The Florida Pancreas Collaborative Next-Generation Biobank: Infrastructure to Reduce Disparities and Improve Survival for a Diverse Cohort of Patients with Pancreatic Cancer.

Background: Well-annotated, high-quality biorepositories provide a valuable platform to support translational research. However, most biorepositories have poor representation of minority groups, limiting the ability to address health disparities. Methods: We describe the establishment of the Florida Pancreas Collaborative (FPC), the first state-wide prospective cohort study and biorepository designed to address the higher burden of pancreatic cancer (PaCa) in African Americans (AA) compared to Non-Hispanic Whites (NHW) and Hispanic/Latinx (H/L). We provide an overview of stakeholders; study eligibility and design; recruitment strategies; standard operating procedures to collect, process, store, and transfer biospecimens, medical images, and data; our cloud-based data management platform; and progress regarding recruitment and biobanking. Results: The FPC consists of multidisciplinary teams from fifteen Florida medical institutions. From March 2019 through August 2020, 350 patients were assessed for eligibility, 323 met inclusion/exclusion criteria, and 305 (94%) enrolled, including 228 NHW, 30 AA, and 47 H/L, with 94%, 100%, and 94% participation rates, respectively. A high percentage of participants have donated blood (87%), pancreatic tumor tissue (41%), computed tomography scans (76%), and questionnaires (62%). Conclusions: This biorepository addresses a critical gap in PaCa research and has potential to advance translational studies intended to minimize disparities and reduce PaCa-related morbidity and mortality.

Robotic vascular resections during Whipple procedure.

Indications for resection of pancreatic cancers have evolved to include selected patients with involvement of peri-pancreatic vascular structures. Open Whipple procedures have been the standard approach for patients requiring reconstruction of the portal vein (PV) or superior mesenteric vein (SMV). Recently, high-volume centers are performing minimally invasive Whipple procedures with portovenous resections. Our institution has performed seventy robotic Whipple procedures with concomitant vascular resections. This report outlines our technique.

Long-term effectiveness of localization studies and intraoperative parathormone monitoring in patients undergoing reoperative parathyroidectomy for persistent or recurrent hyperparathyroidism.

BACKGROUND: Reoperative parathyroidectomy (RPTX) for persistent or recurrent hyperparathyroidism is associated with a high rate of operative failure. The long-term effectiveness of RPTX using localization studies and intraoperative parathormone monitoring (IPM) was examined. METHODS: Retrospective analysis of prospectively collected data from patients undergoing targeted RPTX with IPM for persistent or recurrent hyperparathyroidism was performed. Persistent hyperparathyroidism was defined as elevated calcium and parathormone (PTH) levels above normal range less than 6 months after parathyroidectomy. Recurrent hyperparathyroidism was defined as elevated calcium and PTH levels greater than 6 months after successful parathyroidectomy. Sensitivity and positive predictive value (PPV) for sestamibi, surgeon-performed ultrasound, intraoperative PTH dynamics, and surgical outcomes were evaluated. RESULTS: Of the 1,064 patients, 69 patients underwent 72 RPTXs with localizing studies and IPM. Sestamibi (n = 69) had a sensitivity of 74% and a PPV of 83%, whereas surgeon-performed ultrasound (n = 38) had a sensitivity of 55% and a PPV of 76%. IPM had a sensitivity of 100% and a PPV of 98%. An intraoperative PTH drop greater than or equal to 50% was predictive of operative success (P < .01). Overall, operative success and recurrence were 94% and 1.4%, with a mean patient follow-up of 59 ± 12.8 months. CONCLUSION: RPTX can be performed in a targeted approach using preoperative localization studies and IPM, leading to a low rate of complications and a high rate of long-term operative success.

Superficial Spreading Melanomas in Children: An Analysis of Outcomes Using the Surveillance, Epidemiology, and End Results (SEER) Database.

