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Background Initial staging of rhabdomyosarcoma is crucial for prognosis and to tailor the treatment. The standard radiology workup (SRW) includes magnetic resonance imaging, chest computed tomography (CT) and bone scintigraphy, but 18 Fluorine-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) (18F-FDG-PET/CT (PET-CT)) use is increasing. The aim of this study was to evaluate the impact of PET-CT in the initial staging of patients with metastatic rhabdomyosarcoma enrolled in the European protocol MTS2008. Methods Two authors retrospectively reviewed the SRW and PET-CT reports comparing the number and sites of metastases detected. For bone marrow involvement, PET-CT and bone marrow aspirates/biopsies were compared. Results Among 263 metastatic patients enrolled from October 2008 to December 2016, 121 had PET-CT performed at diagnosis, and for 118 of 121 patients, both PET-CT and radiological reports were available for review. PET-CT showed higher sensitivity than SRW in the ability to detect locoregional (96.2% versus 78.5%, P value = 0.0013) and distant lymph node involvement (94.8% versus 79.3%, P value = 0.0242), but sensitivity was lower for intrathoracic sites (lung 79.6% versus 100%, P value = 0.0025). For bone metastasis, PET-CT was more sensitive than bone scintigraphy (96.4% versus 67.9%, P value = 0.0116). The PET-CT sensitivity and specificity to detect marrow involvement were 91.8% and 93.8%, respectively. The mean number of metastatic sites was 1.94 (range 0–5) with PET-CT and 1.72 (range 0–5) with SRW. In four patients (3.4%), PET-CT changed the staging from localised to metastatic disease. Conclusion PET can identify metastatic disease not evident on SRW in a small number of patients. This is because of its higher ability to recognise lymph node and bone involvement. Chest CT remains essential to detect lesions in intrathoracic sites, which can be performed in a one stop-shot routine examination or on a dedicated chest CT scan. PET-CT could replace bone scintigraphy to study bone involvement.

Rhabdomyosarcoma is the commonest soft tissue sarcoma in children. Clinicians need vigilance to recognize the different signs and symptoms this tumor can present with because of variable sites of origin. Diagnosis requires a safe biopsy that obtains sufficient tissue for pathologic, genetic, and biological characterization of the tumor. Treatment depends on accurate staging with imaging and surgical sampling of draining lymph nodes. A multidisciplinary team assigns patients to risk-based therapy. Patients require chemotherapy and usually a combination of complex, site-specific surgery and/or radiotherapy. Outcomes for localized rhabdomyosarcoma continue to improve but new treatments are required for metastatic and relapsed disease.

Rhabdomyosarcoma (RMS) is the most common form of soft tissue sarcoma in children. We report the results of the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study, which prospectively evaluated the reduction of chemotherapy in patients with embryonal RMS (ERMS) after initial surgery.Between October 2005 and December 2016, all patients with localised ERMS with an initial microscopically complete resection (IRS group I) with lymph node-negative (N0) were prospectively enrolled in the low-risk (n = 70, subgroup A; age 10 years and tumour size ≤ 5 cm) or standard-risk group (n = 108, subgroup B; age ≥ 10 years or tumour size 5 cm. Subgroup A received 8 courses of vincristine and dactinomycin (VA) for 22 weeks; subgroup B received 4 courses of VA with ifosfamide (IVA) and 5 courses of VA for 25 weeks.The 5-year event-free survival (EFS) and overall survival (OS) were 90.8% (95% confidence interval [CI]: 85.0-94.4) and 95.7% (95% CI: 90.5-98.1), respectively (n = 178). The EFS and OS were 95.5% (95% CI: 86.8-98.5) and 100% (subgroupA), and 87.8% (95% CI: 79.3-93.0) and 93.0% (95% CI: 84.8-96.8)(subgroup B), respectively. Bearman stage 2 veno-occlusive disease (VOD) occurred in 4 very young patients.VA treatment for 8 courses was effective and well tolerated by the subgroup of patients with low-risk ERMS (group A). Four courses of IVA and 5 courses of VA instead of 9 courses of IVA also has very good results. Careful monitoring for liver toxicity is important in very young patients. European union drug regulating authorities clinical trials EUDRACT No. 2005-000217-35.

