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The case of an 18.5-month-old girl with watery diarrhea and poor weight gain is discussed to arrive at a better understanding of ganglioneuroma with immunoreactivity to vasoactive intestinal peptide antigen. The treatment methodologies and management regime for the same is also highlighted.

A study investigates breast cancer surveillance practices among women cancer survivors, previously treated with chest radiation for a childhood cancer. Results suggest that young women at risk following chest radiation for a pediatric cancer are not being routinely and appropriately screened for breast cancer.

As advancements in the treatment of childhood cancer have resulted in increasing survival rates, the psychosocial functioning of child patients has become an increasingly important issue. In this pilot study, we investigate the relationships among parents' characteristics, children's characteristics, and the quality of life experienced by children who are diagnosed with cancer. Forty-seven mothers, sixteen fathers, and nineteen children completed measures about their own psychological functioning as well as measures about the children's quality of life. Mothers' ratings of their children's quality of life were correlated positively with the ratings provided by both fathers and the children themselves. In addition, significant relationships were found between mothers' depression and parenting stress and children's quality of life as well as between mothers' and fathers' anxiety and children's quality of life. Finally, using regression analyses, mothers', fathers', and children's ratings of their own characteristics predicted significantly their ratings of the children's quality of life. The importance of examining the psychological characteristics of family members when assessing the quality of life of children who are diagnosed with cancer is discussed. Keywords Cancer * Quality of Life * Psychological Symptoms * Parents * Children

BACKGROUND: The potential for widespread exposure to agricultural pesticides through drift during application raises concerns about possible health effects to exposed children living in areas of high agricultural activity. OBJECTIVES: We evaluated whether residence in a county with greater agricultural activity was associated with risk of developing cancer in children < 15 years of age. METHODS: Incidence data for U.S. children 0-14 years of age diagnosed with cancer between 1995 and 2001 were provided by member registries of the North American Association of Central Cancer Registries. We determined percent cropland for each county using agricultural census data, and used the overall study distribution to classify agriculturally intense counties. We estimated odds ratios and 95% confidence intervals for all ages and 5-year age groups for total cancers and selected cancer sites using logistic regression. RESULTS: Our study results showed statistically significant increased risk estimates for many types of childhood cancers associated with residence at diagnosis in counties having a moderate to high level of agricultural activity, with a remarkably consistent dose-response effect seen for counties having [greater than or equal to] 60% of the total county acreage devoted to farming. Risk for different cancers varied by type of crop. CONCLUSIONS: Although interpretation is limited by the ecologic design, in this study we were able to evaluate rarer childhood cancers across a diverse agricultural topography. The findings of this exploratory study support a continued interest in the possible impact of long-term, low-level pesticide exposure in communities located in agriculturally intense areas. KEY WORDS: agricultural pesticides, childhood cancers, farming, pediatric cancers. Environ Health Perspect 116:559-565 (2008). doi:10.1289/ehp.9967 available via http://dx.doi.org/ [Online 10 January 2008], Increased incidence of certain cancers among farmers and workers employed in agricultural settings has been reported in a variety of epidemiologic studies, raising concerns about exposure to agricultural chemicals in [...]

Using procedures identical to a study published 10 years earlier, parents of 49 children with cancer (48 mothers, 33 fathers) and 49 healthy classmates (49 mothers, 29 fathers) completed measures [...]

A study was conducted to investigate the large and geographically diverse cohort that is followed in the childhood Cancer Survivor Study (CCSS), with the goal of determining the prevalence, incidence, and severity of chronic health conditions in adult survivors of childhood cancer, and to determine the risk of chronic conditions in the survivors, as compared with their siblings. The findings suggest that survivors of childhood cancer have a high rate of illness owing to chronic health conditions.

Background: Image-guided core needle biopsy is widely used in paediatric oncology, but many protocols continue to discourage this practice. No published randomized studies compare image-guided needle biopsy with surgical techniques. Objective: To perform a systematic review of the literature on image-guided core needle biopsy in paediatric oncology. Materials and methods: Several computerized databases were searched using the terms [(needle OR core) AND (biops*[ti]) AND (paediatric OR pediatric OR child OR children OR childhood OR boy OR girl)[ti]] to identify series of more than five cases of needle core biopsy for tumour diagnosis in children. Data from included studies were combined to calculate pooled estimates of adequacy, accuracy and complication rates. Results: Thirteen studies fulfilled the inclusion criteria. Overall biopsy adequacy rate (defined as sufficient to make a diagnosis) was 94% (95% CI 92-96%). The diagnostic accuracy rate in cases with adequate material (defined as achieving the correct specific diagnosis) was 94% (95% CI 92-96%). Complications requiring treatment occurred in 1%. Conclusions: Available pooled data suggest that about 95% of image-guided needle core biopsies provide an adequate sample for diagnosis of malignant disease in childhood. In such cases, the pathological diagnosis is correct in about 95%. Complications are rare. Keywords Needle biopsy * Tumour * Diagnosis * Histopathology * Children