PURPOSE: This study aims to review the incidence and outcomes for children with superficial spreading melanomas (SSM) using population-based data. METHODS: The Surveillance, Epidemiology, and End Results database (1974-2009) was analyzed for all patients less than 20 years of age diagnosed with SSM. RESULTS: Overall, 724 patients were identified. The annual age-adjusted incidence was 1.78 cases per 1,000,000 in 2009. Most patients were adolescents (N = 620, 85.6%), girls (N = 478, 66.0%), and Caucasian (N = 670, 92.5%). Most tumors were located on the trunk (N = 308, 42.5%) or lower extremities (N = 160, 22.1%). Overall and disease-specific survivals were 95.6% and 97.6%, respectively. Lymph node positivity decreased disease-specific survival among patients undergoing lymph node dissection (86% vs. 100% for negative lymph nodes, P = 0.001). CONCLUSIONS: Incidence of SSM increased in the early part of the study, but has remained relatively stable over the past 2 decades. Girls are at higher risk of development of the disease. Outcomes for children with SSM are very favorable.

Trends in the incidence and treatment of necrotizing soft tissue infections: an analysis of the National Hospital Discharge Survey.

Necrotizing soft tissue infections are a rare but potentially fatal condition of the soft tissues caused by virulent, toxin-producing bacteria. In the United States, there is an estimated annual incidence of 0.04 cases per 1000 annually, but previous estimates of the Centers for Disease Control and Prevention had the incidence at 500 to 1500 cases yearly. Early reports of mortality were variable with rates ranging from 46 to 76% but outcomes have been improving over time. The National Hospital Discharge Survey was analyzed to study current trends in the demographics, incidence, use, and mortality of patients diagnosed with necrotizing soft tissue infections. The authors analyzed the 1999, 2002, and 2007 National Hospital Discharge Survey by using a sampling weighting method. A total of 13,648 cases of necrotizing soft tissue infections were identified in 2007. This represents an increase from 12,153 cases in 2002 and 6612 cases in 1999. In the 9 years from 1999 to 2007 the gross incidence of necrotizing soft tissue infections more than doubled. Hospital stay was essentially unchanged within study years, at 16 days. Mean age increased from approximately 50 years in 1999 to 54 years in 2007. Further, mortality went from 10.45% in 1999 to 9.75% in the 2007 survey. The population-adjusted incidence rate increased 91% in the studied years. Rising use of immunosupression, exponential growth in the incidence of obesity, and type 2 diabetes could be a major contributing factor. The mortality rate is far below the rate in reports published from as early as 20 years ago, and at 9.75% compares with modern case series, but is a more accurate measure of mortality in this condition.

A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States.

BACKGROUND: The aim of this study was to examine national outcomes in newborn patients with esophageal atresia and tracheoesophageal fistula (EA/TEF) in the United Sates. METHODS: Kids' Inpatient Database (KID) is designed to identify, track, and analyze national outcomes for hospitalized children in the United States. Inpatient admissions for pediatric patients with EA/TEF for kids' Inpatient Database years 2000, 2003, 2006, and 2009 were analyzed. Patient demographics, socioeconomic measures, disposition, survival and surgical procedures performed were analyzed using standard statistical methods. RESULTS: A total of 4168 cases were identified with diagnosis of EA/TEF. The overall in-hospital mortality was 9%. Univariate analysis revealed lower survival in patients with associated acute respiratory distress syndrome, ventricular septal defect (VSD), birth weight (BW) < 1500 g, gestational age (GA), time of operation within 24 h of admission, coexisting renal anomaly, imperforate anus, African American race, and lowest economic status. Multivariate logistic regression identified BW < 1500 g (odds ratio [OR] = 4.5, P < 0.001), operation within 24 h (OR = 6.9, P < 0.001), GA <28 wk (OR = 2.2, P < 0.030), and presence of VSD (OR = 3.8, P < 0.001) as independent predictors of in-hospital mortality. Children's general hospital and children's unit in a general hospital were found to have a lower mortality rate compared with not identified as a children's hospital after excluding immediate transfers (P = 0.008). CONCLUSIONS: BW < 1500 g, operation within 24 h, GA < 28 wk, and presence of VSD are the factors that predict higher mortality in EA/TEF population. Despite dealing with more complicated cases, children's general hospital and children's unit in a general hospital were able to achieve a lower mortality rate than not identified as a children's hospital.

