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Objective: Survivors of pediatric head and neck rhabdomyosarcoma (HNRMS) are at risk of developing endocrinopathies following local treatment, resulting from radiation damage to the pituitary gland, hypothalamus, or thyroid gland, often at a young age. Our aim was to determine the prevalence of endocrine dysfunction in long-term HNRMS survivors and compare the prevalence of anterior pituitary insufficiency (API) among different local treatment strategies: external beam radiation with photons, external beam radiation with protons, microscopically radical surgery combined with external irradiation, and macroscopic radical surgery combined with brachytherapy., Design and Methods: Head and neck rhabdomyosarcoma survivors treated between 1993 and 2017, with ≥2 years of follow-up, without recurrent disease or secondary malignancy were eligible for this study. The presence of any endocrine dysfunction was assessed cross-sectionally using Common Terminology Criteria of Adverse Events grading, anthropometrics, and biochemical testing. Retrospective chart review was added to this clinical assessment., Results: Ninety-six survivors with long follow-up time (median, 9 years) were included. Any endocrinopathy was present in 35% of survivors, with 88% having pituitary, 6% peripheral (thyroid), and 6% combined insufficiencies. None had gonadal insufficiency. Growth hormone deficiency was diagnosed in 31 (32%) survivors, with additional pituitary insufficiencies in 12 (39%). In 8%, central precocious puberty preceded API. None of the survivors given brachytherapy had API., Conclusions: The prevalence of pituitary dysfunction in HNRMS survivors is high, emphasizing the importance of systematic endocrine assessment during follow-up, including pubertal development and growth. Efforts should be made to further reduce extraneous irradiation to endocrine organs to prevent dysfunction later in life., Competing Interests: Conflict of interest: None., (© The Author(s) 2025. Published by Oxford University Press on behalf of European Society of Endocrinology.)

Background: We examined the association between late-stage diagnosis and individual- and community-level sociodemographic and socioeconomic characteristics among patients with pediatric Hodgkin lymphoma and rhabdomyosarcoma (RMS)., Methods: We obtained Children's Oncology Group data from 1999 to 2021 including summary stage [local (L), regional (R), and distant (D)], tumor subtype, demographics, and ZIP Code at diagnosis. We linked ZIP Codes to county-level redlining scores (C, D = greatest redlining), the Child Opportunity Index, and measures of segregation (racial dissimilarity indices). Logistic regressions calculated odds ratios for late-stage diagnosis and by race within tumor subtype., Results: In total, 5,956 patients with Hodgkin lymphoma and 2,800 patients with RMS were included. Late-stage diagnosis of Hodgkin lymphoma was correlated with Black race [ORDistant(D) vs. regional/local (R&L) = 1.38 (1.13-1.68)], being uninsured [ORD vs. R&L = 1.38 (1.09-1.75)], and subtype [nodular sclerosis vs. Other Hodgkin lymphoma: ORD vs. R&L = 1.64 (1.34-2.01), Untyped: ORD vs. R&L = 1.30 (1.04-1.63)]. Late-stage RMS was correlated with bilingual households [ORDistant/regional(D&R) vs. local(L) = 2.66 (1.03-6.91)] and tumor type [alveolar vs. embryonal ORD vs. R&L = 6.16 (5.00-7.58)]. Community-level factors associated with late-stage Hodgkin lymphoma were greater Black (OR80-100% = 1.83; 95% CI = 1.11-3.02) and Hispanic (OR60-79% = 1.30; 95% CI = 1.05-1.60) dissimilarity indices. Late-stage diagnosis for RMS was associated with more redlined census tracts within counties (OR = 1.54; 95% CI = 1.02-2.35) and low/very low Child Opportunity Index (OR = 1.21; 95% CI = 1.02-1.45)., Conclusions: Novel markers of community deprivation, such as redlining and racial segregation, were correlated with cancer outcomes for children with Hodgkin lymphoma and RMS in this first disparities study using Children's Oncology Group registries., Impact: The interplay of multilevel risk factors provides important consideration for efforts to improve early detection of pediatric cancer diagnosis., (©2024 American Association for Cancer Research.)

Background: Orbital rhabdomyosarcoma (RMS), the most common primary malignant orbital tumor in childhood, presents unique challenges in management due to its genetic basis and abnormal cellular proliferation. Management has evolved from surgical excision to multimodal approaches, including surgery, radiotherapy, and chemotherapy. This review explores trends in epidemiology, pathophysiological insights, and treatment evolution to delineate optimal therapeutic strategies., Methods: A literature search was conducted across PubMed and Scopus databases up to March 4, 2024, using keywords related to orbital RMS. Studies were selected based on predefined criteria, focusing on clinical presentation, diagnostic modalities, management strategies, and outcomes. Data extraction and analysis were performed independently by 2 reviewers, with quality assessment based on the Oxford Center for Evidence-Based Medicine 2011 guidelines and Joanna Briggs Institute checklists., Results: The review synthesized data from 17 studies, highlighting demographic patterns, clinical characteristics, staging, and treatment approaches. Most patients were male and white, with a higher proportion of early-stages diagnoses. The embryonal subtype was the most common, and treatment has shifted toward radiation therapy and combined modalities. In orbital RMS, several modalities are applied in treatment. There are radiotherapy, surgical interventions, and chemotherapy. Recent studies put more emphasis on the long-term outcome of the disease about orbital bone development and facial symmetry., Conclusions: Management of orbital RMS has evolved significantly, with a current emphasis on multimodal treatment strategies. Future research should focus on optimizing therapeutic approaches to balance effective tumor control with the preservation of orbital function and esthetics., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Soft tissue sarcomas (STS) represent a heterogeneous group of extraskeletal mesenchymal tumors that affect individuals throughout the entire age continuum. Despite this pervasive influence, key differences exist in the presentation of these sarcomas across varying age groups that have prevented a more uniform approach to management. Notably, rhabdomyosarcoma (RMS) is more common in children, while most nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) subtypes are more prevalent in adults. Older patients with NRSTS appear to have more molecularly complex biology and often present with more advanced disease compared with children. Poorer outcome disparities are observed in older patients with RMS despite receiving similar treatment as younger patients. In this review, we highlight differences in epidemiology, biology, and management paradigms for pediatric and adult patients with STS and explore opportunities for a unified approach to enhance the care and outcomes within the AYA population.

Purpose: To study the epidemiological profile of various pediatric orbital tumors and determine their clinicopathological correlation over 20 years., Methods: A retrospective analysis of all pediatric patients with orbital tumors from the medical records between 1995 and 2015 was done. Tumors were categorized based on their cellular origin. Demographic details, clinical and histopathological diagnoses, frequency, and clinicopathological correlation were studied., Results: A total of 265 pediatric patients with orbital tumors were analyzed, of which 145 (54.7%) patients were males and 120 (45.3%) were females. The mean age was 7 years (median: 6 years; range: 10 days-16 years). The distribution of lesions in each group was as follows: vasculogenic, 76 (28.7%); lipogenic and myxoid, 66 (25%); myogenic, 31 (11.7%); optic nerve and meningeal, 22 (8.3%); lymphoid and lymphoproliferative, 18 (6.8%); secondary orbital tumors, 14 (5.2%); histiocytic, 9 (3.4%); miscellaneous, 9 (3.4%), lacrimal gland, 8 (3%); mesenchymal with neural differentiation, 6 (2%); osseous, 4 (1.5%), and fibrocystic, 2 (0.8%). The most common benign and malignant pediatric orbital tumors were dermoid cysts (65, 24.5%) and rhabdomyosarcoma (31, 11.7%). A statistically significant ( P < 0.0001) clinicopathological correlation was observed in 208 (83.2%) out of 250 biopsy-proven cases., Conclusion: Pediatric orbital neoplasms include a broad spectrum of benign and malignant lesions. Vasculogenic tumors constitute the majority of them, followed by lipogenic and myxoid tumors. A thorough knowledge of the incidence, clinical profile, imaging features, and histopathology of specific orbital tumors aids in accurate diagnosis and their successful management., (Copyright © 2023 Copyright: © 2023 Indian Journal of Ophthalmology.)

