Jennifer H. Aldrink

Intestinal location of necrotizing enterocolitis among infants with congenital heart disease

Objective:To determine whether differences exist in the location of necrotizing enterocolitis (NEC) in infants with congenital heart disease (CHD) versus those without CHD.Study design:Retrospective cohort study utilizing 11 years of patient data. Inclusion criterion was surgical exploration for NEC. Presence or absence of CHD was determined. Surgical and/or pathology reports were reviewed to identify the location of NEC. Data were analyzed by t-tests and χ2 analyses.Result:One hundred and sixty-seven patients met the inclusion criteria. CHD infants had a higher percentage of mortality. There was no difference in the location of NEC between non-CHD and CHD patients, with the predominant location being the small intestine in both. In addition, there was no difference in the location of NEC between preterm non-CHD patients and full-term CHD patients with the small intestine again being the primary site.Conclusion:Despite differences in gestational age between non-CHD and CHD patients, the location of NEC in these infants did not differ.

Severe intraoperative hypertension after induction of anesthesia in a child with a neuroblastoma

Neuroblastomas are the most common, non-central nervous system tumor of childhood. Similar to pheochromocytomas, they are derived from neural crest cells and therefore retain the potential to synthesize catecholamines. Unlike pheochromocytomas, however, perioperative issues related to blood pressure instability with hypertension are uncommon. We report details of a 3-year-old child with a neuroblastoma who developed severe hypertension and end-organ effects after induction of anesthesia. The association of such problems with neuroblastoma is reviewed and options for perioperative care presented.

Mechanical bowel preparation for children undergoing elective colorectal surgery.

Objectives: Adult literature supports the elimination of mechanical bowel preparation (MBP) for elective colorectal surgical procedures. Prospective data for the pediatric population regarding the utility of MBP are lacking. The primary aim of this study was to compare infectious complications, specifically anastomotic leak, intraabdominal abscess, and wound infection in patients who received MBP with those who did not. Methods: A randomized pilot study comparing MBP with polyethylene glycol with no MBP was performed. Patients, 0 to 21 years old, undergoing elective colorectal surgery were eligible and randomized within 4 age strata. Statistical analyses were performed using &khgr;2 or Fisher exact test for categorical data and t test or Wilcoxon 2-sample test for continuous data. Results: Forty-four patients were enrolled in the study from December 2010 to February 2013, of which 24 (55%) received MBP and 20 (45%) did not. Two patients (5%) had anastomotic leak, 4 (9%) had intraabdominal infection, and 7 (16%) had wound infections. The rate of anastomotic leak, intraabdominal abscess, and wound infection did not differ between the 2 groups. Conclusions: MBP for elective colorectal surgery in children does not affect the incidence of infectious complications. A larger multiinstitutional study is necessary to validate the results of this single-institution pilot study.

What is new in pediatric surgical oncology

Purpose of review As pediatric oncology has become more complex, designing and maintaining pediatric surgical protocols require greater expertise. The primary purpose of this review is to summarize the changes in protocols and new study findings, which have changed surgical practice for children with solid tumors. Recent findings The most common solid tumors treated by surgery are reviewed. There are new methods of surgical techniques such as the use of sentinel lymph node techniques for the assessment of lymph nodes in pediatric sarcoma. New studies showing excellent outcomes for the nonoperative treatment for young patients with neuroblastoma are discussed. Surgical quality with the prevention of tumor rupture and the assessment of lymph nodes is essential for good outcomes in patients with Wilms tumor. The international collaborations within germ cell and liver tumors have provided excellent new study regimens and welcomed a new era of studies that are robust and have the power to change the outcomes for these rare tumors. Summary Surgical practice is evolving with the molecular advances in pediatric oncology. Technical advances and quality improvement endeavors to ensure protocol adherence are essential. International partnerships have allowed for greater advances particularly for rare tumors and improve overall surgical outcomes.