Surgical treatment of patients with mildly elevated parathormone and calcium levels.

BACKGROUND: Patients with mildly elevated parathormone (PTH) and calcium levels consistent for primary hyperparathyroidism (pHPT) may present with more underlying multiglandular disease (MGD) and higher operative failure and recurrence rates than those with conventional, or "classic" pHPT. This study compared the clinical characteristics and surgical outcomes of patients with biochemically mild versus conventional pHPT. METHODS: A series of 707 consecutive patients underwent initial targeted parathyroidectomy with intraoperative parathormone monitoring (IPM) at a single institution. Biochemically mild (BM) pHPT was defined as PTH > 65 and <100 pg/ml with serum calcium >10.4 and <11 mg/dl. Conventional pHPT was defined as calcium ≥11 mg/dl and PTH ≥ 100 pg/ml. Prospectively collected data for all patients, including operative indication, preoperative laboratory values, imaging, IPM dynamics, and postoperative laboratory values were retrospectively reviewed. Additional assessments included presence of MGD, bilateral neck exploration (BNE), single-gland volume, and operative failure or success, and recurrence. RESULTS: Of 60 patients with BM-pHPT, 46 reported preoperative bone pain, kidney stones, fatigue, and/or mental disturbances. The remaining 14 BM-pHPT patients underwent parathyroidectomy based on published asymptomatic guidelines. Patients with BM-pHPT had significantly more kidney stones, MGD, and BNE. Average single-gland volume and postoperative PTH levels were significantly lower in BM-pHPT patients. There were no significant differences between groups regarding preoperative localization accuracy, IPM dynamics, or operative success/failure, recurrence rates. CONCLUSIONS: BM-pHPT patients had more MGD requiring BNE but achieved operative success rates similar to those of patients with conventional disease. IPM successfully identifies MGD in BM-pHPT patients, who should be counseled regarding more extensive operations than limited parathyroidectomy.

A review of 218 pediatric cases of hepatocellular carcinoma.

PURPOSE: This study evaluates the incidence trends and clinical outcomes of children with hepatocellular carcinoma (HCC) and assesses factors predictive of patient survival. METHODS: The Surveillance, Epidemiology, and End Results registry was queried from 1973 to 2009 for all patients between ages 0 and 19 with primary HCC. Demographics, tumor histology, surgical intervention, and patient survival were collected. RESULTS: Overall, 218 patients were identified. The annual age-adjusted incidence was 0.05 cases per 100,000 in 2009. Fibrolamellar subtype tumors were exclusive to children >5years old and exhibited greater survival compared to non-fibrolamellar subtype (57% vs. 28%, respectively, p=0.002). Tumor extirpation for patients with resectable disease significantly improved overall survival at 5years compared to no surgery (60% vs. 0%, respectively, p<0.0001). Overall 5-, 10- and 20-year survival for the entire cohort was 24%, 23%, and 8%, respectively. Independent prognostic factors of lower mortality according to multivariate analysis were surgical resection (hazard ratio (HR)=0.18), non-Hispanic ethnicity (HR=0.52), and local disease at presentation (HR=0.46). CONCLUSION: Over the past four decades, the incidence of HCC has remained relatively stable. Children of Hispanic ethnicity have high mortality rates. However, HCC resection for curative intent significantly improves outcomes.

Revision decompression and collagen nerve wrap for recurrent and persistent compression neuropathies of the upper extremity.

