Juan E. Sola

Pediatric Thyroid Carcinoma: Incidence and Outcomes in 1753 Patients

OBJECTIVE To examine outcomes and predictors of survival for pediatric patients with thyroid carcinoma. METHODS The Surveillance, Epidemiology, and End Results (SEER) registry from 1973 to 2004 was queried for all patients with thyroid carcinoma less than 20 y of age. RESULTS A total of 1753 patients with malignant thyroid neoplasms were identified with an age-adjusted annual incidence of 0.54 cases per 100,000 persons. There has been a significant increase in the annual incidence by 1.1% per y. Female patients outnumbered males 4 to 1. Tumors were classified as papillary (n=1044, 60%), follicular variant of papillary (n=389, 23%), follicular (n=165, 10%), and medullary (n=87, 5%). The majority of patients presented with localized and regional disease. Overall mean survival time (MST) was 30.5 y. The MST for females was 40 y, whereas males survived an average of 20.4 y (P=0.0001). Patients with medullary cancer had significantly shorter mean survival than those with papillary cancer (P=0.006). Surgical treatment significantly improved outcome. Multivariate analysis demonstrated that male gender, nonpapillary histology, distant disease, and no surgery were all independent prognostic factors of worse outcome. For patients with medullary thyroid carcinoma, radiation therapy was also identified as an independent predictor of lower survival. CONCLUSION The incidence of pediatric thyroid cancer is increasing. Females have a higher incidence than males, but enjoy longer survival. Papillary thyroid cancer has overall excellent survival. Male gender, nonpapillary tumor, distant metastases, and nonsurgical treatment all predict worse outcome.

Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients.

OBJECTIVE The purpose of the study was to examine current incidence trends and outcomes for children with pancreatic malignancies. METHODS The Surveillance, Epidemiology, and End Results registry (1973-2004) was examined for pediatric patients with pancreatic malignancies (up to 19 years of age). RESULTS Malignant pancreatic neoplasms were identified in 58 patients. Females outnumbered males 1.9 to 1 (38 vs 20) for an age population-adjusted incidence of 0.021 and 0.015 per 100,000. Overall, 70% (n = 41) of patients were white. Asians had the highest incidence. Tumors were classified as exocrine (n = 31, 53.4%), endocrine (n = 19, 32.8%), or sarcomas (n = 5, 8.6%). Exocrine tumors included pancreatoblastoma (n = 10), solid-cystic tumor (SCT) (n = 10), ductal adenocarcinoma (DA) (n = 7), and acinar cell carcinoma (ACC) (n = 4). All SCTs and 80% of pancreatoblastomas were seen in females, whereas males had a higher incidence of DA 71% (P = .036). Ductal adenocarcinoma, SCT, acinar cell carcinoma, sarcomas, and endocrine tumors were more common in children older than 10 years, whereas pancreatoblastoma was more common in younger children (P = .045). Almost half of patients (n = 25) presented with distant metastasis; of these, 44% were endocrine tumors. Survival was significantly greater for female patients (P = .004) and for those who had surgery (P = .001) by both univariate and multivariate analysis. There was a significant difference in tumor type 15-year survival with DA having the worst (23%) and SCT the best (100%). CONCLUSIONS Pediatric pancreatic neoplasms are uncommon and carry a variable prognosis. Both female sex and surgery were independent predictors of improved survival.

Laparoscopic vs open pyloromyotomy: a systematic review and meta-analysis

PURPOSE The aim of the study was to determine whether laparoscopic pyloromyotomy (LP) or open pyloromyotomy (OP) is the most effective intervention in infants with hypertrophic pyloric stenosis. METHODS A systematic review of the published literature was undertaken in February 2009. Prospective studies comparing LP and OP were selected. Age, weight, complications, duration of operation, time to full feedings, postoperative vomiting, and postoperative length of stay (LOS) data were extracted. RESULTS Six prospective studies (5 level I, 1 level II) with 625 (303 LP, 322 OP) participants met selection criteria. Combined estimates indicated that LP had a lower total complication rate (odds ratio [OR], 0.58 [0.35, 0.97]; P = .04), mostly due to a lower wound complication rate (OR, 0.42 for LP [0.20, 0.91]; P = .03). Patients who underwent LP also had shorter time to full feedings (mean difference [MD], -11.52 hours [-12.77, -10.27]; P < .00001) and shorter postoperative LOS (MD, -5.71 hours [-8.90, -2.52]; P = .0005). No statistically significant differences were noted in the rates of mucosal perforation, wound infection, postoperative emesis, or operating time. Incomplete pyloromyotomy occurred in 6 patients who underwent LP (OR, 7.74 [0.94, 63.38]; P = .06). CONCLUSIONS This meta-analysis favors the laparoscopic approach with significantly reduced rate of total complications, which is mostly due to a lower wound complication rate.