Several cancer predisposition syndromes (CPS) are reported to predispose to rhabdomyosarcoma, most frequently in children with embryonal rhabdomyosarcoma. There are lingering questions over the role of CPS in individuals with alveolar rhabdomyosarcoma (ARMS), which are frequently driven by FOXO1 fusion oncoproteins. We conducted a systematic review to identify patients with FOXO1 fusion-positive ARMS (FP-ARMS) who underwent germline DNA sequencing. We estimated the prevalence of pathogenic/likely pathogenic (P/LP) variants in cancer predisposing genes (CPGs) and of CPSs. We included 19 publications reporting on 191 patients with FP-ARMS. P/LP variants in CPGs were identified in 26/191 (13.6%) patients, nine (4.9%) of which were associated with a CPS diagnosis. Evidence for causal associations between CPSs and FP-ARMS could not be assessed with available data from this review. Only one patient was affected with a CPS known to predispose to rhabdomyosarcoma, Li-Fraumeni syndrome. Typical CPS associations with rhabdomyosarcoma are rare, but not nonexistent, in patients with FP-ARMS. FOXO1 fusion status, alone, is insufficient for clinicians to rely on to distinguish between patients with/without CPS., (© 2023 Wiley Periodicals LLC.)

This report describes the results of an observational study dedicated to rhabdomyosarcoma developed by the Asociación de Hemato-oncología Pediatrica de Centro América (AHOPCA) between 2001 and 2018. Overall, 337 previously untreated patients < 18 years old were included in the analysis; 58% had unresected disease, and 19% were metastatic at diagnosis. With a median follow-up of 6.6 years, five-year event-free and overall survival rates were 30% and 33%, respectively. Local progression/relapse was the main cause of treatment failure., (© 2022 Wiley Periodicals LLC.)

Purpose: Malignant tumours of the vagina are very rare in children. The purpose of this study was to retrospectively analyse the clinical presentation, treatment, and outcome of vaginal tumours in children treated in a single institution., Methods: This study retrospectively analysed the clinical data of children diagnosed with vaginal malignant tumours who were admitted to the Beijing Children's Hospital of Capital Medical University from January 2007 to December 2020 and followed these patients to observe their prognoses and outcomes., Results: During 13 years, a total of 33 children were included in this study, including 13 children with rhabdomyosarcoma and 20 children with endodermal sinus tumours. The average age at diagnosis was 20.4 months. The main clinical manifestations were vaginal bleeding and protruding masses. Of the 13 children with vaginal rhabdomyosarcoma, 12 were treated with multidrug chemotherapy combined with conservative tumour resection, and their tumours completely resolved; only one child underwent vaginectomy and hysterectomy. Twenty children with vaginal endodermal sinus received PEB chemotherapy. Among these patients, the tumour disappeared after chemotherapy in 12 children, and the remaining nodular tumour foci in 8 children were confirmed to be necrotic tissue by pathology., Conclusion: Our research confirms that chemotherapy combined with conservative surgical treatment is effective for treating children with vaginal malignancies., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Pediatric head and neck cancer (PHNC) is rare and its nonspecific clinical manifestations may often lead to delayed diagnosis. We aimed to describe the signs, symptoms, and clinicopathological characteristics of PHNC., Material and Methods: Medical records were retrospectively reviewed for all PHNC cases diagnosed from 1986 to 2016 affecting patients aged 19-years and younger from a tertiary referral center in Brazil. Demographic variables, anatomical site of primary tumors, histopathological diagnoses, signs and symptoms, and patterns of misdiagnosis were collected and interpreted by statistical and descriptive analysis., Results: A total of 253 PHNC cases were included. The mean age was 9.3 years and male patients were more frequently affected (60.9%). Burkitt lymphoma (23.7%), nasopharyngeal carcinoma (15.8%), and rhabdomyosarcoma (15.4%) were the most common cancer types. The nasopharynx (28.9%), cervical/lymph node region (25.3%), and craniofacial bones (8.3%) were the predominant anatomical sites. Tumor/swelling (68.4%), was the clinical finding often presented. The univariable analysis showed association between tumor histology and clinical variables such as sex (p=0.022), age (p<0.0001), anatomical location (p<0.0001) tumor/swelling (p=0.034), pain (p=0.031), systemic/general manifestations (p=0.004), nasal/breathing alterations (p=0.012), orbital/ocular alterations (p<0.0001). Misdiagnosis such as tonsillitis, otitis, and abscess were frequent., Conclusions: Although the clinical findings of PHNC are often unspecific, this study provided signs and symptoms with significant correlations between tumor histology. The suspicion of malignancy should be considered when the main signs and symptoms reported here appear and persist, in order to conduct a timely diagnosis.

Background: Delay in diagnosis and treatment initiation can be associated with adverse outcomes in children with cancer. Diagnostic interval (DI) is defined as the time between the date of first health care contact for symptoms related to cancer to the date of cancer diagnosis, and treatment interval (TI) is defined as interval between the definitive cancer diagnosis and cancer treatment initiation. We aimed to determine the predictors of DI and TI in children with rhabdomyosarcoma (RMS) and their association with event-free survival (EFS) and overall survival (OS)., Methods: Using the Cancer in Young People in Canada (CYP-C) national population-based database, we conducted a retrospective cohort study of children (0-14.99 years) newly diagnosed with RMS between 2001 and 2015 in Canada. Quantile regression was used to assess the predictors of DI and TI, and Cox regression was used to determine if these intervals were associated with EFS and OS., Results: Median DI and TI were 16.5 days (interquartile range [IQR] 6.0-38.0) and 5 days (IQR 0-12), respectively. DI and TI were not significantly associated with age at diagnosis, sex, race, tumor site, stage or histology, treatment region, distance from treatment center, income quintile or diagnosis year (all p > .05). DI and TI were not associated with EFS (DI: hazard ratio [HR] 1.00, 95% CI 0.96-1.05, p = .871; TI: HR 1.03, 95% CI 1.00-1.05, p = .053) or OS (DI: HR 0.99, 95% CI 0.94-1.05, p = .797; TI: HR 1.02, 95% CI 0.99-1.05, p = .155)., Conclusions: In the publicly funded Canadian health care system, DI and TI did not affect the survival of children with RMS., (© 2021 Wiley Periodicals LLC.)

Therapy-related myeloid neoplasm (t-MN) in the pediatric population is not well characterized. We studied 12 pediatric patients diagnosed with t-MN in our institution since 2006. The median age at the t-MN diagnoses was 14.8 years (range, 9 to 20 y). The primary malignancies included 9 solid tumors and 3 hematopoietic malignancies. Rhabdomyosarcoma (n=4) was the most common primary malignancy. Five of the 9 patients with solid tumors and all 3 patients with hematopoietic malignancies had primary neoplasms involving bone marrow. The median latency period was 5.2 years (range, 1.8 to 13.8 y). Thrombocytopenia was present in all patients at the t-MN diagnoses. Complete or partial monosomy of chromosome 5 or 7 were the 2 most common cytogenetic abnormalities. A quarter of patients demonstrated a genetic predisposition to t-MN: 1 with Li-Fraumeni syndrome with a germline TP53 R248Q mutation, 1 with Noonan syndrome with a somatic mutation (PTPN11 S502T), and 1 with a constitutive chromosomal translocation [t(X;9)(p22;q34)] and a germline TP53 L130V mutation. Outcomes remain poor. Two patients survived 3 and 5.1 years after hematopoietic stem cell transplantation., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm., (© 2021. Federación de Sociedades Españolas de Oncología (FESEO).)