BACKGROUND: Recurrent or persistent compression neuropathies of the upper extremity, including carpal and cubital tunnel syndrome, present a difficult treatment challenge to the hand or peripheral nerve surgeon. Collagen conduits have been used successfully for decades in nerve injury repair, but have not been studied in the treatment of compression neuropathy. METHODS: Patients with recurrent or persistent compression neuropathies treated with a repeat decompression and collagen wrap from a 5-year period were retrieved from the Computerized Patient Record System database and 15 patient records were identified. A systematic review was performed for all articles from 1946 to 2012 on secondary carpal and cubital tunnel syndrome. RESULTS: The mean age of the 15 patients treated was 63.3 years and ranged from 35 to 86 years. The patients with revision carpal tunnel decompression had an 89% subjective response rate, whereas those with revision cubital tunnel decompression had an 83% resolution or improvement of symptoms. Visual analog scale decreased from a preoperative mean 2.47 to 0.47 postoperatively and the mean number of opiate medications decreased from 0.67 to 0.40. We identified 32 papers using various treatment strategies for recurrent carpal tunnel syndrome with success rates ranging from 53% to 100%. We identified 18 papers on recurrent cubital tunnel syndrome, with success rates ranging from 33% to 100%, with a weighted success of 78.1% overall but 71.7% in the submuscular transposition group. CONCLUSIONS: Here we report on the novel technique of using a collagen matrix wrap in recurrent compression neuropathies with good success. The collagen wrap allows nerve gliding, protection from perineural scar formation, and a favorable microenvironment. Submuscular transposition seems to be no better than other methods of decompression for recurrent cubital tunnel syndrome in contrary to traditional teaching.

Merkel cell carcinoma of the hand and upper extremity: current trends and outcomes.

Merkel cell carcinomas represent an uncommon yet aggressive skin cancer. We sought to identify changes in incidence and predictors of outcomes and survival of patients with Merkel cell carcinomas of the hand and upper extremity. The Surveillance, Epidemiology and End Results database was used to identify all patients with Merkel cell carcinomas of the skin located specifically in the hand and upper extremity. Overall, 5211 cases were identified in the period from 1986 through 2009. The age-adjusted incidence of Merkel cell carcinoma of the hand and upper extremity increased from 0.02 cases per 100,000 in 1986 to 0.14 cases per 100,000 in 2009. The mean age of these patients was 75 years and positive regional lymph nodes were identified in 33%. Rate of metastasis was 4.1%. Overall survival for the study cohort was 49%. Multivariate analysis identified tumor size >5 cm, positive regional lymph nodes and metastasis at diagnosis as independent predictors of mortality. The incidence of Merkel cell carcinomas has increased substantially over the study period. Overall survival for Merkel cell carcinomas of the body and hand and upper extremity is related to tumor size and extent of disease at time of clinical presentation. Merkel cell carcinomas of the hand and upper extremity tend to be diagnosed at an earlier stage with lower rates of regional and systemic spread, and subsequently have a higher overall survival rate.

Malignant tumors of the parotid gland in children: incidence and outcomes.

BACKGROUND: Population-based analysis of the incidence, demographics, and management outcomes in children with malignant tumors of the parotid gland. METHODS: Surveillance, Epidemiology, and End Results database (1973-2009) was researched for all patients younger than 20 years. RESULTS: Overall, 284 patients were identified. Annual incidence of these tumors was 1.43 cases per million. The highest incidence occurred in girls (0.86/1,000,000), black children (0.849/1,000,000), and adolescents (1.56/1,000,000). Median age at diagnosis was 13.5 years. Most patients were 10 years or older (n = 256, 90%). Most patients presented with local disease (n = 207, 76%). Only 3% had metastasis at time of diagnosis. Most tumors were mucoepidermoid carcinomas (n = 139, 49%) or acinar cell carcinomas (n = 113, 40%). There were no differences in survival between mucoepidermoid and acinar cell carcinomas (96% vs 98% respectively, P = 0.317). Overall mortality was 4.6% over the study period. Overall survival was 96% at 5 years, 95% at 10 years, and 83% at 20 years. Adolescents had significantly higher mortality rates (7.1% vs 1.6% for children <15 years of age, P = 0.23). Multivariate analysis identified the use of adjuvant radiation therapy (hazard ratio, 6.01; 95% confidence interval, 1.15-31.45; P = 0.034) as the only independent predictor of poor outcome. CONCLUSIONS: Malignant parotid gland tumors are most common in adolescents, and this subgroup has worse outcomes. The role of radiotherapy remains controversial.

