Background Parathyroid carcinoma (PC) is a rare malignancy with a moderate prognosis. The staging system, prognostic indicators, and optimal surgical management are still under debate. This large ...cohort explores prognostic factors for PC. Methods 1,022 cases of PC in the 1998–2011 National Cancer Data Base that underwent surgery were examined for predictors of lower overall survival (OS) and relative risk (RR) of death at 5 years. Results The 5-year OS was 81.1% in 528 patients with ≥60 months of follow-up. The overall cohort was mainly non-Hispanic (96.5%), white (77.4%), and insured (94.3%), with a median age of 57 years. Mean OS was lower and RR of death greater in older ( P < .001), black ( P = .007) patients with a secondary malignancy ( P = .015) and ≥2 comorbidities ( P = .005), whose surgical specimen had positive surgical margins ( P = .026) or positive lymph nodes ( P < .001). Multivariate cox regression demonstrated that positive lymph nodes (hazard ratio HR, 6.47; 95% CI, 1.81–23.11) and older age (HR, 2.35; 95% CI, 1.25–4.43) were associated with lower OS. Conclusion PC is a rare malignancy with a 5-year OS of 81.1%. Positive lymph nodes and older age predict lower OS and an increased risk of death.
Background Well-differentiated thyroid cancer is the most common endocrine malignancy in children. Adult literature has demonstrated socioeconomic disparities in patients undergoing thyroidectomy, ...but the effects of socioeconomic status on the management of pediatric well-differentiated thyroid cancer remains poorly understood. Methods Patients ≤21 years of age with well-differentiated thyroid cancer remains were reviewed from the National Cancer Data Base. Three socioeconomic surrogate variables were identified: insurance type, median income, and educational quartile. Tumor characteristics, diagnostic intervals, and clinical outcomes were compared within each socioeconomic surrogate variable. Results A total of 9,585 children with well-differentiated thyroid cancer remains were reviewed. In multivariate analysis, lower income, lower educational quartile, and insurance status were associated with higher stage at diagnosis. Furthermore, lower income quartile was associated with a longer time from diagnosis to treatment ( P < .002). Similarly, uninsured children had a longer time from diagnosis to treatment (28 days) compared with those with government (19 days) or private (18 days) insurance ( P < .001). Despite being diagnosed at a higher stage and having a longer time interval between diagnosis and treatment, there was no significant difference in either overall survival or rates of unplanned readmissions based on any of the socioeconomic surrogate variables. Conclusion Children from lower income families and those lacking insurance experienced a longer period from diagnosis to treatment of their well-differentiated thyroid cancer remains. These patients also presented with higher stage disease. These data suggest a delay in care for children from low-income families. Although these findings did not translate into worse outcomes for well-differentiated thyroid cancer remains, future efforts should focus on reducing these differences.
Abstract Background Primary hepatic neoplasms in children are rare tumors. All malignant and medically refractive benign primary pediatric liver tumors ultimately require surgical resection for cure. ...Accurate preoperative imaging including multidetector helical computerized tomography or magnetic resonance imaging (MRI) is necessary to determine resectability. In the literature intraoperative ultrasound (IOUS) has proven to be a vital adjunct to liver surgery in adults, but this is not well established in children. Materials and methods Between April 2003 and November 2014, children (<18-y-old) with a primary liver neoplasm, preoperatively evaluated with multidetector helical computerized tomography or MRI, who had IOUS used at the time of surgery were retrospectively reviewed. Results Preoperative evaluation with high-resolution MRI and IOUS were discordant in 4 of 19 patients (21%). In one case, right hepatic vein involvement was not accurately assessed with MRI. Two cases showed tumor involvement in segment IV by MRI; however, IOUS revealed no medial segment involvement. The final patient had a large (>5 cm), solitary hepatic adenoma on MRI, but IOUS in this case revealed diffuse adenomatosis. The operative management was altered in three of these cases. Conclusions Although MRI can provide a detailed view of the hepatic anatomy and is an invaluable tool for preoperative planning for the pediatric patient with a primary liver neoplasm, IOUS may provide further and more up to date delineation of tumor extent and should be considered a crucial element in operative planning for hepatectomy in children.
