Birth weight, fetal growth, and risk of pediatric rhabdomyosarcoma: an updated record linkage study in California.

PURPOSE: The purpose of the study was to examine whether birth characteristics affect the risk of rhabdomyosarcoma (RMS) in children and adolescents younger than 19 years. METHODS: A total of 722 RMS cases diagnosed at the age of 0-19 years during 1988-2011 were identified from the California Cancer Registry and matched by birth date, sex, and race to 2,888 controls using California birth records. Conditional logistic regression was used to estimate the risk of RMS associated with birth weight, gestational age, and size for gestational age. RESULTS: High birth weight (odds ratio [OR]: 1.00; 95% confidence interval [CI]: 0.78-1.29) and large for gestational age (LGA; OR: 0.94, 95% CI: 0.72-1.23) were not associated with RMS risk overall. Among non-Hispanic whites, the ORs were 1.33 for high birth weight (95% CI: 0.94-1.89) and 1.17 for LGA (95% CI: 0.78-1.75); no indications of association were observed for other racial or ethnic groups (P interaction <.10). Compared with normal gestational age, preterm (<37 weeks) and post-term (>40 weeks) babies had 16%-18% lower risks of RMS overall, after adjusting for birth weight. CONCLUSIONS: In the largest study to date, there was an indication of association between high birth weight, LGA, and increased RMS risk among non-Hispanic white children and adolescents, but not in other racial or ethnic groups.

[Epidemiological and clinical characteristics of primary ocular cancers in blacks: our experience with 111 cases]. / Aspects épidémiologiques et cliniques des cancers oculaires primitifs du mélanoderme : notre expérience à propos de 111 cas.

INTRODUCTION: In this work, the authors aim to study clinical and epidemiological characteristics of ocular and orbital primary cancers in sub-Saharan African. PATIENTS AND METHODS: This is a retrospective study over a period of 21 years, from 1984 to 2004, including all cases of ocular cancer, histologically proven after surgery of the globe or the orbit. For each patient, we studied the following parameters: age, sex, reason(s) for consultation, the affected eye, and histological result of the operative specimen. These data were collected by studying the departmental surgical registry, patient medical records and the tumor registry of the anatomicopathology laboratory of a tertiary care hospital in sub-Saharan Africa. RESULTS: We collected data on 111 black patients, among whom 15 cases (13.5%) presented with bilateral involvement, for a total of 126 eyes. The sex ratio was 1.17. Presenting signs showed a predominance of leukocoria (30.2%) followed by proptosis (21.7%) and in third place, protruding conjunctival mass (10.8%). Retinoblastoma was found most frequently, representing 66.6% of the oculo-orbital tumors and 95.45% of the tumors of the globe; followed by epidermoid carcinoma, representing 15.08% of cases. Malignant melanoma was third most common, representing 4.76%, with 83% arising in the anterior uvea and 7% in the choroid. Basal cell carcinoma and rhabdomyosarcoma follow in fourth place. Basal cell carcinoma constituted half (50%) of the eyelid tumors. Rhabdomyosarcoma was the most common orbital tumor in our series (57%). Next were lymphomas with conjunctival localization (2.38%), acute leukemia with orbital localization (1.59%) and rare tumors, palpebral dermatofibrosarcoma (0.79%), an orbital angiosarcoma (0.79%), a glioblastoma of the globe (0.79%) and a malignant solitary fibrous tumor of the orbit (0.79%). CONCLUSION: Ocular and orbital primary cancers in blacks remain tumors of the young, equally distributed between the sexes. Retinoblastoma is the most frequent, followed by epidermoid carcinoma. The globe is the preferential localization of these cancers.

Chromosomal and genetic imbalances in Chinese patients with rhabdomyosarcoma detected by high-resolution array comparative genomic hybridization.