Efficacy of localization studies and intraoperative parathormone monitoring in the surgical management of hyperfunctioning ectopic parathyroid glands.

BACKGROUND: Hyperfunctioning ectopic glands remain an operative challenge in patients with sporadic primary hyperparathyroidism. This study examines the incidence of ectopic glands and the utility of sestamibi scans (MIBI), surgeon-performed ultrasonography, and intraoperative parathormone monitoring in such patients undergoing parathyroidectomy. METHODS: We conducted a retrospective analysis of prospectively collected data from patients who underwent parathyroidectomy from 1980 to 2011 for sporadic primary hyperparathyroidism at a single institution. Demographics, localizing imaging studies, intraoperative parathyroid monitoring dynamics, and surgical outcome for patients with hyperfunctioning ectopic parathyroid glands were studied. RESULTS: Among 1,195 patients who underwent parathyroidectomy for sporadic primary hyperparathyroidism, 120 patients (10%) had hyperfunctioning ectopic glands, which were localized to the neck (n = 66) and mediastinum (n = 54). MIBI had a sensitivity of 85%, specificity of 97%, and positive predictive value (PPV) of 91% for ectopic glands in the neck, whereas in the mediastinum there was a sensitivity of 88%, specificity of 95%, and PPV of 86%. Surgeon-performed ultrasonography had a sensitivity of 81%, specificity of 98%, and PPV of 95% for neck ectopic glands. The overall accuracy of surgeon-performed ultrasonography, MIBI, and intraoperative parathyroid monitoring in the neck or mediastinum was 93%. Overall, operative success was 93% with a multiglandular disease rate of 5%. CONCLUSION: A high operative success rate for hyperfunctioning ectopic glands can be achieved using localization studies and intraoperative parathyroid monitoring. Nevertheless, surgeon judgment remains paramount in the operative direction of this patient population.

Trends in the surgical treatment of cubital tunnel syndrome: an analysis of the national survey of ambulatory surgery database.

PURPOSE: To ascertain trends in the surgical treatment of ulnar nerve compression at the elbow within the United States. METHODS: We analyzed the National Survey of Ambulatory Surgery to study trends in the treatment of cubital tunnel syndrome from 1994 to 2006. The National Survey of Ambulatory Surgery provides a comprehensive overview of ambulatory surgical procedures performed in the United States. Patients identified in the database with surgically treated cubital tunnel syndrome were verified by members of our research staff and compiled into these 3 groups: decompression, transposition, and other. The data were then statistically analyzed for trends in treatment, utilization, and demographics. RESULTS: A total of 52,133 surgical procedures were recorded in the National Survey of Ambulatory Surgery for the treatment of ulnar nerve compression in 2006. This represents an increase from 26,283 in 1994 and 35,406 in 1996. In the 11 years from 1996 to 2006, the total surgical procedures on the ulnar nerve increased by 47%. Transposition went from 49% of all cubital tunnel procedures in the 1990s to 38% in 2006. In 2006, women were much more likely to have a simple decompression (70%) than a transposition or other technique. Decompression had a mean surgical time of 48 minutes, and transposition had a mean surgical time of 59 minutes. CONCLUSIONS: The percentage of transpositions used in the treatment of cubital syndrome has decreased to 37% in the last survey. Possible reasons include expanded indications or changing surgical preferences. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic II.

Sex variability of fine-needle aspiration reliability in the diagnosis of malignancy in thyroid nodules ≥4 cm.