A Population-Based Analysis of 1037 Malignant Ovarian Tumors in the Pediatric Population

BACKGROUND Concerns of malignant potential have impacted the utilization of ovarian salvage for treatment of ovarian masses in children. METHODS The Surveillance, Epidemiology, and End Results (SEER) registry was analyzed for all females < or =19 y diagnosed with an ovarian tumor between 1973 and 2005. RESULTS Overall, 1037 pediatric patients with ovarian tumors were identified. Approximately 61.7% of tumors occurred in patients 15 to 19 y old. The age-adjusted incidence of all malignant pediatric ovarian tumors in those < or =9 y was 0.102 versus 1.072 per 100,000 in those aged 10 to 19 y. The majority of cases (57.4%) present at an early localized stage. The predominant pathology was germ cell tumors in all age groups (77.4%). Overall 5- and 10-y survival rates are 91.7% and 91.4%, respectively. By multivariate analysis, advanced disease stage (HR 3.17, P<0.001), lack of surgery (HR 4.49, P =0.039), and poorly differentiated tumors (HR 3.40, P=0.011) were associated with worse outcomes. CONCLUSIONS Malignant ovarian tumors are rare, particularly in patients under 5 y of age. Furthermore, the most common histologies are of low metastatic potential and carry high cure rates. Thus, the surgeon should implement ovarian-sparing strategies on the affected ovary unless a malignancy is clearly suspected and conserve the contralateral ovary in all children.

Malignant breast cancer in children: a review of 75 patients.

OBJECTIVE To determine incidence trends and outcomes for pediatric patients with malignant breast disease. METHODS The Surveillance, Epidemiology, and End Results registry was examined for all females 19 years of age and younger diagnosed with a malignant breast tumor between 1973 and 2004. RESULTS A total of 75 patients with malignant breast tumors were identified. Overall, 14.5% of patients had in situ tumors, and 85.5% had invasive disease. Tumors were classified as being either carcinomas (n = 41, 54.7%) or sarcomas (n = 34, 45.3%). The majority of sarcomatous lesions were phyllodes tumors (n = 29, 85.5%), whereas most carcinomas were of a ductal etiology (n = 19, 46.3%). The age-adjusted incidence of all malignant pediatric breast tumors in 2003 was 0.08 cases per 100,000 people (0.03 carcinoma and 0.06 sarcoma cases per 100,000 people). In the carcinoma group, regionally advanced disease was present in 11 patients (26.8%), whereas only 3 patients (7.3%) presented with metastatic disease. All patients with sarcomatous tumors presented with localized disease. Adjuvant radiation therapy was administered in only 9.8% of carcinomas and 8.8% of sarcomas, and 85.4% of carcinoma patients and 97.1% of sarcoma patients underwent surgical resection for their primary disease. Subgroup analysis revealed 5- and 10-year survival rates of 89.6% for patients with sarcomatous tumors and 63.1% and 54.3% for carcinomas. CONCLUSIONS Malignant pediatric breast malignancies remain relatively rare. The two most common histologies of breast neoplasms in children are malignant carcinomas followed by sarcomas. Although uncommon, malignant disease must be considered in the differential diagnosis of the pediatric patient with a breast mass.

Pediatric FAST and Elevated Liver Transaminases: An Effective Screening Tool in Blunt Abdominal Trauma

BACKGROUND The current standard for the evaluation of children with blunt abdominal trauma (BAT) consists of physical examination, screening lab values, and computed tomography (CT) scan. We sought to determine if the focused assessment with sonography for trauma (FAST) combined with elevated liver transaminases (AST/ALT) could be used as a screening tool for intra-abdominal injury (IAI) in pediatric patients with BAT. METHODS Registry data at a level 1 trauma center was retrospectively reviewed from 1991-2007. Data collected on BAT patients under the age of 16 y included demographics, injury mechanism, ISS, GCS, imaging studies, serum ALT and AST levels, and disposition. AST and ALT were considered positive if either one was >100 IU/L. RESULTS Overall, 3171 cases were identified. A total of 1008 (31.8%) patients received CT scan, 1148 (36.2%) had FAST, and 497 (15.7%) patients received both. Of the 497 patients, 400 (87.1%) also had AST and ALT measured. FAST was 50% sensitive, 91% specific, with a positive predictive value (PPV) of 68%, negative predictive value (NPV) of 83%, and accuracy of 80%. Combining FAST with elevated AST or ALT resulted in a statistically significant increase in all measures (sensitivity 88%, specificity 98%, PPV 94%, NPV 96%, accuracy 96%). CONCLUSIONS FAST combined with AST or ALT > 100 IU/L is an effective screening tool for IAI in children following BAT. Pediatric patients with a negative FAST and liver transaminases < 100 IU/L should be observed rather than subjected to the radiation risk of CT.