Global microRNA profiling for diagnostic appraisal of melanocytic Spitz tumors

BACKGROUNDnMelanoma skin cancer remains the leading cause of skin cancer-related deaths. Spitz lesions represent a subset of melanocytic skin lesions characterized by epithelioid or spindled melanocytes organized in nests. These lesions occupy a spectrum ranging from benign Spitz and atypical Spitz lesions all the way to malignant Spitz tumors. Appropriate management is reliant on accurate diagnostic classification, yet this effort remains challenging using current light microscopic techniques. The discovery of novel biomarkers such as microRNAs (miR) may ultimately be a useful diagnostic adjunct for the evaluation of Spitz lesions. miR expression profiles have been suggested for non-Spitz melanomas but have yet to be ascribed to Spitz lesions. We hypothesized that distinct miR expression profiles would be associated with different lesions along the Spitz spectrum.nnnMATERIALS AND METHODSnRNAs extracted from paraffin-embedded, formalin-fixed tissues of 11 resected skin lesions including benign nevi (nxa0=xa02), benign Spitz lesions (nxa0=xa03), atypical Spitz lesions (nxa0=xa03), and malignant Spitz tumors (nxa0=xa03) were analyzed by the NanoString platform for simultaneous evaluation of over 800 miRs in each patient sample.nnnRESULTSnBenign Spitz lesions had increased expression of miR-21-5p and miR-363-3p compared with those of benign nevi. Malignant Spitz lesions exhibited overexpression of miR-21-5p, miR-155-5p, and miR-1283 relative to both benign nevi and benign Spitz tumors. Notably, atypical Spitz tumors had increased expression of miR-451a and decreased expression of miR-155-5p expression relative to malignant Spitz lesions. Conversely, atypical Spitz lesions had increased expression of miR-21-5p, miR-34a-5p, miR-451a, miR-1283, and miR-1260a relative to benign Spitz tumors.nnnCONCLUSIONSnOverall, distinct miR profiles are suggested among Spitz lesions of varying malignant potential with some similarities to non-Spitz melanoma tumors. This work demonstrates the feasibility of this analytic method and forms the basis for further validation studies.

Obesity is a Risk Factor for Renal Toxicity and Wound Complications among a Cohort of Pediatric Cancer Patients at a Single Tertiary Care Institution

Background: Evidence exists in the adult literature linking obesity to an elevated risk for the development of certain malignancies, and to an increase in treatment-related complications. Little information is available examining this relationship in the obese pediatric oncology population. Methods: We performed a retrospective analysis for all patients with a malignancy and treated at our institution between January, 2005 and December, 2009. Body mass index (BMI) was calculated, categorizing them as obese or nonobese based upon their BMI percentile. Stratification by tumor type (leukemia/lymphoma, solid tumor) was performed. Data on the incidence of fifteen potential complications were recorded. Results: Sixty-three patients (17%) were classified as obese, and 302 (83%) as nonobese. Wound complications occurred more frequently in obese patients with leukemia/lymphoma compared to nonobese patients (13.2% vs. 1.6%, p=0.0075). Renal toxicity occurred more frequently in the obese patients than in the nonobese patients (38.1% vs. 26.2% (p=0.06). Conclusions: In a growing population of obese pediatric patients, certain malignancy-related treatment complications may occur at an increased incidence, including higher rates of renal toxicity and wound complications. This report highlights early treatment-related complications by BMI in pediatric patients with cancer, and demonstrates the need for an individualized approach in treating this population.

Classification of Indeterminate Melanocytic Lesions by MicroRNA Profiling.

