Renal masses in children may be discovered during routine clinical examination or incidentally during the course of diagnostic or therapeutic procedures for other causes. Renal cancers are rare in the pediatric population and include a spectrum of pathologies that may challenge the clinician in choosing the optimal treatment. Correct identification of the lesion may be difficult, and the appropriate surgical procedure is paramount for lesions suspected to be malignant. The purpose of this article is to provide a comprehensive overview regarding the spectrum of renal tumors in the pediatric population, both benign and malignant, and their surgical management.
The switch from a SOLE to a FOLE in PN-dependent children with cholestasis and dyslipidemia was associated with a dramatic improvement in serum triglyceride and VLDL concentrations, a significant increase in serum omega-3 (n-3) fatty acids (EPA and DHA), and a decrease in serum omega-6 fatty acids (arachidonic acid). A FOLE may be the preferred lipid emulsion in patients with PN-cholestasis, dyslipidemia, or both. This trial is registered at clinicaltrials.gov as NCT00910104.
Children with intestinal failure suffer from insufficient intestinal length or function, making them dependent on parenteral nutrition (PN) for growth and survival. PN and its components are associated with many complications ranging from simple electrolyte abnormalities to life-threatening PNassociated liver disease, which is also called intestinal failure-associated liver disease (ILALD). From a nutrition perspective, the ultimate goal is to provide adequate caloric requirements and make the transition from PN to full enteral Nutrition (EN) successful. Upon review of the literature, we have summarized the most effective and innovative PN and EN therapies for this patient population. Antibiotic-coated catheters and antibiotic or ethanol locks can be implemented, as they appear effective in reducing catheter-related infection and thus further reduce the risk of IFALD. Lipid emulsions should be given judiciously. The use of an omega-3 fatty acid-based formulation should be considered in patients who develop IFALD. Trophic feeding is important for intestinal adaptation, and EN should be initiated early to help wean patients from PN. Long term management of children with IF continues to be an emerging field. We have entered uncharted territory as more children survive complications of IF, including IFALD. Careful monitoring and individualized management to ensure maintenance of growth with avoidance of complications are the keys to successful patient outcomes.
The discovery of an appendiceal carcinoid tumor found incidentally or during the course of diagnostic or therapeutic procedures is a burden to both the patient and clinician. The role of the correct surgical operation is paramount for lesions suspected to be malignant. In the pediatric population, appendiceal carcinoids continue to challenge the clinician in choosing the optimal treatment when lesions are larger than 2 cm or involve the appendical base. While the criteria used to define these distinct lesions are available in most cases, the management and treatment are still debated and controversial when considering more radical surgical intervention. The purpose of this article is to give an overview regarding the history, diagnosis, histopathology, management, and controversies associated with appendiceal carcinoid in the pediatric population.
Background
Osteosarcoma (OS) and the Ewing sarcoma family of tumors (ESFT) are the most common primary pediatric bone malignancies. We sought to assess the diagnostic accuracy of initial tumor biopsies in patients with OS or ESFT at a pediatric cancer center.
Methods
All biopsies performed at initial presentation of patients with OS or ESFT at our institution from 2003 to 2012 were retrospectively reviewed. Diagnostic accuracy and incidence of complications were correlated with study variables using logistic regression analysis.
Results
One hundred forty-two biopsies were performed in 105 patients (median age 13.4 years, range: 1.8-23.0), 104 (73.2%) OS and 38 (27.8%) ESFT. Thirty-one (21.8%) were performed on metastatic sites. Eighty-five (76.6%) of 111 primary site biopsies were open procedures, and 26 were percutaneous (23.4%). Primary site biopsies were successful in 94.1% of open and 73.1% of percutaneous procedures. Odds of obtaining a successful diagnostic specimen were 7.8 times higher with open approach (CI: 1.6-36.8). Metastatic site biopsies were successful in 66.7% of percutaneous and 100% of open and thoracoscopic procedures.
Conclusion
Biopsy of metastatic sites was equal to primary site in obtaining diagnostic material with the added benefit of accurate staging, with few adverse events and high diagnostic yield.
Purpose
Tumor biopsies are central to the diagnosis and management of cancer and are critical to efforts in personalized medicine and targeted therapeutics. We sought to evaluate the safety and accuracy of biopsies in children with cancer.
Patients and Methods
All biopsies performed in children at our institution with a suspected or established diagnosis of cancer from 2003 to 2012 were retrospectively reviewed. Patient characteristics, and disease- and procedure-related factors were correlated with procedure-related complications and diagnostic accuracy, using logistic regression analysis.
Results
One thousand seventy-three biopsies were performed in 808 patients. Of 1025 biopsies with adequate follow-up, 79 (7.7%) were associated with an adverse event, 35 (3.4%) of which were minor (Grade 1–2) and 32 (3.1%) were major (Grade 3–4). The most common major adverse events were blood transfusion (>10cc/kg, 24 cases) and infection requiring intravenous antibiotics (6 cases). Eleven deaths (1.4%) occurred within 30 post-procedure days but the procedure may have contributed to the outcome in only two. Nine hundred twenty-six (90.3%) biopsies provided definitive histologic diagnoses. Using multivariable analysis, biopsy site, pre-procedure hematocrit, and body mass index (BMI) were associated with risk of post-procedural complications (P<0.0001, P<0.0001, and P=0.0029, respectively). Excisional biopsy and biopsy site were independently associated with obtaining a diagnostic result (P=0.0002 and P=0.0008, respectively).
Conclusion
Tumor biopsies in children with cancer are associated with a low incidence of complications and a high rate of diagnostic accuracy. The predictive factors identified for adverse outcomes may aid risk assessment and pre-procedural counseling.
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