BACKGROUND: The effect of sex on fine-needle aspiration (FNA) diagnosis of thyroid cancer remains unknown. This study determines the reliability of FNA when evaluating thyroid nodules ≥4 cm in women and men. METHODS: Prospectively collected data of 1,068 patients who underwent FNA and thyroidectomy at a tertiary medical center were retrospectively reviewed. Data were stratified by sex and thyroid nodule size ≥4 cm. RESULTS: The FNA false-negative rate for thyroid malignancy in women and men was 17% and 0%, respectively. FNA was less predictive of malignancy in women (odd ratio = 31.7; 95% confidence interval, 19.2 to 52.5; P < .0001) compared with men (odds ratio = 51.7; 95% confidence interval, 11.8 to 225.1; P < .0001) with thyroid nodules ≥4 cm. CONCLUSIONS: For the diagnosis of malignancy in large thyroid nodules, FNA may be less reliable in women compared with men. This study advocates using a more aggressive approach that includes surgical resection for definitive diagnosis in women with thyroid nodules ≥4 cm.

A systematic review of the literature on the outcomes of treatment for recurrent and persistent carpal tunnel syndrome.

BACKGROUND: Recurrent and persistent carpal tunnel syndrome is an uncommon but potentially difficult surgical dilemma. Many surgical treatment options have been described in the literature without comparative data on outcome. METHODS: A systematic review on recurrent carpal tunnel syndrome was performed for all articles from 1946 to 2012 in MEDLINE, EMBASE, CENTRAL, and hand-searched reference lists from all identified articles. Twenty-three articles were screened and identified from the time period 1972 to 2012, representing two general treatment groups: decompression with flap interposition and repeated open decompression. A meta-analysis was then performed, generating forest and funnel plots of the data. RESULTS: In total, 294 patients from 14 studies in the flap arm of the meta-analysis had a weighted success rate of 86 percent (95 percent CI, 0.75 to 0.96), and 364 patients from nine studies in the nonflap arm had a weighted 75 percent success rate (95 percent CI, 0.66 to 0.84). Heterogeneity was statistically analyzed and revealed low heterogeneity with the I statistic. Forest plots were created and analyzed between subgroups, and chi-square analysis revealed a highly statistically significant difference (p = 0.001). The odds ratio of success in the nonflap group was 0.50 (95 percent CI, 0.33 to 0.75). CONCLUSIONS: Decompression with the use of vascularized flap coverage appears to have a higher success rate over simple repeated decompression. The relevance of these data is pertinent to all hand surgeons, as they could have an impact on treatment guidelines for this relatively uncommon but problematic condition, but further prospective study is needed. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Analysis of 855 upper extremity fistulas created using a standard protocol: the role of graft extension to achieve functional status.

The Fistula First Breakthrough Initiative (FFBI) has been one of the most important national programs to help achieve considerable improvements in the care of patients on chronic hemodialysis. FFBI has helped place guidelines to push practitioners to reduce the use of tunneled central venous catheters and to increase the rate of arteriovenous fistula use in patients requiring chronic hemodialysis access. However, despite current guidelines, no specific protocols exist for the creation and management of autogenous arteriovenous fistulas and outcomes at most centers are below national benchmarks. In this study, we examine the effectiveness of a standard protocol used at our institution for the creation of autogenous upper extremity fistulas for hemodialysis access in achieving early cannulation and early removal of tunneled dialysis catheters. Our review encompasses 855 consecutive autogenous fistulas created over a 10-year period. Our findings suggest that the use of a standard protocol for creation and management of autogenous fistulas can help increase the rate of functional accesses over national benchmarks. Additionally, extension/conversion of malfunctioning fistulas to grafts appears to be an excellent method to expedite removal of a tunneled dialysis catheter with concomitant preservation of a fistula.

Risk of concomitant malignancy in hyperfunctioning adrenal incidentalomas.

