involving 174 patients from the Surveillance, Epidemiology, and End Results database (1973-2010).

INTRODUCTION: Atypical teratoid rhabdoid tumors (ATRTs) are rare, highly malignant embryonal tumors of the central nervous system (CNS) accounting for 20% of CNS tumors in children under the age of 3. This study examines a large cohort of ATRT patients to determine demographic, clinical, and pathologic factors which impact prognosis and survival. METHODS: Demographic and clinical data were abstracted on 174 ATRT patients (171 pediatric patients age <20 and 3 adult patients age ≥20) from the Surveillance, Epidemiology, and End Results database (1973-2010). Standard statistical methodology was used. RESULTS: A total of 174 ATRT cases (mean age of 2.84 years) were identified. ATRT had a higher incidence in males (56.3%), Caucasians (59.1%), and children <3 years of age (80.5%), P<0.001. The most common primary sites were the cerebellum (17.8%), ventricles (16.1%), and frontal lobe (12.6%). Mean overall survival was 3.2±0.4 years, while overall and cancer-specific mortality were 63.2% and 56.3%, respectively, P=0.005. Most ATRT cases were treated with surgery alone (58.0%), followed by a combination of surgery and radiation (34.3%), no treatment (6.5%), and radiation alone (1.2%). The use of combination therapy has increased significantly (16.1%) since 2005 (P<0.001), while primary surgical resection and radiation therapy rates remain relatively unchanged. The longest survival was observed among ATRT patients receiving combination therapy (5.9±0.7 years), followed by radiation alone (2.8±1.2 years), and surgery alone (1.9±0.4 years), P<0.001. Multivariable analysis identified only distant metastases (OR =4.6) as independently associated with increased mortality, whereas combination therapy (OR =0.4) was associated with reduced mortality, P<0.005. CONCLUSION: ATRT is a rare and highly aggressive embryonal malignancy of the CNS that presents more often as locoregional tumors >4 cm in male Caucasian children of age <3 years, involving the cerebellum, ventricles, or frontal lobe. Combination therapy significantly improves survival, and its use has been increasing since 2005.

Hepatocellular Carcinoma in the Pediatric Population: A Population Based Clinical Outcomes Study Involving 257 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011).

Introduction. Hepatocellular carcinoma (HCC) is a rare pediatric cancer accounting for 0.5% of all pediatric malignancies. This study examines a large cohort of HCC patients in an effort to define the factors impacting clinical outcomes in pediatric HCC patients compared to adults. Methods. Demographic and clinical data on 63,771 HCC patients (257 pediatric patients ≤ 19 and 63,514 adult patients age ≥ 20) were abstracted from the SEER database (1973-2011). Results. HCC was more common among males (59.5% pediatric and 75.1% adults) and Caucasians (50.4% and 50.5%), p < 0.05. Children more often presented with fibrolamellar variant HCC (24.1% versus 0.3%, p = 0.71) and advanced HCC, including distant disease (33.1% versus 20.8%, p < 0.001), and tumors > 4 cm in size (79.6% versus 62.0%, p = 0.02). Pediatric HCC patients undergoing surgery (13.107 versus 8.324 years, p < 0.001) had longer survival than adult HCC patients. Overall mortality was lower (65.8% versus 82.0%, p < 0.001) in the pediatric HCC group. Conclusion. HCC is a rare pediatric malignancy that presents most often as an advanced tumor, >4 cm in Caucasian males. Children with HCC achieve significantly longer mean overall survival compared to adults with HCC, primarily attributable to the more favorable fibrolamellar histologic variant, and more aggressive surgical intervention, which significantly improves survival.