Purpose: Survival of children with rhabdomyosarcoma that suffer from recurrent or progressive disease is poor. Identifying these patients upfront remains challenging, indicating a need for improvement of risk stratification. Detection of tumor-derived mRNA in bone marrow (BM) and peripheral blood (PB) using reverse-transcriptase qPCR (RT-qPCR) is a more sensitive method to detect disseminated disease. We identified a panel of genes to optimize risk stratification by RT-qPCR., Experimental Design: Candidate genes were selected using gene expression data from rhabdomyosarcoma and healthy hematologic tissues, and a multiplexed RT-qPCR was developed. Significance of molecular disease was determined in a cohort of 99 Dutch patients with rhabdomyosarcoma (72 localized and 27 metastasized) treated according to the European pediatric Soft tissue sarcoma Study Group (EpSSG) RMS2005 protocol., Results: We identified the following 11 rhabdomyosarcoma markers: ZIC1, ACTC1, MEGF10, PDLIM3, SNAI2, CDH11, TMEM47, MYOD1, MYOG , and PAX3/7-FOXO1 . RT-qPCR was performed for this 11-marker panel on BM and PB samples from the patient cohort. Five-year event-free survival (EFS) was 35.5% [95% confidence interval (CI), 17.5%-53.5%] for the 33/99 RNA-positive patients, versus 88.0% (95% CI, 78.9%-97.2%) for the 66/99 RNA-negative patients ( P < 0.0001). Five-year overall survival (OS) was 54.8% (95% CI, 36.2%-73.4%) and 93.7% (95% CI, 86.6%-100.0%), respectively ( P < 0.0001). RNA panel positivity was negatively associated with EFS (Hazard Ratio = 9.52; 95% CI, 3.23-28.02), whereas the RMS2005 risk group stratification was not, in the multivariate Cox regression model., Conclusions: This study shows a strong association between PCR-based detection of disseminated disease at diagnosis with clinical outcome in pediatric patients with rhabdomyosarcoma, also compared with conventional risk stratification. This warrants further validation in prospective trials as additional technique for risk stratification., (©2021 The Authors; Published by the American Association for Cancer Research.)

The objectives of this study were to describe the clinicopathologic features and treatment outcomes of childhood rhabdomyosarcoma in a resource-constrained setting. All cases of childhood rhabdomyosarcoma seen over a 10-year period (July 2006 to June 2016) at the University College Hospital, Ibadan, Nigeria were reviewed. Data were extracted from the database of the pediatric Hematology/Oncology Unit of the hospital and analyzed. Ethical approval was obtained from the Institutional Ethics Committee. Fifty children were seen comprising 30 men and 20 women with bimodal ages of 4 and 5 years. Median duration of illness was 16 weeks and the most common primary tumor site was the head-and-neck region in 27 (54%) of cases. The histologic subtypes were embryonal in 30 (60%), alveolar in 9 (18%), and not specified in 11 (22%). The Intergroup Rhabdomyosarcoma Study group TNM Pretreatment stages were stage I in 15 (30%), stage III in 17 (34%), and stage IV in 18 (36%). Treatment included chemotherapy, surgery, and radiotherapy and abandoned in 20 (40%) cases. Median survival was 45 weeks (95% confidence interval: 16.4-73.6) and 5 (10%) patients were alive and disease free, 4 years or more after diagnosis. Outcome of childhood rhabdomyosarcoma is poor and early diagnosis and improved access to treatment are recommended., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Rhabdomyosarcoma (RMS) of the female genitourinary tract (FGU-RMS) located at the vagina or uterus is one of the most favorable RMS sites. Little is known about treatment and outcome in infants and relapsed disease (RD)., Methods: Characteristics, treatment, and outcome of 71 children with FGU-RMS registered within five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry (1981-2019) were evaluated., Results: FGU-RMS was diagnosed in 67 patients with localized disease (LD) at a median age of 2.89 years (0.09-18.08). Multimodal treatment consisted of chemotherapy (CHT) (n = 66), secondary surgery (n = 32), and radiotherapy (n = 11). Age at diagnosis ≤12 months was the only significant negative prognostic factor influencing the event-free survival (EFS). Ten-year EFS and overall survival (OS) for infants ≤12 months were 50% and 81%, respectively. In contrast, children with LD >1 year and ≤10 years had a 10-year EFS and OS of 78% and 94% (P = .038), and >10 years of 82% and 88%, respectively (P = .53). Metastatic disease was observed in four patients of which three are alive. RD occurred in five of 12 infants ≤1 year and 10/55 children at a median of 1.38 years (0.53-2.97) after initial diagnosis. Treatment of patients with RD consisted of multimodal treatment (n = 13) or resection only (n = 2). Nine patients (60%) were alive in clinical remission at a median of 7.02 years (1.23-16.72) after diagnosis of RD., Conclusion: Infants with FGU-RMS have a higher relapse rate than older children with FGU-RMS, but prognosis is fair., (© 2021 Wiley Periodicals LLC.)