Rhabdomyosarcoma of the extremities present with two main challenges: correct evaluation of initial regional nodal involvement and define adequate local treatment. Methods Pediatric patients with localized rhabdomyosarcoma of the extremity included in the EpSSG‐RMS2005 study between 2005 and 2014 were evaluated for staging, treatment, and survival. The outcome was compared to the preceding European SIOP‐MMT studies. Results Of the 162 patients included, histology was unfavorable in 113 (70%), 124 (77%) were younger than 10 years, 128 (79%) were IRS III, and 47 (29%) were node‐positive. A regional node biopsy was performed in 97 patients (60%) and modified the lymph node stage in 15/97 (16%). Primary and delayed surgery was performed in 155 (96%) and radiotherapy delivered in 118 (73%) patients. Relapse occurred in 61 cases (38%), local in 14 (23%), regional in 13 (21%), distant in 22 (36%), and combined relapse in 12 (20%) with five progressive diseases (8%) and four secondary tumors (7%). Five‐year event free (EFS) and overall survival (OS) were 58.4% (95%CI, 50.3‐65.7) and 71.7% (63.6‐78.4), respectively. In the previous studies MMT89 and MMT95, tumor surgery was performed in 32/53 (60%) and 74/82(90%), respectively, and radiotherapy delivered in 13/53 (25%) and 26/82 (30%), respectively. Five‐year EFS and OS were 35.6%, and 50.3% in MMT89 and 54.3% and 68.2% in the MMT95 study. Conclusions Even if the lymph node staging was not always complete according to the RMS2005 protocol, node sampling changed lymph node status in a significant number of patients. Despite the higher rate of patients treated with locoregional radiotherapy, survival in RMS2005 did not improve compared to the previous European SIOP‐MMT95 study., Adequate staging is important for the treatment of extremity RMS. Despite local therapy intensification, survival in RMS2005 did not improve compared to the previous European SIOP‐MMT95 study.

The treatment of paratesticular rhabdomyosarcoma (PT-RMS) has varied over time and by cooperative group. The International Soft Tissue Sarcoma Database Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft tissue sarcoma Study Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS). The INSTRuCT surgical committee has been given charge of the development of internationally applicable consensus guidelines for the surgical treatment of rhabdomyosarcoma. This clinical consensus opinion document addresses accepted principles and areas of controversy, such as scrotal violation and retroperitoneal nodal evaluation, providing an evidence-based guideline for the surgical treatment for PT-RMS.

BACKGROUND Paratesticular rhabdomyosarcoma (PT RMS) is rare compared to benign scrotal pathology. Inappropriate first surgery (InFS) required supplementary treatment to maintain excellent outcomes. Initial staging of regional lymph nodes is important. The aim of this study was to determine to what extent the quality of locoregional approach impacted on patient morbidity and survival. DESIGN/METHODS Analysis was performed on all nonmetastatic PT RMS patients enrolled in the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 protocol. Aspects assessed were adherence to surgical guidelines and impact of protocol violations, relapse analysis, and survival outcomes. RESULTS Analysis was performed on 237 patients, with median follow up of 67.1 months. Median age was 9.0 years. InFS occurred in 75 of 237 (32%) patients. InFS required intensified chemotherapy (10) and local therapy. After InFS, 61 required primary reexcision and five delayed surgery. Of 26 recurrences, the risk of relapse was higher in patients ≥10 years (21/26) and was mainly locoregional in 16 of 26 recurrences (± metastatic). Sixteen of 26 died with 14 of 16 patients ≥10 years. Nodal relapse neither occurred when N1 nodes were identified at diagnosis, nor after surgical staging. Five-year overall survival (OS) at age

Timely presentation to care for people newly diagnosed with HIV is critical to optimize health outcomes and reduce onward HIV transmission. Studies describing presentation to care following diagnosis during a hospital admission are lacking. We sought to assess the timeliness of presentation to care and to identify factors associated with delayed presentation. We conducted a population-level study using health administrative databases. Participants were all individuals older than 16 and newly diagnosed with HIV during hospital admission in Ontario, Canada, between April 1, 2007 and March 31, 2015. We used modified Poisson regression models to derive relative risk ratios for the association between sociodemographic and clinical variables and the presentation to out-patient HIV care by 90 days following hospital discharge. Among 372 patients who received a primary HIV diagnosis in hospital, 83.6% presented to care by 90 days. Following multivariable analysis, we did not find associations between patient sociodemographic or clinical characteristics and presentation to care by 90 days. In a secondary analysis of 483 patients diagnosed during hospitalization but for whom HIV was not recorded as the principal reason for admission, 73.1% presented to care by 90 days. Following multivariable adjustment, we found immigrants from countries with generalized HIV epidemics (RR 1.265, 95% CI 1.133-1.413) were more likely to present to care, whereas timely presentation was less likely for people with a mental health diagnosis (RR 0.817, 95% CI 0.742-0.898) and women (RR 0.748, 95% CI 0.559-1.001). Future work should evaluate mechanisms to facilitate presentation to care among these populations.