Abstract Background Appendiceal carcinoid tumors, also know as well-differentiated neuroendocrine neoplasms, are rare lesions detected incidentally after appendectomy in children. There are limited ...data about the natural history of these tumors, and guidelines regarding family counseling and need for additional surgery or follow-up imaging are not established in the pediatric age group. The purpose of this study was to review our institutional experience with appendiceal carcinoid tumors to provide data that might improve management. Methods After institutional review board approval, the charts of all patients treated at our institution for an appendiceal carcinoid tumor between 2002 and 2014 were reviewed. Data collected included patient demographics, pathologic details, postoperative management, and follow-up information. Descriptive analyses were performed. Results Twenty-eight patients were identified, which represents an incidence of 0.2% of children undergoing appendectomy during that time interval. The mean age at surgery was 13.8 ± 2.1 y; 54% were females. Two patients had symptoms suspicious for carcinoid syndrome at presentation, though none had evidence of metastatic disease. The mean tumor size was 0.73 ± 0.4 cm. Five patients (18%) underwent subsequent ileocecectomy or right hemicolectomy because of pathologic findings of invasion of the mesoappendix ( n = 4) or lymphovascular invasion and subserosal extension ( n = 1), two of whom had residual disease in the resected specimen (one in a lymph node). No recurrences have been detected at mean follow-up of 1.8 y. Conclusions Appendiceal carcinoid tumors are discovered incidentally in about 0.2% of children undergoing appendectomy. Based on findings from a large contemporary series, we can conclude that these tumors are generally small and demonstrate lymphovascular invasion or mesenteric extension in fewer than 20% of cases. Prospective, multicenter studies are necessary to better define the indication for ileocecectomy and follow-up imaging protocols.
Abstract Background Hospitalized infants >1 y old often require central venous catheters (CVC) for prolonged therapy. There are limited data describing the complication profile for this young ...population. The purpose of this study was to review outcomes associated with CVC insertion in this high-risk group and compare them to those in older children to develop directed quality improvement projects. Materials and methods Patients receiving their first CVC, a tunneled central line or port-a-cath, from 2007–2010 were included. Femoral, non-tunneled, and hemodialysis catheters were excluded. Patients aged 0–12.0 mo (infants) were compared with those 12.1–36 mo (toddlers). Complications (<30 d) included infection, malposition, malfunction, intraoperative, and the need for operative exchange. Statistical analysis included Student t -test, chi-square, and a Kaplan–Meier survival analysis. Results We identified 115 infants and 129 toddlers who underwent CVC insertion during the study period. Complication rates were higher in the infant group than in the toddler group, as was the operative exchange rate. Higher infection rates in the infant group appeared to contribute to the difference in early complications and exchange rates. A survival analysis indicated improved catheter duration in toddlers ( P = 0.001). Conclusions In this cohort study, infants had a higher early complication rate, mostly attributable to infection, than their older counterparts. This difference could be explained by increased use of a tunneled central line for daily total parented nutrition in infants with gastrointestinal anomalies, as opposed to port-a-cath for chemotherapy infusion in older children. These data have prompted a number of targeted quality improvement initiatives to address relevant complications in this infant population.
Abstract Background Lipoblastomas are rare, benign, soft tissue tumors that occur primarily in young children. Treatment includes complete excision and surveillance for recurrence. Lipoblastomas can ...be indistinguishable from other benign lipomatous tumors and liposarcomas. Cytogenetic analysis can provide the definitive diagnosis in questionable cases, because benign and malignant lipomatous tumors exhibit specific nonrandom cytogenetic abnormalities. The purpose of the present study was to discuss the disease management and outcomes in a large contemporary group of patients with lipoblastoma. Materials and methods A retrospective chart review of patients diagnosed with lipoblastoma presenting from 2000–2011 was conducted. The data from these patients were compared with data from a previously published historical group of patients (1985–1999) from the same children's hospital. Results We identified 37 patients in the contemporary cohort group and compared them with 25 patients from the historical group. The tumor involvement sites were similar. The current cohort group had a lower recurrence rate, although this might have been underestimated owing to a shorter follow-up period (median 1.4 y, range 2 wk to 11.0 y). Preoperative imaging findings led to an incorrect diagnosis in 62% of the patients. Cytogenetic analysis was used to help determine the final diagnosis in 50% of the cases. In 39% of cases, translocations involved the long arm of chromosome 8, the most common anomaly in lipoblastoma. Conclusions Lipoblastomas are rare tumors in young children that can be misclassified as other malignant or benign lipomatous tumors with markedly different outcomes and treatments. We recommend that cytogenetic analysis be routinely used for all pediatric lipomatous tumors to provide an accurate diagnosis and guide appropriate therapy and follow-up.
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