Byline: Tutku Soyer (1), Adegbrahim Karnak (1), Arbay O. Ciftci (1), Mehmet Emin Senocak (1), F. Cahit Tanyel (1), Nebil Buyukpamukcu (1) Keywords: Chest wall tumor; Child; Reconstruction; Resection; Surgery Abstract: Chest wall tumors (CWT) are rarely seen in childhood and surgery constitutes a complementary part of the therapy. The early and late results of CWT resection and chest wall reconstruction were evaluated retrospectively. The children who underwent chest wall resection for CWT between January 1990 and November 2003 were evaluated retrospectively. Seventeen children (male/female=12/5, mean age: 7.58 years) underwent chest wall resection for CWT. Fifteen patients underwent initial biopsy (tru-cut, n=8 or open biopsy, n=7) and two underwent initial resection. The diagnosis was malignant tumor in 12 (70%) and benign in 5 (30%). They were Ewing's sarcoma (ES) (n=4), primitive neuroectodermal tumor (PNET) (n=3), Askin's tumor (n=1), rhabdomyosarcoma (RMS) (n=2), neuroblastoma (n=2), osteochondroma (n=1), aneurysmal bone cyst (n=2) and hamartoma (n=2). Preoperative chemotherapy was given to most patients with malignant tumor. All patients had only local tumor at the time of resection. Thoracotomy was performed in all patients. All tumor tissues with the affected rib/ribs were resected en bloc with the adjacent tissues. The number of resected ribs was 1 (n=6), 2 (n=7) and 3 (n=4). Chest wall defects were repaired primarily (n=8) or with grafts (n=9). Dura (n=4), Neuro-patch (n=3) and Goretex (n=2) were used for closure. Wound infection and pleural fistula occurred in one patient. Patients with benign tumor were free of complaints or complications during follow up. All patients with malignant tumor received postoperative chemotherapy. Local recurrence did not occur in all patients. Five patients developed distant metastasis and two died. Scoliosis was encountered in one patient during follow-up. Since most of the CWT are malignant and not initially suitable for surgical excision, the management includes tissue diagnosis either by tru-cut or open biopsy. Determination of malignant condition should be followed by an intensive chemotherapy. Chest wall resection is planned to control local disease. Chest wall reconstruction may be needed for large defects following resection of CWT. Prosthetic materials can be used safely. Early complications of the surgery are limited. The patients should be closely followed up for late complications such as scoliosis, restrictive pulmonary disease and for the development of metastasis, which is a part of natural course of malignant CWT in children. Author Affiliation: (1) Department of Pediatric Surgery, Faculty of Medicine, Hacettepe University, 06100, SA+-hhiye Ankara, Turkey Article History: Registration Date: 25/08/2005 Accepted Date: 03/08/2005 Online Date: 19/11/2005

Byline: Floyd H. Gilles (1), C. Jane Tavare (1), Alan Leviton (2), E. Tessa Hedley-Whyte (3), Anna Sotrel (4), Lester Adelman (5), Richard L. Davis (6), Lucy B. Rorke (7) Keywords: brain tumor; child; grading; posterior fossa Abstract: The Daumas-Duport grading scheme (DDGS) utilizes four histologic features in an additive method (grade 1 if none present, grade 2 if only one is present, etc.). Its efficacy in achieving prognostically homogeneous groups of childhood infratentorial neuroglial tumors and its concordance with World Health Organization (WHO) diagnoses has not been evaluated. We investigated these questions using the Childhood Brain Tumor Consortium (CBTC) database of 1241 neuroglial tumors limited to the infratentorial compartment. We calculated survival function estimates for various DDGS grades as well as the histologic features within each grade. The feature of endothelial prominence improved survival expectation, whereas the remaining three features of nuclear atypia, mitoses, and necrosis were associated with worsened survival. Survival estimates for tumors with DDGS grades 2 and 3 did not differ. Some grades contained feature subsets with significantly different survival distributions. The survival distributions of DDGS grade 1, DDGS grade 2 with only endothelial prominence, and DDGS grade 3 with nuclear atypia and endothelial prominence were not significantly different. DDGS grade within WHO diagnoses had no significant effect on survival expectation. We conclude that grading by summation of only four histologic features, as in the DDGS, is inappropriate for assessment of childhood neuroglial tumors. A classification scheme considering the complete histologic content is more likely to provide clinically useful diagnoses. Such a scheme, based on the CBTC database is available. This scheme uses 26 histologic features identified as reliable in read--reread studies. Author Affiliation: (1) Department of Pathology and Laboratory Medicine, Division of Neuropathology, Childrens Hospital Los Angeles and University of Southern California School of Medicine, 4650 Sunset Boulevard, MS 43, Los Angeles, CA, 90027, USA (2) Department of Neuroepidemiology, Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA, USA (3) Department of Neuropathology, Massachusetts General Hospital and Harvard Medical School, 14 Fruit Street, Boston, MA, 02114-2696, USA (4) Department of Pathology, Miami Children's Hospital, 3100 S.W. 62nd Avenue, Miami, FL, 33155, USA (5) Department of Pathology Laboratory, Tufts--New England Medical Center Hospital, 750 Washington Street, Tufts--NEMC 802, Boston, MA, 02111, USA (6) Department of Pathology, University of California San Francisco, HSW 501--School of Medicine, 3rd and Parnassus, San Francisco, CA, 94143-0506, USA (7) Department of Neuropathology, Children's Hospital of Philadelphia and University of Pennsylvania, 324 S. 34th Street, Philadelphia, PA, 19104, USA Article History: Registration Date: 01/01/2003 Received Date: 21/06/2003 Accepted Date: 15/10/2003 Online Date: 04/03/2004 Article note: a See Appendix.