Background Risk factors for cause-specific mortality have not been widely studied among people with HIV infection. Our objectives were to estimate rates of and risk factors for all-cause and cause-specific mortality from 1995 to 2014 among HIV-infected people in Ontario. Methods We conducted a population-based retrospective cohort study using provincial health databases of people with HIV infection who were aged 16 years or more, were residents of Ontario between 1995 and 2014, and had HIV infection according to a previously validated algorithm. We used International Classification of Diseases codes to classify the underlying cause of death and estimated age-adjusted mortality rates per 100 person-years for 1995 to 2014. We used descriptive statistics to characterize the cohort at baseline and calculated adjusted mortality rate ratios (RRs) using generalized estimating equations. Results Among 23 043 people, the all-cause mortality rate declined from 6.69 to 1.53 per 100 person-years over the study period, and the rate of death from HIV/AIDS declined from 4.75 to 0.46 per 100 person-years. Concomitantly, the proportions of deaths due to cancer, cardiovascular disease and other noncommunicable diseases rose; however, rates remained constant or declined. Compared to males, females had higher mortality due to cardiovascular disease (adjusted RR 1.36, 95% confidence interval [CI] 1.04-1.77), noncommunicable causes (adjusted RR 1.75, 95% CI 1.39-2.20) and, by 2010-2014, any cause (adjusted RR 1.19, 95% CI 1.02-1.38). Residing in a low-income neighbourhood was associated with increased risk for most causes, including HIV/AIDS (adjusted RR in 2010-2014 1.86, 95% CI 1.49-2.31). Rural residence was associated with increased mortality due to malignant disease (adjusted RR 1.60, 95% CI 1.10-2.34) and noncommunicable disease (adjusted RR 1.86, 95% CI 1.25-2.77). Being an immigrant was associated with lower risk of death from all causes. Interpretation Over the study period, death was increasingly due to common chronic conditions rather than to HIV infection itself. Care should incorporate the prevention and management of these conditions, especially among females and those residing in rural and low-income areas.

Background: Rhabdomyosarcoma is an aggressive tumour that can develop in almost any part of the body. Doxorubicin is an effective drug against rhabdomyosarcoma, but its role in combination with an established multidrug regimen remains controversial. Therefore, we aimed to evaluate the possible benefit of early dose intensification with doxorubicin in patients with non-metastatic rhabdomyosarcoma. Methods: We did a multicentre, open-label, randomised controlled, phase 3 trial involving 108 hospitals from 14 countries. We included patients older than 6 months but younger than 21 years with a pathologically proven diagnosis of rhabdomyosarcoma. We assigned each patient to a specific subgroup according to the EpSSG stratification system. Those with embryonal rhabdomyosarcoma incompletely resected and localised at unfavourable sites with or without nodal involvement, or those with alveolar rhabdomyosarcoma without nodal involvement were considered at high risk of relapse. These high-risk patients were randomly assigned (1:1) to receive either nine cycles of IVA (ifosfamide 3 g/m2 given as a 3-h intravenous infusion on days 1 and 2, vincristine 1·5 mg/m2 weekly during the first 7 weeks then only on day 1 of each cycle [given as a single intravenous injection], and dactinomycin 1·5 mg/m2 on day 1 given as a single intravenous injection) or four cycles of IVA with doxorubicin 30 mg/m2 given as a 4-h intravenous infusion on days 1 and 2 followed by five cycles of IVA. The interval between cycles was 3 weeks. Randomisation was done using a web-based system and was stratified (block sizes of four) by enrolling country and risk subgroup. Neither investigators nor patients were masked to treatment allocation. The primary endpoint was 3-year event-free survival assessed by the investigator at each centre in the intention-to-treat population. Patients who received at least one dose of study treatment were considered in the safety analysis. In agreement with the independent data monitoring committee, the study was closed to patient entry on Dec 16, 2013, after futility analysis. This trial is registered with EudraCT, number 2005-000217-35, and is currently in follow-up. Findings: Between Oct 1, 2005, and Dec 16, 2013, 484 patients were randomly assigned to receive each chemotherapy regimen (242 in the IVA group and 242 in the IVA plus doxorubicin group). Median follow-up was 63·9 months (IQR 44·6–78·9). The 3-year event-free survival was 67·5% (95% CI 61·2–73·1) in the IVA plus doxorubicin group and 63·3% (56·8–69·0) in the IVA group (hazard ratio 0·87, 95% CI 0·65–1·16; p=0·33). Grade 3–4 leucopenia (232 [93%] of 249 patients in the IVA plus doxorubicin group vs 194 [85%] of 227 in the IVA group; p=0·0061), anaemia (195 [78%] vs 111 [49%]; p

Circumcision is a relatively brief and safe procedure when performed by a medical professional. Recently, clamp-assisted circumcisions in the adult male have gained increased interest due to potential public health benefits. With the heightened interest has come an increased accessibility to such devices, creating the opportunity for ill-advised home attempts at circumcision by private citizens. To our knowledge, we present the first reported case of poorly executed self-circumcision in an adult male.