Objectives: Clinical trials have reported increases in the survival of pediatric rhabdomyosarcoma (RMS) from 25% in 1970 to 73% in 2001. The purpose of this study was to examine whether survival of pediatric patients with RMS of the head and neck improved at the US population level., Methods: A population-based cohort of patients with rhabdomyosarcoma of the head and neck aged 0-19 years in the Surveillance, Epidemiology, and End Results (SEER) registry from 1973 to 2013 was queried. The cumulative incidence competing risks (CICR) method was used to estimate risk and survival trends., Results: 718 cases were identified for analysis. Survival rates at 1-, 5-, and 10-years after diagnosis were 91.2%, 73.2%, and 69.4% respectively. Survival rates at 1 year after diagnosis increased from 82.6% to 93.1% during the study period. In the subdistributional hazard analysis, there was a significantly improved disease-specific risk of death in the first year after diagnosis. Overall risk of death did not improve significantly. Favorable prognostic factors included age <10 years at diagnosis, smaller tumor size, absence of distant metastasis, localized tumors, earlier stage at presentation, grossly complete surgical resection, and embryonal or botryoid histology., Conclusions: Disease-specific survival in the first year following diagnosis improved, but the change in overall survival at the population level was not statistically significant. These findings should be interpreted in light of the inclusion of patients with distant metastasis at diagnosis, who have poor prognoses, together with the limited statistical power afforded in studies of rare diseases., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Purpose: The aim of this study was to better understand the incidence and the clinical characteristics of cardiac radiation-induced sarcomas (RIS)., Material and Methods: We used the surveillance, epidemiology, and end results (SEER) program cancer registry data, the largest cancer database in the United States in order to identify all cardiac RIS between 1973 and 2015. We relied on the Memorial Sloan-Kettering Cancer Center (MSKCC)-modified 1948 Cahan criterions for RIS identification., Results: Out of 8,136,951 cancer patients from the SEER database, we identified 448 patients diagnosed with cardiac sarcomas. Of these 448 cardiac sarcoma patients, two were considered to have developed a cardiac RIS: a metastatic rhabdomyosarcoma occurring after one to two years following lung carcinoma irradiation, and a soft tissue sarcoma (of unspecified type) developed six years after radiation therapy for an aggressive left-sided breast carcinoma. Based on this observation, we estimated that cardiac RIS represented about 0.4% (95% CI 0.1%-1.6%) of all cardiac sarcomas. A literature review has been conducted and yielded three additional cases of cardiac RIS., Conclusion: Cardiac RIS are extremely rare malignancies, associated with a very pejorative prognosis. The two reported histologies are angiosarcomas and rhabdomyosarcomas, which might be over-represented among cardiac RIS. A metastatic evolution is possible for cardiac radiation-induced rhabdomyosarcomas. Surgical excision, when feasible, is a therapeutic option and is the only specific treatment reported to this date., (Copyright © 2020 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Objective: Paratesticular tumors (PTT) are rare and form a heterogenous group, ranging from benign to malignant high grade sarcomas. This study was undertaken to describe the clinicopathological spectrum of PTTs received over a 20-year period., Methods: All primary and secondary PTTs diagnosed from 2000 to 2019 in the pathology department of a tertiary care hospital in North India were retrospectively reviewed. Gross, histopathological features and immunohistochemistry (IHC) findings were correlated with clinical details., Results: A total of 169 intra-scrotal tumors were diagnosed during the study period, out of which there were 30 PTTs (in 27 patients) comprising 17.75%. Age range was 4 to 85 years (median 58 years). Benign PTTs were the commonest (n = 21, 70%), followed by metastasis to the paratesticular region (n = 6, 20%) and then primary malignant PTTs (n = 3, 10%). The commonest benign PTT was lipoma (n = 16, 76.19%), followed by adenomatoid tumor (n = 3, 14.28%) with one case each (4.76%) of cellular angiofibroma and hemangioma. Among primary malignant PTT, there were two cases of rhabdomyosarcoma, and one case of biphasic malignant mesothelioma. Metastatic tumors included four cases of prostatic adenocarcinoma, and one case each of pancreatic signet ring cell carcinoma and clear cell renal cell carcinoma., Conclusion: PTTs show a wide clinicopathological spectrum. Benign PTTs are commoner than malignant PTTs. Meticulous grossing and histopathological examination supplemented by IHC is essential for an accurate diagnosis of this heterogenous class of tumors, which influences the role of adjuvant therapy and patient prognosis., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Increasing evidence suggests that Duchenne muscular dystrophy (DMD) gene is involved in the occurrence of different types of cancer. Moreover, development of sarcomas was reported in mdx mice, the murine model of DMD, in older age. So far, nine isolated DMD patients were reported with concomitant cancer, four of whom with rhabdomyosarcoma (RMS), but no systematic investigation was performed about the true incidence of cancer in DMD., Methods: All members of the Italian Association of Myology were asked about the occurrence of cancer in their DMD patients in the last 30 years., Results: Four DMD patients with cancer were reported after checking 2455 medical records. One developed brain tumour at the age of 35 years. Two patients had alveolar RMS at 14 and 17 years of age. The fourth patient had a benign enchondroma when 11-year-old., Conclusion: Prevalence of cancer in general in the Italian DMD patients does not seem to be different from that in the general population with the same age range. Although the small numbers herein presented do not allow definitive conclusion, the frequent occurrence of RMS in DMD patients raises an alert for basic researchers and clinicians. The role of DMD gene in cancer merits further investigations.

Introduction: Rhabdomyosarcoma (RMS) is the most common soft tissue head and neck sarcoma in children. Stringent analysis of survival data is imperative to optimize treatment., Methods: The National Cancer Database (NCDB, 2004-2016) and the Surveillance, Epidemiology, and End Results Program (SEER, 1975-2016) were queried for patients ages 0-19 with RMS of the head and neck. Survival trends were analyzed using univariable logistic regression and Chi-square pairwise comparisons. Survival by treatment was analyzed using log-rank tests, Kaplan Meier, and multivariable Cox-proportional hazards regression., Results: 1147 (63.3% age <10 years, 54.3% male) and 459 (71.4% age <10 years, 53.6% male) patients were identified in NCDB and SEER, respectively. In NCDB, embryonal (n = 625, 54.5%) and alveolar (n = 300, 26.2%) were the most common histology and nonparameningeal/non-orbital (n = 634, 55.3%), followed by parameningeal (n = 303, 26.4%) and orbital (n = 210, 18.3%) the most common location. Five-year overall survival (OS) was 70.3% with lower mortality risk for embryonal histology (adjusted HR [aHR] = 0.69, p = 0.0038). Orbital tumors had greatest survival (5-year OS = 92.4%) of all sites, and their mortality risk was higher with chemotherapy and radiation compared to surgery and radiation (aHR = 6.27, p = 0.0302). SEER analysis showed average increased survival by 4% per year (p < 0.0001), but no significant 5-year OS difference when comparing 1976-1980 and 2006-2010 (p = 0.0843)., Conclusions: Major prognostic factors for survival in childhood RMS of the head and neck were embryonal histology, orbital site, extent of disease, and use of SRT for orbital tumors. Larger population studies are required to demonstrate survival differences between treatment modalities for other sites., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Approximately 50% of children with cancer in the United States who are aged <15 years receive primary treatment on a therapeutic clinical trial. To the authors' knowledge, it remains unknown whether trial enrollment has a clinical benefit compared with the best alternative standard therapy and/or off trial (ie, clinical trial effect). The authors conducted a retrospective matched cohort study to compare the morbidity and mortality of pediatric patients with cancer who are treated on a phase 3 clinical trial compared with those receiving standard therapy and/or off trial., Methods: Subjects were aged birth to 19 years; were diagnosed between 2000 and 2010 with acute lymphocytic leukemia (ALL), acute myeloid leukemia (AML), rhabdomyosarcoma, or neuroblastoma; and had received initial treatment at the Children's Hospital of Philadelphia. On-trial and off-trial subjects were matched based on age, race, ethnicity, a diagnosis of Down syndrome (for patients with ALL or AML), prognostic risk level, date of diagnosis, and tumor type., Results: A total of 428 participants were matched in 214 pairs (152 pairs for ALL, 24 pairs for AML, 32 pairs for rhabdomyosarcoma, and 6 pairs for neuroblastoma). The 5-year survival rate did not differ between those treated on trial versus those treated with standard therapy and/or off trial (86.9% vs 82.2%; P = .093). On-trial patients had a 32% lower odds of having worse (higher) mortality-morbidity composite scores, although this did not reach statistical significance (odds ratio, 0.68; 95% confidence interval, 0.45-1.03 [P = .070])., Conclusions: There was no statistically significant difference in outcomes noted between those patients treated on trial and those treated with standard therapy and/or off trial. However, in partial support of the clinical trial effect, the results of the current study indicate a trend toward more favorable outcomes in children treated on trial compared with those treated with standard therapy and/or off trial. These findings can support decision making regarding enrollment in pediatric phase 3 clinical trials., (© 2020 American Cancer Society.)

TP53 p.R337H germline mutation is highly prevalent in the Southern region of Brazil. We sought to investigate TP53 p.R337H mutation in pediatric tumor samples from a population settled in a geographic area of high prevalence for this variant. Mutation assessment and genetic counseling for carriers/relatives were provided. 6/57 tumor samples were heterozygous for TP53 p.R337H. As expected, a high frequency was observed within adrenocortical tumors (3/3) and choroid plexus carcinomas (2/2). Interestingly, the TP53 R337H mutation was found in one case of pediatric rhabdomyosarcoma with Li-Fraumeni pedigree. Our finding expands the spectrum of childhood cancer associated with this germline mutation.