PurposeIdentification of indeterminate melanocytic skin lesions capable of neoplastic progression is suboptimal and may potentially result in unnecessary morbidity from surgery. MicroRNAs (miRs) may be useful in classifying indeterminate Spitz tumors as having high or low risk for malignant behavior.MethodsRNA was extracted from paraffin-embedded tissues of benign nevi, benign Spitz tumors, indeterminate Spitz tumors, and Spitzoid melanomas in adults (nxa0=xa062) and children (nxa0=xa028). The expression profile of 12 miRs in adults (6 miRs in children) was analyzed by real-time polymerase chain reaction.ResultsBenign Spitz lesions were characterized by decreased expression of miR-125b and miR-211, and upregulation of miR-22, compared with benign nevi (pxa0<xa00.05). A comparison of Spitzoid melanomas to benign nevi revealed overexpression of miR-21, miR-150, and miR-155 in the malignant primaries (pxa0<xa00.05). In adults, Spitzoid melanomas exhibited upregulation of miR-21, miR-150, and miR-155 compared with indeterminate Spitz lesions. Indeterminate Spitz lesions with low-risk pathologic features had lower miR-21 and miR-155 expression compared with Spitzoid melanoma tumors in adults (pxa0<xa00.05), while pathologic high-risk indeterminate Spitz lesions had increased levels of miR-200c expression compared with low-risk indeterminate lesions (pxa0<xa00.05). Pediatric Spitzoid melanomas exhibited increased miR-21 expression compared with indeterminate Spitz lesions (pxa0<xa00.05). Moreover, miR-155 expression was increased in indeterminate lesions with mitotic counts >1 and depth of invasion >1xa0mm, suggesting miR-155 expression is associated with histological characteristics.ConclusionsmiR expression profiles can be measured in indeterminate Spitz tumors and correlate with markers of malignant potential.

Fatal extraintestinal adrenal malignancy in a 12-year-old girl with familial adenomatous polyposis.

Familial adenomatous polyposis (FAP) is a disease of significant morbidity and risk of early mortality caused by colorectal or periampullary cancer, or noncancerous desmoid tumors. In the pediatric and adolescent FAP population, precancerous and cancerous tumors of the colon, thyroid, liver, and cen

Technical Considerations for Nephron-Sparing Surgery in Children: What Is Needed to Preserve Renal Units?

BACKGROUNDnChemotherapy is used preoperatively for children with bilateral Wilms tumor (BWT) or unilateral high-risk Wilms tumor (UHRWT) to promote tumor regression to facilitate renal preservation with nephron-sparing surgery (NSS). In adults, various surgical techniques have been described to preserve renal tissue. Few studies have examined the use of surgical adjuncts in NSS in children with renal tumors.nnnMETHODSnWe performed a multi-institutional retrospective review of patients with BWT or UHRWT. Patient demographics, tumor size at diagnosis, following neoadjuvant chemotherapy, utilization of surgical adjuncts including intraoperative ultrasound (IOUS), margin status, complications, renal function, and follow-up were recorded.nnnRESULTSnThe cohort comprised 23 patients: 18 BWT, 3 UHRWT, and 2 patients with solitary kidney. Twenty-two of the 23 patients had successful NSS. IOUS was used 19 times, and seven had positive margins after surgery. Cooling/vascular isolation was used six times. At a median follow-up of 18 mo, median estimated glomerular filtration rate Schwartz was 126xa0mL/min/1.73xa0m2 and median serum creatinine 0.39xa0mg/dL in the 22 patients who had successful NSS. There have been no tumor recurrences.nnnCONCLUSIONSnIn patients with BWT and UHRWT, surgical adjuncts such as cooling/vascular isolation are uncommonly performed. IOUS may be helpful but does not guarantee negative microscopic margins.nnnLEVEL OF EVIDENCEnLevel 4, Case series with no comparison group.

Update on neuroblastoma

Neuroblastoma is an embryonic cancer arising from neural crest stem cells. This cancer is the most common malignancy in infants and the most common extracranial solid tumor in children. The clinical course may be highly variable with the possibility of spontaneous regression in the youngest patients and increased risk of aggressive disease in older children. Clinical heterogeneity is a consequence of the diverse biologic characteristics that determine patient risk and survival. This review will focus on current progress in neuroblastoma staging, risk stratification, and treatment strategies based on advancing knowledge in tumor biology and genetic characterization. TYPE OF STUDY: Review article. LEVEL OF EVIDENCE: Level II.