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children. Although associations between ARMS tumorigenesis and PAX3, PAX7, and FKHR are well recognized, the complete genetic etiology underlying RMS pathogenesis and progression remains unclear. Chromosomal copy number variations (CNVs) and the involved genes may play important roles in the pathogenesis and progression of human malignancies. Using high-resolution array comparative genomic hybridization (aCGH), we examined 20 formalin-fixed, paraffin-embedded (FFPE) RMS tumors to explore the involvement of the relevant chromosomal regions with resident genes in RMS tumorigenesis. In RMS, frequent gains were identified on chromosome regions 12q13.3-q14.1, 12q24.31, 17q25.1, 1q21.1, and 7q11.23, whereas frequent losses were observed on chromosome regions 5q13.2, 14q32.33, and 15q11.2. Amplifications were observed on chromosome regions 9p13.3, 12q13.3-q14.1, 12q15, and 16p13.11, whereas deletions were detected on chromosome regions 1p36.33, 1p13.1, 2q11.1, 5q13.2, 8p23.1, 9p24.3, and 16p11.2. Frequent gains were detected in GLI1, GEFT, OS9, and CDK4 (12q13.3-q14.1), being 60% in embryonal rhabdomyosarcoma (ERMS) and 66.67% in alveolar rhabdomyosarcoma (ARMS), respectively. However, frequent losses were detected in IGHG1, IGHM, IGHG3, and IGHG4 (14q32.33), being 70% in ERMS and 55.56% in and ARMS, respectively. Frequent gains were detected in TYROBP, HCST, LRFN3, and ALKBH6 (19q13.12) in ERMS but not in ARMS. The frequency of TYROBP, HCST, LRFN3, and ALKBH6 gains is significantly different in ERMS versus ARMS (P=0.011). The results suggest that novel TYROBP, HCST, LRFN3, and ALKBH6 genes may play important roles in ERMS. The technique used is a feasible approach for array comparative genomic hybridization analysis in archival tumor samples.

Lymph node management in patients with paratesticular rhabdomyosarcoma: a population-based analysis.

BACKGROUND: Paratesticular rhabdomyosarcoma (PTRMS) is the most common primary solid tumor arising from the mesenchymal tissue of the testis. Traditionally, retroperitoneal lymph node dissection is not recommended for children aged <10 years because of the morbidity of the procedure and low risk of retroperitoneal lymph node involvement. In the current study, the authors analyzed the patient and tumor characteristics of PTRMS as well as survival outcomes associated with lymph node dissection status. METHODS: A total of 255 cases of PTRMS were identified from the patient data reported by the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute from 1973 through 2009. RESULTS: Among 173 patients aged ≥ 10 years, lymph node dissection was found to improve the 5-year overall survival (OS) rate from 64% to 86% (P < 0.01). Conversely, patients aged <10 years fared extremely well regardless of lymph node dissection status; the 5-year OS rate was 100% and 97%, respectively, for patients who did versus those who did not undergo lymph node dissection (P = .37). The yield of positive lymph nodes was approximately ≥ 20% when < 11 lymph nodes were removed. The incidence of lymph node involvement was also higher in older patients compared with younger patients (40% vs 8%). Radiotherapy improved the OS rate in patients with lymph node involvement (5-year OS rate: 90% with vs 36% without radiation; P < .0001). CONCLUSIONS: Lymph node dissection is recommended in patients aged ≥10 years. Radiotherapy is beneficial in patients with lymph node-positive disease.

Disparities in cancer outcomes: lessons learned from children with cancer.

Disparities in cancer burden by race/ethnicity have been reported, primarily in adults with cancer. However, there appear to be gaps in the pediatric oncology literature with regards to a comprehensive overview on this topic. Extant literature is used to highlight the results of studies focusing on racial and ethnic disparities in outcome observed in selected childhood cancers. A comprehensive approach is utilized to understand possible underlying causes of disparities in cancer outcomes, and to highlight the gaps that currently exist. This review helps define areas of future research that could help develop targeted, disease-specific approaches to eliminate the disparities.

Children from ethnic minorities have benefited equally as other children from contemporary therapy for rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study Group.

PURPOSE: To define the clinical characteristics of rhabdomyosarcoma (RMS) occurring in children from ethnic minorities and determine whether these children have benefited equally from advances in therapy. PATIENTS AND METHODS: This was a retrospective cohort analysis of children treated on the Intergroup Rhabdomyosarcoma Study Group protocols between 1984 and 1997. The clinical features and outcomes of 336 African-American children and 286 children from other ethnic minorities were compared with those of white children (n = 1,721). RESULTS: African-American, other ethnic group, and white children enjoyed similar 5-year failure-free survivals (FFS) of 61%, 61%, and 66%, respectively, P =.15. Compared with white children, nonwhite patients more often had (1) invasive, T2 tumors (P =.03); (2) stage 2 or 3 tumors (P =.003); (3) large tumors (more than 5 cm, P <.006); and/or (4) tumors with positive regional nodes (ie, N1, P =.002). Using Cox proportional hazards analysis, seven patient risk categories were defined with significant differences in outcome. This model was then used to search for other factors associated with FFS after adjusting for these risk categories. Only T stage and age remained associated with FFS (P =.001 and P <.001, respectively). After adjusting for T stage, risk category, and age, we explored the relationship of ethnic group to FFS and found that, compared with whites, the relative risk of failure was 1.14 for African-American patients and 1.2 for other ethnic minority patients, values that are not significantly different. CONCLUSION: Patients from ethnic minority groups more often have larger, invasive tumors with positive lymph nodes. Nevertheless, they have benefited as equally as white children from the dramatic progress in therapy of RMS.