Objective: To describe different types of primary extraocular muscle (EOM) tumours based on the results of imaging studies, peroperative clinical picture and their histopathological diagnosis., Study Design: Case series., Place and Duration of Study: Al-Shifa Trust Eye Hospital, Rawalpindi, from July 2001 to January 2017., Methodology: A retrospective analysis of 640 diagnosed orbital tumours was carried out using non-randomised sampling technique, and the prevalence of primary EOM tumours was determined. Based on the results of imaging studies, the clinical picture observed during surgery (orbitotomy) and the histopathological diagnosis, primary EOM tumours were divided into different types, accordingly., Results: Nineteen (n=19) primary EOM tumours (frequency of 2.96%) had 12 types of histopathological diagnoses, and were categorised into inflammatory tumours (n=8, 42%), vascular tumours (n=4, 21%), lymphoproliferative tumours (n=3, 16%), neurogenic tumours (n=2, 10.5%) and myogenic tumours (n=2, 10.5%). The recti were involved more frequently than obliques (n=15, 78.94% and n=4, 21.06%, respectively). All the patients presented with proptosis of varying degree with some degree of globe rotation and had surgical excision/appropriate management. Visual acuity was not affected in any of the patients. Four (n=4, 21.05%) tumours were malignant (NHL, ASPS, myeloid sarcoma and rhabdomyosarcoma) and these patients underwent chemotherapy and/or radiotherapy., Conclusion: Biopsy-proven primary EOM tumours were devisable into five broad categories. Patients with primary EOM tumours presented with proptosis and impaired ocular motiliy. The primary EOM tumours involved both the recti and the obliques and were excised surgically with favourable outcomes in most cases.

Background: Malignant orbito-ocular tumors are major causes of morbidity and mortality in the developing countries. Their presentation, morphologic type, and management are challenging, and earlier reports were mostly retrospective surveys. Histological analysis is essential in making correct diagnosis. Here, we determined the clinical presentations and histopathologic types of orbito-ocular tumors in some tertiary hospitals in Kano, Nigeria., Materials and Methods: This was a prospective cross-sectional multicenter clinical and pathologic study conducted between June 2012 and May 2013. A structured pro forma was used to record findings, patient's biodata, clinical presentation, type of surgical intervention, and histological diagnosis. Data were analyzed with STATA version 11.0., Results: Sixty-seven eye tumors were observed among 61 patients. There were 43 males and 18 females (male-to-female ratio = 2.4:1). Patients had a mean of 13.4 ± 18.3 with a median of 4.0 (0.5-70) years with a double peak in age distribution. The most common features were visual loss (90.2%), leukocoria (59%), proptosis (55%), and fungating mass (49.2%). The symptoms were for ≥6 months in 57.4% and unilateral in 82.1%. B-scan showed retinal masses and calcifications in 13.5% and 4.5%, respectively. Confirmation by histology was achieved in 91% with the most common tumor being retinoblastoma, conjunctival squamous cell carcinoma (SCC), and rhabdomyosarcoma with frequencies of 38 (56.6%), 11 (16.4%), and 6 (9.0%), respectively. Six patients were HIV positive and all had SCC., Conclusions: Retinoblastoma was the most common malignant orbito-ocular tumor observed in this study. Patients often present late to the hospital with visual loss., Competing Interests: None

Orbital lesions vary in their classification, incidence, and presentation depending on the age and geographic distribution. Such lesions in the pediatric age group have been studied extensively because of the possibility of faster progression of orbital involvement and the higher risk of morbidity in this age group in which vision is still developing. In Saudi Arabia, orbital lesions were studied over a 6-year period in the late 1980s, when retinoblastoma cases used to present late with orbital involvement. In this study, we revisited the same topic 20 years later aiming to find out the most recent prevalence of orbital lesions in a similar population of patients over a longer period (14 years) in the same eye center, and compare the current results to other reports worldwide. A total of 107 lesions from 106 patients were identified by tissue diagnosis, of which more than half of the lesions were benign cystic (being the most common), vasculogenic, and inflammatory in 63% [95% confidence interval (CI, 53.3-72.0)] of all biopsied lesions. Neoplasms accounted for 37% [95% CI (28.0-45.8)] with rhabdomyosarcoma being the most common, accounting for about one third of neoplasms, and no orbital cases of retinoblastoma were found. Our results demonstrated different distribution of orbital lesions in recent years reflecting the indirect effect of the improved health awareness and medical care in Saudi Arabia. This baseline demographic study is expected to be helpful for further clinical and prognostic studies with emphasis on pediatric orbital malignant lesions, their clinical presentation, management, and prognosis., Competing Interests: The authors have no conflict of interest in relation to this manuscript., (© Atlantis Press International B.V.)

Background: Uterine sarcomas consist a heterogeneous group of mesenchymal gynecological malignancies with unclear therapeutic recommendations and unspecific but poor prognosis, since they usually metastasize and tend to recur very often, even in early stages., Methods: We retrospectively analyzed all female patients with uterine sarcomas treated in our institution over the last 17 years. Clinico-pathological data, treatments and outcomes were recorded. Kaplan-Meier curves were plotted and time-to-event analyses were estimated using Cox regression., Results: Data were retrieved from 61 women with a median age of 53 (range: 27-78) years, at diagnosis. Fifty-one patients were diagnosed with leiomyosarcoma (LMS), 3 with high grade endometrial stromal sarcoma (ESS), 5 with undifferentiated uterine sarcoma (UUS), 1 with Ewing sarcoma (ES) and 1 with Rhabdomyosarcoma (RS). 24 cases had stage I, 7 stage II, 14 stage III and 16 stage IV disease. Median disease-free survival (DFS) in adjuvant approach was 18.83 months, and median overall survival (OS) 31.07 months. High mitotic count (> 15 mitoses) was significantly associated with worse OS (P < 0.001) and worse DFS (P = 0.028)., Conclusions: Mitotic count appears to be independent prognostic factor while further insights are needed to improve adjuvant and palliative treatment of uterine sarcomas.

Objective: To examine the types of pediatric middle ear tumors and review the demographics, management, and survival of pediatric patients with rhabdomyosarcoma (RMS) of the middle ear., Methods: Pediatric patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2014 based on a diagnosis of middle ear tumors using the ICD O-3 code: C30.1: Middle ear primary site. Patients were included from ages 0-18 years., Results: Forty pediatric middle ear tumor cases were identified. Twenty patients were female (50%). Twenty-seven (67.5%) cases were rhabdomyosarcomas (RMS). Pediatric RMS patients tended to be diagnosed in early childhood (mean age 5.30 years, standard deviation 2.9, range 1.00-13.00, 59.3% of patients were ages 5 or below). Most pediatric RMS patients received chemotherapy and radiation therapy as part of the treatment regimen (88.8%). Finally, the 5-year overall and disease-specific survival rates were 59% and 63% respectively., Conclusions: Pediatric middle ear tumors are rare. Females and male pediatric patients are both at risk for middle ear tumors. RMS is the most common malignant middle ear tumor affecting pediatric patients. Despite the use of multimodality therapies, survival rates for pediatric patients with RMS of the middle ear are low. Physicians may consider including middle ear tumors on the differential diagnosis for pediatric patients with symptoms presenting similarly to non-resolving otitis media., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: The designation Rabdomyosarcoma (RMS) is used to describe the malignant mesenchymal neoplasm that exhibits varying degree of skeletal muscle differentiation. RMS is the commonest malignancy of soft tissues afflicting children and adolescent up until age 20. It constitutes 5-10% of pediatric malignancies, and 7-9.5% of pediatric soft tissue sarcomas. The site of origin of RMS can be one of three possibilities: myogenous; extramyogenous; and ectopic (heterotopia). This was a retrospective study of consecutive cases of RMS seen at the Histopathology Department of the Jos University Teaching Hospital, Jos, between January 2007 and December 2016. The study was aimed at analyzing RMS in relation to age, sex, site, and histological type., Methodology: Patients' information including: age; sex; anatomical site of lesion; and histopathological diagnosis were extracted from patients' records in the Medical Records Department and the Histopathology Department. Archival tissue blocks were retrieved, sectioned into 5mm slides, stained with haematoxylin and eosin, and reviewed microscopically to confirm the diagnosis of RMS and sub-classify the disease. All histologically confirmed cases were included in the study, while those with inadequate bio-data and record of topography were excluded., Results: Seventy (70) cases of RMS were seen during the study period. The age range was 3 months to 80 years. The mean age at diagnosis was 23.4±21.1 years. There were 40 (57.4%) males and 30 (42.9%) females with male female ratio of 1.3:1. The head and neck region was the commonest site of occurrence of RMS accounting for 27.1%., Conclusion: The commonest histologic variants of rhabdomyosarcoma were the embryonal, alveolar, and pleomorphic types. More than half of this malignancy occurred in children, and the adolescent. The head and neck, extremities, and trunk in decreasing order were the commonest sites of occurrence of this tumor, with a male predominance.