BACKGROUND: Adrenal masses are common incidental findings on radiologic imaging. The association between malignancy and hormonal hyperactivity found in incidentally discovered adrenal tumors, however, remains unclear. METHODS: A retrospective analysis of prospectively collected data from patients who underwent adrenalectomy for incidentally discovered adrenal tumors at a single institution. Outcomes and operative data were compared by univariate analysis. Area under the curve was used to analyze the effect of tumor size in predicting malignancy. RESULTS: There were 49 patients who initially presented with adrenal incidentalomas that underwent adrenalectomy. Most patients were Caucasian women with an average age of 51 ± 14 years. Of this group, 24 patients underwent resection for hyperfunctioning adrenal glands. There were no significant differences in malignancy rates between hyperfunctional and nonfunctional tumors (4.1% vs. 12.0%, P = 0.32). On final histopathology, there were four patients with adrenal malignancies: two adrenocortical carcinomas and two metastatic from renal carcinoma. Only one patient with a hyperfunctioning adrenal tumor had underlying malignancy. Overall, invasion of adjacent structures (P < 0.001), presence of lymphadenopathy (P = 0.02), metastasis (P = 0.03), irregular tumor margins (P = 0.01), heterogeneity (P = 0.05), and tumor size >6 cm (P = 0.04) on radiologic imaging were strongly associated with malignancy in adrenal incidentalomas. CONCLUSIONS: The risk of concomitant malignancy and hormonal hyperactivity in adrenal incidentalomas is very low. Tumor size (>6 cm) and radiographic features remain the most important predictors of adrenal malignancy, regardless of tumor function.

Predictors of survival and incidence of hepatoblastoma in the paediatric population.

OBJECTIVES: This study evaluates current trends in incidence, clinical outcomes and factors predictive of survival in children with hepatoblastoma (HB). METHODS: The Surveillance, Epidemiology and End Results (SEER) database was queried for the period 1973-2009 for all patients aged <20 years with HB. RESULTS: A total of 606 patients were identified. The age-adjusted incidence was 0.13 patients per 100 000 in 2009. An annual percentage change of 2.18% (95% confidence interval (CI) 1.10-3.27; P < 0.05) was seen over the study period. Overall survival rates at 5, 10 and 20 years were 63%, 61% and 59%, respectively. Ten-year survival rates significantly improved in patients with resectable disease who underwent operative treatment in comparison with those with non-resectable HB (86% versus 39%; P < 0.0001). Multivariate analysis showed surgical treatment (hazard ratio (HR) = 0.23, 95% CI 0.17-0.31; P < 0.0001), Hispanic ethnicity (HR = 0.61, 95% CI 0.43-0.89; P = 0.01), local disease at presentation (HR = 0.43, 95% CI 0.29-0.63; P < 0.0001) and age < 5 years (HR = 0.63, 95% CI 0.41-0.95; P < 0.03) to be independent prognostic factors of survival. CONCLUSIONS: The incidence of paediatric HB has increased over time. Hepatoblastoma is almost exclusively seen in children aged < 5 years. When HB presents after the age of 5 years, the prognosis is most unfavourable. Tumour extirpation markedly improves survival in paediatric patients with local disease.

An analysis of 73 cases of pediatric malignant tumors of the thymus.

BACKGROUND: Tumors of the thymus are very rare in the pediatric population. This study examines the current trends and outcomes of children with thymus tumors. METHODS: The Surveillance, Epidemiology and End Results (SEER) registry was queried for all patients <20 y of age with primary thymic malignancies from 1973 to 2008. RESULTS: A total of 73 pediatric patients were identified with malignant thymic tumors. The median age at diagnosis was 13 y old. Among the 20 patients that presented with distant disease, 70% died. Conversely, among the 23 patients that presented with locoregional disease, 70% survived. Although the overall mean survival time was 89 ± 116 mo, 45% of patients died over the study period. Patients with Hodgkin lymphomas and germ cell tumors exhibited the highest survival (76% and 60% at 10 y, respectively). Multivariate analysis was used to identify local or regional tumor stage (odds ratio = 4.5, 95% confidence interval = 1.4-14.5) and surgical resection (OR = 3.8, 95% confidence interval = 1.4-10.8) as independent predictors of survival. CONCLUSIONS: Malignant thymomas and lymphomas are the most common histological variants of pediatric thymus tumors, and patients with Hodgkin lymphomas exhibit the highest survival. Surgery is more commonly performed on malignant thymomas and is an independent prognostic indicator of survival.