Peritoneal Drainage versus Laparotomy for Necrotizing Enterocolitis and Intestinal Perforation: A Meta-Analysis

BACKGROUND To determine whether peritoneal drain (PD) or laparotomy (LAP) is the most effective intervention in premature neonates with necrotizing enterocolitis (NEC) or intestinal perforation (IP). METHODS A systematic review of the published literature between January 2000 and December 2008 was undertaken. Prospective studies with at least 25 patients in each of the PD and LAP arms were selected. Gestational age, birth weight, operation, and mortality data were extracted. RESULTS Five prospective studies (two level I, three level II) with 523 (273 PD, 250 LAP) participants followed for mortality met selection criteria. Using a fixed effect model, the combined estimate indicates an increased mortality of 55% with PD (OR 1.55, 95% CI: 1.08-2.22, P=0.02) without statistical heterogeneity (chi(2)=5.88, P=0.21). PD patients were 0.78 wk younger (P =0.0002) and 67 g smaller (P =0.0006). Analysis of the three level II trials yielded a combined estimate indicating an excess mortality of 89% with PD patients (95% CI: 1.20-2.98, P =0.006) without statistical heterogeneity (chi(2)=3.74, P=0.15). CONCLUSIONS PD is associated with 55% excess mortality compared with LAP. Pediatric surgeons must individually assess and select patients with NEC and IP for optimal surgical therapy.

Incidence and Outcomes of Malignant Pediatric Lung Neoplasms

BACKGROUND We sought to define current incidence trends and outcomes for children with lung and bronchus tumors. METHODS The SEER registry was queried from 1973 to 2004 for all patients with pulmonary tumors less than 20 y of age. RESULTS Overall, 160 patients were identified. The age-adjusted incidence has remained stable at 0.049 per 100,000 persons. The median age at diagnosis was 16 y. Whites had the highest age-adjusted population incidence at 0.056 per 100,000. Most tumors arose in the lower lobe (37%), followed by the upper lobe (31.2%). The most common histology was endocrine tumor (51.6%), followed by sarcoma (11%), and mucoepidermoid tumor (9%). Overall survival was greater than 381 mo with a 15-y survival of 65%. Males had better survival (>381 versus 288 months). Endocrine and mucoepidermoid tumors had the best survival. Small cell carcinoma had the worst median survival at less than 5 mo. Squamous cell carcinoma and adenocarcinoma both had a 14-mo median survival. Median survival for nonsurgically treated patients was 14 mo with a 10-y survival rate of 32%. Surgery improved the 10-y survival to 75% (P<0.0001). Multivariate analysis demonstrated nonsurgical treatment and nonendocrine tumor histology to be independent prognostic factors of death. CONCLUSION The incidence of pediatric lung cancer remains stable. Several factors, including nonsurgical treatment and nonendocrine tumors confer a poor prognosis. Early diagnosis and surgical therapy provide the best chance for survival.

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.

Pediatric Solid Tumors and Second Malignancies: Characteristics and Survival Outcomes

BACKGROUND To examine the incidence, characteristics, and outcomes for second malignancies following the diagnosis of a primary solid tumor in pediatric patients. METHODS The Surveillance, Epidemiology, and End Results (SEER) database was queried from 1973 to 2005, excluding recurrences, in patients <20 y. RESULTS A total of 31,685 cases of pediatric solid malignancies were identified. Overall, 177 patients were diagnosed with a unique second malignancy before the age 20 (0.56%) The mean follow-up was for 8.5 y (2 mo-30.8 y). Mean age at diagnosis of the primary tumor was 7.7 y. The most common primary malignancies were CNS tumors (22.5%), followed by soft tissue sarcoma (15.8%), retinoblastoma (14.1%), and bone tumors (13%). Hematologic malignancies (35.5%) were the most common second malignancies noted in the cohort, followed by bone tumors (18%) and soft tissue sarcomas (15%). Hematologic malignancies had a shorter latency (3.1 y) compared with solid second tumors (11.6 y). The overall 10-y survival for the entire cohort was 41.5%. For most tumor categories, development of a secondary malignancy was associated with lower 5- and 10-y survival than expected. CONCLUSIONS CNS tumors, retinoblastoma, and soft tissue sarcomas in children are the most common solid primary tumors, with an increased risk of a second malignancy. Leukemia is the most common second malignancy seen in pediatric solid tumors. Second malignancies are associated with significantly reduced survival rates compared with the general childhood cancer population.