Benign, intermediate and malignant soft tissue tumors can be differentiated histologically. Furthermore, the tumors can be subdivided according to their linear differentiation. In the new World Health Organization (WHO) classification of soft tissue tumors from 2013 changes have been made relating to the allocation of known entities, e. g. undifferentiated sarcomas have been formed into a new subgroup and are no longer assigned to the fibrohistiocytic tumors. The term malignant fibrous histiocytoma has been replaced by the undifferentiated sarcoma. Furthermore, two new subgroups were incorporated, the nerve sheath tumors and gastrointestinal stromal tumors. These were previously included in the tumor classification of other organ systems. These changes in the new classification are related to the rapid increase in knowledge of the genetics and the cell biology of soft tissue tumors. Malignant soft tissue tumors only represent 1% of all malignant tumors in adults. The largest subgroup of soft tissue tumors in adults is the adipocytic tumors. The liposarcoma, which belongs to this subgroup is one of the most common malignant soft tissue tumors in adults. In childhood malignant soft tissue tumors represent 15% of malignant tumors and rhabdomyosarcoma is the most common malignant soft tissue tumor.

Objective: Analyze the profile and outcome of children with rhabdomyosarcoma from a pediatric-oncology unit., Design: Retrospective analysis of case records over 23 years (1990-2012)., Setting: Government-run, tertiary-care, university hospital in Northern India., Participants: 159 children (<12-years) with a diagnosis of rhabdomyosarcoma were enrolled. The median age was 4 years; 13% were infants., Main Outcome Measure: Five-year event free survival., Results: The median symptom interval was 2-months. Head and neck region was the most frequent site (44%), followed by tumors in the extremity (15.7%). The majority (67%) of the tumors were located at 'unfavorable' sites; 68% were >5 cm in size. The most frequent (58%) pathological subtype was embryonal. Treatment was based on the 'Intergroup Rhabdomyosarcoma Study (IRS) Group' risk-stratification. 33% were 'low-risk' children, 11% were 'high-risk'. Treatment-refusal (18%) and abandonment (33%) were major impediments. The median ± SE five-year event free survival of those taking treatment was 43.6 ± 6%., Conclusion: Large sized tumors, tumors at unfavorable locations, and treatment refusal/abandonment contributed to inferior outcome in children with rhabdomyosarcoma.

Purpose of Review: Three primary categories of gynecologic cancer are found in pediatric and adolescent patients: stromal carcinomas including juvenile granulosa cell tumors and Sertoli-Leydig cell tumors, rhabdomyosarcomas arising from the vagina and cervix (sarcoma botryoides), and ovarian germ cell tumors which comprise a wide range of histologies. These entities are rare and treatment approaches have focused on decreasing late effects of chemotherapy treatment. Here, we review presentation, histologic classifications, diagnosis, and treatment recommendations for pediatric gynecologic cancers., Recent Findings: Event-free and overall survival for these cancers is high, and the goals of treatment are minimization of morbidity and preservation of fertility with unilateral salpingo-oophorectomies and limited staging. Surveillance of tumor markers after surgery is helpful in monitoring for disease progression and adjuvant chemotherapy is often reserved for patients at recurrence. Recent literature supports avoiding chemotherapy even in high-grade germ cell tumors in the pediatric population.

Background: Adolescents with cancer are enrolled in clinical trials at far lower rates than children. This report compares the number of adolescents (15-19-year-olds) and children (0-14-year-olds) enrolled in the protocols of the European pediatric Soft tissue sarcoma Study Group (EpSSG) with the number of cases expected to occur., Methods: The observed-to-expected (O/E) ratio was detected in the EpSSG countries contributing most of the cases, that is, Italy, France, Spain, the Netherlands, United Kingdom, and Ireland. The observed cases included patients enrolled in any of the EpSSG protocols from October 2008 to October 2015, when all EpSSG protocols were open in these countries. The number of expected cases was calculated from the incidence rates estimated throughout the RARECAREnet database in the countries' population-based cancer registries., Results: In the countries considered, 2,118 cases aged 0-19 years were enrolled in the EpSSG trials from 2008 to 2015: 82.8% were children and 17.2% were adolescents. The O/E ratio was 0.30 among patients 15-19 years old, as opposed to 0.64 for those 0-14 years old. The O/E ratio differed for the different subtypes: in adolescents, it was 0.64 and 0.18 for rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma soft tissue sarcomas (NRSTS), respectively; in children, it was 0.77 and 0.50, respectively. The O/E ratios differed across the countries considered., Conclusions: Adolescents were less well represented than children on the EpSSG protocols, with better enrolment for RMS than for NRSTS for all age groups., (© 2016 Wiley Periodicals, Inc.)

Purpose of Review: To review the recent literature regarding biologic characteristics of pediatric solid tumors in African children., Recent Findings: Data regarding pediatric solid tumors in Africa, while increasing, remain sparse when considering the ethnic and geographic diversity of the continent. Recent work, especially regarding nephroblastoma in Kenya, has identified some biologic variability among local tribes but also when compared with North American tumors. In general, reports from across the continent reveal markedly poorer survival for pediatric patients with solid tumors when compared with high-resourced regions., Summary: Multiple resource-related and infrastructure-related challenges contribute to poorer outcomes, and these require systematic, multidisciplinary, and structured solutions. Socioeconomic factors and limited access to care currently seem to drive the survival outcomes in children with solid cancers in Africa.

The study was aimed at evaluating adherence to treatment protocol and outcome in pediatric parameningeal rhabdomyosarcoma (PM-RMS). We analyzed the characteristics, treatment administered, outcomes and patterns of failure of pediatric PM-RMS, who were treated with multimodality therapy between January 2005 and December 2013.Univariate and multivariate analysis (MVA) was completed to evaluate the impact of various prognostic factors. Thirty-seven patients were treated at our institution. Majority of them had the primary disease in paranasal sinuses (n=13). Majority of the patients belonged to group III (n=30) and stage III (n=24). The overall response rate to treatment was 52.5% (n=21). At a mean follow-up of 19.1 months, 23 patients developed disease progression. The actuarial rates of failure-free survival and overall survival (OS) at 2 years were 40% and 67.5%, respectively. Patients who received >20 weeks of intended chemotherapy schedule (P=0.02) and had complete response to first-line treatment (P=0.0004) were found to have superior failure-free survival on MVA. Complete response was the lone determinant of superior OS on MVA (P=0.006). Majority of patients with PM-RMS present with advanced stage disease. Response to first-line treatment is a significant predictor of superior progression-free survival and OS in these patients.

Soft tissue sarcoma constitutes 8% of all tumors in adolescent and young adults (AYA), with rhabdomyosarcoma (RMS) accounting for 5.2% to 6.5% of the soft tissue sarcoma total within this group. AYAs have a higher propensity for metastasis and inferior outcomes. Metastases to the breast have been reported in ∼3% to 6% of RMS cases. A review of our hospital's tumor registry identified cases of RMS diagnosed between January 1, 2004 and December 31, 2013. A total of 46 patients with RMS were identified, having a mean age of 12.5 years (range, 1 to 49 y). There were 26 males (57%) and 20 females (43%). Eighteen patients (39%) were AYAs, including 10 women. Four patients (8.7%) were identified with breast involvement, all of whom were AYA females. Treatment modalities included chemotherapy, surgical resection, and radiation. One patient is a long-term survivor. Although RMS is uncommon in AYAs, breast involvement occurs almost exclusively in AYA women and is associated with alveolar histology, metastatic disease, and poor outcomes. In total, 4/10 of all AYA females had breast involvement. Routine examination or imaging of the breasts in AYAs with RMS is not currently standard practice at diagnosis or follow-up, but this analysis suggests it should be considered in female AYA patients.