Byline: Megan J. DiFurio (1), Aaron Auerbach (2), Keith J. Kaplan (1) Keywords: Lung cancer; Neuroendocrine differentiation; Pulmonary blastoma; Pulmonary endodermal tumor; Well-differentiated fetal adenocarcinoma Abstract: Well-differentiated fetal adenocarcinoma (WDFA) is a rare tumor of the lung, which has gone by many names over the years. The lesion was first described by Kradin et al., in 1982, who called it 'pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung).' Since then, there have been at least 65 cases reported in the literature. Although there has been no consensus in the literature as to the best pathological term for this entity, the most recent World Health Organization classification of lung and pleural tumors uses the term well-differentiated fetal adenocarcinoma. Characteristically, this lesion consists of an epithelium, which recapitulates fetal lung at 3--5 months of gestation and demonstrates neuroendocrine differentiation. Although the classic age range is 30--40 years, there have been seven reports of WDFA in the pediatric age. We report an additional pediatric case of this tumor and review the pediatric cases in the existing literature. Author Affiliation: (1) Department of Pathology, Walter Reed Army Medical Center, Ward 47, Bldg. 2, 6900 Georgia Avenue N.W., Washington, DC 20307-5001, USA (2) National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA Article History: Registration Date: 01/01/2003 Received Date: 29/04/2003 Accepted Date: 03/08/2003 Online Date: 23/10/2003 Article note: The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.

Byline: Neil James Sebire (), Alan Drummond Ramsay (), Gillian Levitt (), Marian Malone (), Rupert Anthony Risdon () Abstract: Malignant soft tissue tumors other than rhabdomyosarcoma (RMS) are uncommon in infancy, representing approximately 5% of pediatric sarcomas. The pathological categorization of non-RMS soft tissue malignancies from these young patients is complicated by variation in both morphologic and immunohistochemical features. A search covering an 11-year period identified 19 patients presenting at birth or in infancy with a clinical or referral diagnosis of soft tissue sarcoma. After histologic and immunohistochemical review, nine of these tumors were classified as primitive neuroectodermal tumor (PNET), three as infantile hemangiopericytoma (HPC), two as infantile fibrosarcoma (FS), and five as undifferentiated sarcoma. Those identified as undifferentiated sarcomas showed an atypical spindle and ovoid cell morphology, with cellular pleomorphism and high mitotic rate, but lacking the fascicular growth pattern of classic infantile fibrosarcoma. Immunohistochemical staining in this group showed variable weak positivity for a range of markers (desmin, smooth muscle actin, Myo-D1, PGP, NSE, S100, CO56, cytokeratin, and CD99), and did not fit readily into any distinct diagnostic category. In this series, tumors classified as soft tissue PNETs had a poor prognosis despite aggressive treatment. However, once RMS, PNET, and other rare specific lesions are excluded, the remaining undifferentiated sarcomas, despite their unusual morphology and immunohistochemistry, appear to behave in a similar favorable manner to infantile fibrosarcoma. Author Affiliation: () Department of Histopathology, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK, GB Article History: Received Date: 17/02/2002 Accepted Date: 15/03/2002

* Context.--Solitary papillary hyperplastic thyroid nodules (SPHTNs) are frequently encountered in children and teenagers. Although the histologic features are well described, to the best of our knowledge, cytologic findings have [...]