Objective: To examine clinical characteristics associated with bacteremia in febrile nonneutropenic pediatric oncology patients with central venous catheters (CVCs) in the emergency department (ED)., Background: Fever is the primary reason pediatric oncology patients present to the ED. The literature states that 0.9% to 39% of febrile nonneutropenic oncology patients are bacteremic, yet few studies have investigated infectious risk factors in this population., Methods: This was a retrospective cohort study in a pediatric ED, reviewing medical records from 2002 to 2014. Inclusion criteria were patients with cancer, temperature at least 38°C, presence of a CVC, absolute neutrophil count greater than 500 cells/μL, and age less than 22 years. Exclusion criteria were repeat ED visits within 72 hours, bloodwork results not reported by the laboratory, and patients without oncologic history documented at the study hospital. The primary outcome measure is a positive blood culture (+BC). Other variables include age, sex, CVC type, cancer diagnosis, absolute neutrophil count, vital signs, upper respiratory infection (URI) symptoms, and amount of intravenous (IV) normal saline (NS) administered in the ED. Data were analyzed using descriptive statistics and a multiple logistic regression model., Results: A total of 1322 ED visits were sampled, with 534 enrolled, and 39 visits had +BC (7.3%). Variables associated with an increased risk of +BC included the following: absence of URI symptoms (odds ratio [OR], 2.30; 95% CI, 1.13-4.69), neuroblastoma (OR, 3.65; 95% CI, 1.47-9.09), "other" cancer diagnosis (OR, 4.56; 95% CI, 1.93-10.76), tunneled externalized CVC (OR, 5.04; 95% CI, 2.25-11.28), and receiving at least 20 mL/kg IV NS (OR, 2.34; 95% CI, 1.2-4.55). The results of a multiple logistic regression model also showed these variables to be associated with +BC., Conclusion: The absence of URI symptoms, presence of an externalized CVC, neuroblastoma or other cancer diagnosis, and receiving at least 20 mL/kg IV NS in the ED are associated with increased risk of bacteremia in nonneutropenic pediatric oncology patients with a CVC., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Introduction: Breast metastasis is rare in childhood malignancies. Soft tissue sarcomas, especially rhabdomyosarcomas (RMS), and hematologic neoplasms, such as lymphomas, are the most common tumors that metastasize to the breast, albeit rare., Materials and Methods: All cases with breast metastasis within a cohort of 200 RMS patients followed in our institution during 1990 to 2014 were assessed retrospectively and the literature was reviewed., Results: There were 3 adolescent female patients with breast metastasis. All had alveolar histology. The primary tumors were in the parameningeal sites, extremities, and the perineum, respectively. Two patients had breast metastasis at diagnosis, and 1 during follow-up. In 1 breast lesion, there was a complete response to chemotherapy, and in another there was no response to chemotherapy, and the patient underwent radical mastectomy. In the third patient, there was partial response, and lesions progressed. All patients died with recurrent/progressive disease, 2 with no recurrence in the breast. In the English literature, there are 70 cases including our cases. All but 1 involve female patients, all adolescents, most have alveolar histology and poor prognosis. All had chemotherapy, whereas some had surgery and/or radiotherapy for local treatment., Conclusion: Breast metastasis should be considered in adolescent female patients with RMS. Optimal management is not clear. Besides chemotherapy, mastectomy and radiotherapy should be considered on a case basis.

Rhabdomyosarcoma, most common soft tissue tumor in children, represent 8% of solid tumors in children. Conversely, in adults, this histology is very rare and no consensual recommendation is supported. If gynecological localization is one of the most frequent in children, it is a minority in adults. The management of this type of tumor is based on treatment multimodality combining surgery, chemotherapy, radiotherapy and brachytherapy. This pathological separate entity differs from other sarcomas by its greater sensitivity to chemotherapy and radiotherapy. The aim of this study is to conduct a general review of diagnostic and treatment of genital tract rhabdomyosarcoma in adults, and to report pathological characteristics of this type of tumor., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Head and neck cancer emergency presentations are uncommon but persistent. However, there is little published literature on this aspect of cancer and patient demographics. This study aimed to assess the incidence, patient profile, tumour site and stage of emergency cancer presentations in our region., Method: Retrospective review of regional cancer database over a five-year period., Results: Emergency presentations accounted for 7 per cent of all cases. There was no difference in patient age and risk factors between the emergency and non-emergency presentations. The emergency presentation group showed a greater proportion of female patients compared to the non-emergency presentation group (30 vs 15 per cent). In all emergency presentations, the cancer was at advanced stages. Oropharyngeal cancer was the commonest emergency presentation of cancer, but the third commonest in the non-emergency group., Conclusion: Emergency presentations are increasing annually. Female patients and oropharyngeal cancer showed greater representation compared to male patients and laryngeal cancer.

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Aim: Positive outcomes for paediatric rhabdomyosarcoma (RMS) were high in Sweden during the 1990s, but the last decade has seen decreasing trends in overall survival rates. We investigated the incidence, patient and disease characteristics, treatment and outcome of RMS to see whether any reason could be found for this decline., Methods: This study included 210 children under the age of 15 who were diagnosed with RMS and whose details were recorded in the population-based Swedish Childhood Cancer Registry from 1984 to 2010., Results: The overall annual incidence of RMS was 4.9 per million, and the 5-year overall survival rates were 59 ± 7% in 1984-1989, 78 ± 5% in 1990-1999 and 71 ± 5% in 2000-2010. When patients with localised disease were analysed separately, there was no difference in the 5-year survival rates between 1990 and 1999 (82 ± 5%) and 2000-2010 (81 ± 5%), but the outcome in 1984-1989 (53 ± 8%) was significantly worse. The prevalence of metastatic disease was unexpectedly high during 2000-2010 (28%, p = 0.010), compared to an overall mean of 18% for the whole study period., Conclusion: Our results suggest that a higher rate of metastatic disease may explain the declining trend in overall survival rates in paediatric RMS in Sweden over the last decade., (©2015 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

Background: Childhood cancer becomes a public health problem in developing countries which aggravates the burden of childhood mortality by infectious diseases and malnutrition. In poor countries, the death rate for most pediatric cancers is almost 100%. This study attempts to determine the magnitude, patterns and trends of pediatric malignancies in the study area which is important in re-evaluating existing services and in improving facilities and patient care., Methods: A retrospective study of 3 year period were carried out among all children aged below 15 years old admitted into the pediatric wards of Gondar University Hospital, Northwest Ethiopia. The charts of all children aged below 15 years old admitted in the pediatric wards due to cancer were reviewed by using the data collection format. Data were entered and analyzed using SPSS version 20 statistical package., Result: A total of 71 cancer cases were diagnosed and admitted to the pediatrics ward during the study period. More than two-third of the study subjects 50 (70.4%) were males. The mean age of study subjects was 7 ± 4 year where majority 26 (36.6%) of the study subjects were ≥10 years. Of all, 43 (60.6%) were hematological malignancy followed by Wilms tumor 13 (18.3%), Neuroblastoma 5 (7%), Rhabdomyosarcoma 3 (4.2%), Brain tumor 3 (4.2%), Hepatoblastoma 2 (2.8%). More than two-third of cases were found to be concomitantly malnourished being stunted, wasted and under weight. Nearly half of patients had not received chemotherapy and majority of those started chemotherapy did not complete all the treatment cycles. Shortage and absence of safe and affordable chemotherapy drugs were the major reasons for therapy interruption., Conclusion: The study shows increasing childhood cancer cases over the years. Hematological malignancy takes the leading prevalence followed by Wilms tumor and Neuroblastoma. The majority of cases were also discharged without any clinical change that had the only death option. Therefore, the government and the hospital should give emphasis to establish cancer therapy centers and insure accessibility and affordability of chemotherapy drugs.