Byline: P.K. Ramdial (1), A. Madaree (2) Keywords: Key words: cranial, childhood, malignant, solitary fibrous tumor, CD-34 antigen Abstract: Although solitary fibrous tumor (SFT) was originally described as a pleural tumor, an increasing number of extrapleural sites of SFTs have been documented. This has been attributed not only to the heightened awareness of the spectrum of histopathological features that characterizes SFTs but also to the recognition of the role of CD34 immunostaining in soft tissue tumors in general, and in SFTs in particular. Despite the large number of documented extrapleural SFTs in adults, cranial SFTs are rare, having been documented in the meninges, scalp, and infratemporal fossa. Extrapleural SFTs are, to date, an unrecognized entity in children. We document an aggressive fibrous scalp lesion in a 30-month-old female child that demonstrated features common to benign cranial fasciitis and SFT. However, based on bright, diffuse CD34 antigen immunopositivity, a diagnosis of SFT was made. The need to include the CD34 antigen stain in a panel of immunohistochemical markers used to assess spindle cell lesions of childhood is emphasized. Author Affiliation: (1) Department of Pathology, Faculty of Medicine, University of Natal, Private Bag 7, Congella, 4013, South Africa, ZA (2) Department of Plastic and Reconstructive Surgery, Faculty of Medicine, University of Natal, Private Bag 7, Congella, 4013, Kwazulu Natal, South Africa, ZA Article note: Received March 29, 1999 accepted February 28, 2000.

Byline: Sherrie L. Perkins (1) Keywords: Key words: lymphoblastic lymphoma, large cell lymphoma, anaplastic large cell lymphoma, Burkitt's lymphoma, pediatric non-Hodgkin's lymphoma Abstract: Non-Hodgkin's lymphomas (NHL) in children and adolescents represent about 10% of childhood cancers. Although the types of NHL commonly seen in this population are relatively limited to lymphoblastic lymphomas, Burkitt's and Burkitt-like lymphomas, and large cell lymphomas, correct diagnosis and classification are essential for optimal therapy. Careful handling of pathologic specimens, along with collection of proper materials for ancillary studies such as immunophenotyping, cytogenetics, or molecular studies, will aid the pathologist in reaching a correct diagnosis. Specific morphologic, immunophenotypic, and genetic features of the commonly seen types of pediatric NHL are described. Author Affiliation: (1) Department of Pathology, University of Utah Health Sciences and ARUP Laboratories, 50 N. Medical Drive, Salt Lake City, UT 84132, USA, US

Byline: Daniel R. Boue (1), David M. Parham (2), Bruce Webber (3), William M. Crist (4), Stephen J. Qualman (1) Keywords: Key words: ectomesenchymoma, malignancy, neuroectodermal/neuroblastic, rhabdomyosarcoma Abstract: Ectomesenchymomas (EM) are rare malignant neoplasms usually consisting of rhabdomyosarcoma (RMS) with a neural component. Only 21 cases have been previously reported. Here we extend the clinicopathologic spectrum of EM by describing our findings in 15 cases. Only 5 patients were infants 10 were a$?3 years old and 5 were aY=6 years old. No male predilection was observed 7 were female. The originating institutional diagnoses were RMS (12), undifferentiated sarcoma (1), or EM (2), suggesting underdiagnosis of this entity. The primary tumor sites included external genital (5), pelvis/abdomen (6), head and neck (3), and extremity (1). The size of the primary neoplasm was usually aY=5 cm at diagnosis but dissemination only occurred in a minority. Local infiltration was not uncommon. These neoplasms were typically multilobate, thinly encapsulated, hemorrhagic, and necrotic. Light microscopic features were highly variable, but embryonal RMS with scattered or clustered ganglion cells, often in lacunae, was characteristic. In some cases, primitive neuroblastic or neuroectodermal areas were found and/or a component of alveolar RMS was seen. Focal anaplasia was occasionally observed. Mitotic activity appears higher than previously appreciated and some necrosis was invariably present. Electron microscopy was performed in 11 cases, which confirmed skeletal muscle +- neural differentiation. Cytogenetic studies performed in five cases revealed no specific abnormality. Monoclonal neuron-specific enolase was the best marker of ganglion cells and primitive neural elements. MIC-2 (CD99) membrane expression was not definitively present in any of the six cases examined. A number of the above parameters appear to be of some prognostic significance, but overall, these neoplasms appear to have a similar outcome as would be predicted for their RMS element alone (exclusive of any neural component), with respect to the RMS subtype, age of the patient, and anatomic location of the neoplasm. Author Affiliation: (1) Department of Laboratory Medicine and IRSG Pathology Center, Children's Hospital and Ohio State University School of Medicine, 700 Children's Drive, Columbus, OH 43205, USA, US (2) Department of Pediatric Pathology, Arkansas Children's Hospital, University of Arkansas--Medical School, 800 Marshall Street, Little Rock, AR 72202, USA, US (3) Department of Pathology, Baptist Memorial Hospital, 899 Madison Avenue, Memphis, TN 38146, USA, US (4) Department of Pediatric and Adolescent Medicine, Mayo Clinic, Desk East 9 (PEDS), 200 First Street SW, Rochester, MN 55905, USA, US