Relatively little is known about the epidemiology and factors underlying susceptibility to childhood rhabdomyosarcoma (RMS). To better characterize genetic susceptibility to childhood RMS, we evaluated the role of family history of cancer using data from the largest case-control study of RMS and the Utah Population Database (UPDB). RMS cases (n = 322) were obtained from the Children's Oncology Group (COG). Population-based controls (n = 322) were pair-matched to cases on race, sex, and age. Conditional logistic regression was used to evaluate the association between family history of cancer and childhood RMS. The results were validated using the UPDB, from which 130 RMS cases were identified and matched to controls (n = 1300) on sex and year of birth. The results were combined to generate summary odds ratios (OR(s) ) and 95% confidence intervals (CI). Having a first-degree relative with a cancer history was more common in RMS cases than controls (OR(s) = 1.39, 95% CI: 0.97-1.98). Notably, this association was stronger among those with embryonal RMS (OR(s) = 2.44, 95% CI: 1.54-3.86). Moreover, having a first-degree relative who was younger at diagnosis of cancer (<30 years) was associated with a greater risk of RMS (OR(s) = 2.37, 95% CI: 1.34-4.18). In the largest analysis of its kind, we found that most children diagnosed with RMS did not have a family history of cancer. However, our results indicate an increased risk of RMS (particularly embryonal RMS) in children who have a first-degree relative with cancer, and among those whose relatives were diagnosed with cancer at <30 years of age., (© 2015 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Background: The role, optimal dose, and efficacy of radiotherapy (RT) for the treatment of bone metastases in rhabdomyosarcoma (RMS) and Ewing sarcoma (ES) are unclear., Procedure: All patients with ES or RMS who received RT for bone metastases with curative intent during frontline therapy at Memorial Sloan Kettering Cancer Center (MSKCC) between 1995 and 2013 were reviewed. Among the 30 patients (8 RMS and 22 ES), 49 bone metastases were irradiated., Results: Median biologically effective dose (BED) was 42.4 Gy (range, 34.9-59.7) for RMS and 50.7 Gy (range, 31.3-65.8) for ES. Tumor recurrence occurred in six of 49 irradiated bone metastases. Cumulative incidence of local failure at a treated metastatic site was 6.6% at 1 year and 9.0% at 3 years. Dose, fractionation, and RT technique did not impact local control at an irradiated site. The presence of >5 bone metastases was associated with worse local control at an irradiated site (P = 0.07). The 3-year EFS was 33% in RMS and 16% in ES., Conclusions: RT appears to be an effective modality of local control for bone metastases in ES and RMS. Local control at sites of metastatic bone irradiation is similar to local control at the primary site after definitive RT. Doses in the biologic range prescribed for the definitive treatment of primary disease should be used for metastatic sites of disease., Competing Interests: Nothing to declare., (© 2014 Wiley Periodicals, Inc.)

Background: Rhabdomyosarcoma is the most common soft tissue sarcoma in children ≤ 15 years of age. There is, however, a paucity of reports on the pattern of its occurrence in Nigeria and other parts of Africa., Objective: The aim of this review is to highlight the age and sex distribution, anatomical location and morphological characteristics of rhabdomyosarcoma among children in Kano, northwestern Nigeria., Patients and Method: This is a 14 year retrospective study of all cases of rhabdomyosarcomas occurring in children diagnosed at the Pathology Department of Aminu Kano Teaching Hospital, Kano, Nigeria. Clinical data obtained included sex, age, tumour site and histologic subtype., Results: Fifty two children were seen with a male to female ratio of 1 : 1.3. The ages of these Patients ranged from 4 months to 15 years with a mean (SD) age of 6.99 (4.1) years and peak age frequency in the 0 to 5 years age group. The embryonal variant was the most common histological subtype (69%). The others were alveolar (27%) and pleomorphic (4%) variants. The head and neck was the most affected region (44%) and the most common primary site was the orbit (19.2%)., Conclusion: Rhabdomyosarcoma is the most common childhood soft tissue sarcoma in Kano. It occurs in females more frequently than males and the most common histologic subtype is embryonal rhabdomyosarcoma affecting predominantly the orbit.

Aim: To present the epidemiologic data (age, gender, size etc) and histopathologic and immunohistochemical features of cases of rhabdomyosarcoma (RMS) reported in our department., Settings: Section of Histopathology, Department of Pathology and Microbiology, Aga Khan University Hospital, Karachi, Pakistan., Duration: All cases of RMS diagnosed between January 1, 2003 and December 31, 2012 were included in the study., Results: A total of 277 cases were included. Embryonal rhabdomyosarcoma (ERMS) was by far the dominant histologic type (87.4%) followed by alveolar type (ARMS)(9.4%). ERMS was much more common in males (64.0%)and over 65% of cases occurred in the first decade of life (over 90% in the first two decades). Head and neck region was commonest site for ERMS (46.7%), followed by the genitourinary system (16.1%). Over 65% cases of ARMS occurred in the extremities. Over 80% cases of ARMS occurred in the first 3 decades of life. Immunohistochemical staining for Desmin and MyoD1 was positive in 96.7% and 85.4% respectively., Conclusions: The epidemiologic data and microscopic findings in our patients are similar to international published data on rhabdomyosarcoma.

Objective: To evaluate the role of parental military service-related exposures and rhabdomyosarcoma (RMS) risk in offspring using data from a large case-control study of childhood RMS., Study Design: Cases (n = 319) were enrolled from the third trial run by the Intergroup Rhabdomyosarcoma Study Group. Population-based controls (n = 319) were pair-matched to cases on race, sex, and age. Conditional logistic regression was used to evaluate parental military service-related exposures and their associations with childhood RMS by generating aORs and 95% CIs. Statistical significance was defined as P < .05., Results: There were no significant associations between parental military service and childhood RMS. The strongest association was with maternal military service; however, this association was attenuated and did not remain significant after adjusting for covariates (aOR = 2.75, 95% CI 0.71, 10.62). An elevated effect estimate was found when assessing paternal exposure to Agent Orange (AO) and childhood RMS but was not statistically significant (aOR = 1.72, 95% CI 0.55, 5.41)., Conclusions: We found little evidence that parental military service of AO exposure influences the risk of RMS in offspring. These findings are notable in light of the continuing controversies surrounding the intergenerational effects of AO exposure., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Sarcomas constitute 1% of all malignancies, but 10% occur in the head and neck (HN), and they are poorly understood. We present a large series of Soft Tissue HN sarcomas in Mexican patients with survival analysis., Study Design: This is a retrospective study of cases in a cancer hospital., Methods: Review of files and pathology material. Literature review., Results: We analysed 108 patients (55 men / 53 women). The age at presentation was 37 years. The original diagnosis changed in nine cases (8.3%). The most common subtype was rhabdomyosarcoma. Ninety percent of tumours were deep, 91% were high grade, 44% had metastasis, 63% measured >5 cm, overall 5-year survival (5 y-OS) was 48%, and histological high grade was associated with poor survival (p = 0.026)., Conclusion: Sarcomas of the HN are rare. The most affected sites were paranasal sinuses. The majority of tumours were deep, > 5 cm and high grade, 50% had metastasis, the 5 y-OS was 48% and the only independent factor associated with 5 y-OS was histologic grade., (Copyright © 2014 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)