Soft tissue lesions in neonates are relatively common. They cause concern for parents and can also present diagnostic difficulties for health professionals. Fortunately most are benign and usually vascular or developmental in origin. However, a small number of these lesions are found to be malignant and can present significant challenges in terms of management and may carry a poor prognosis. Therefore it is important to develop an approach to the diagnosis and management of all soft tissue lesions that will allow such tumours to be distinguished from the more common benign lesions.

Objectives To evaluate the impact of local therapies on the outcome of patients with liver-bile duct rhabdomyosarcoma (LBDRMS). Methods Data of 30 patients included in the EpSSG-RMS 2005 study were analyzed. Results The median age at diagnosis was 3 years (11 months-8 years). All patients had non-alveolar histology. Fifteen patients had a tumor > 5 cm and six had enlarged regional lymph nodes on imaging. Eight patients (27%) had primary surgery (1 R0). Six of them received external beam radiotherapy (EBRT). All are in first complete remission (CR1) except one (R1, EBRT+ , local relapse, death). Six patients (20%) received EBRT without surgery: one had local relapse and died. Sixteen patients (53%) underwent delayed surgery, with 12 achieving R0 margins, which were higher than those in the primary surgery group (P = 0.003). Three patients with R0 margins received EBRT; one had a metastatic relapse and died. Nine patients with R0 resection did not receive EBRT, three relapsed locally (two deaths). Four R1 patients received additional EBRT without relapses. Local relapse occurred in two among 19 patients with EBRT and three among 11 without EBRT (P = 0.326). At a median follow-up of 61 months (48-84 months), five patients died; all had a tumor size > 5 cm (P = 0.01). The five-year overall survival was 85% (95% CI, 65-94), and event-free survival was 76% (95% CI, 54-89). Conclusion This analysis did not show any significant difference in outcome between irradiated and nonirradiated patients. Local relapse in LBDRMS is related to initial tumor size and is often fatal.

High-flow priapism secondary to posttraumatic arteriovenous fistula (AVF) formation is a condition that can be easily diagnosed via penile color Doppler ultrasonography. In order to make the diagnosis, however, adequate coverage of the shaft and perineum is required when performing the scan. This case highlights failure to properly cover these areas, resulting in missed diagnosis of penile AVF.

Wilms’ tumours (WT) with retrohepatic vascular extension traditionally requires cardiac bypass for complete excision. We share our experience of these complex cases. A retrospective review was performed of children with WT with retrohepatic vascular extension presenting to two UK children’s hospitals. Tumour stage, chemotherapy, level of vascular extension, operative details and complication data were analysed. Ten children were identified. Mean age 6.6 years (range 3.3–8.2 years); tumour side 6 right, 2 left, 2 bilateral. Level of tumour extension was to the right atrium in two, diaphragm in two, hepatic vein (HV) level in four and retrohepatic inferior vena cava (IVC) in one patient. Following chemotherapy it reduced to hepatic veins (5) or below (4). Surgery involved radical nephrectomy and complete mobilisation of the liver off the IVC, which was then clamped, opened and the thrombus excised. There were no intraoperative complications. Mean hospital stay was 9.77 days (7–20 days). Histology showed viable tumour thrombus in six patients. One patient died after 1 year from metastatic disease. Retrohepatic extension of WT can be managed without bypass using pre-operative chemotherapy and by complete liver mobilisation. The tumour was always adherent to IVC and required sharp dissection.

Peritoneal encapsulation is a rare congenital cause of bowel obstruction in children. We present the case of a 12-year-old male with severe dehydration and recurrent episodes of vomiting. This pathology should be considered in cases of bowel obstruction with a virgin abdomen.

PurposeInguinal hernias are a common problem amongst neonates, and a potential source of significant morbidity through incarceration of the hernia. Expedited surgical repair can prevent incarceration and reduce complications. The department at Bristol Children's Hospital aimed to minimise waiting times for surgery thereby reducing incarceration and complication rates. This paper aims to examine how this was done.Design/methodology/approachBristol Children's Hospital provides local and regional (tertiary referral) services. Retrospective audit of waiting times from referral to surgery was performed against the departmental standard of four weeks. Data were collected on frequency of incarceration and any complications. Following service restructure, second cycle audit was performed to assess the impact of change.FindingsFirst cycle audit (n=74) found median waiting time 20 days, (Mean 28.4 days, Range 0‐138 days). A total of 28 per cent of patients waited longer than the target four weeks from referral. Incarceration occurred in 13 per cent of patients awaiting surgery. Two patients (2.7 per cent) experienced complications. Following service restructuring second cycle audit (n=28) found waiting times were significantly reduced (median two days, mean 7.0, pPractical implicationsThis study demonstrates a successful model of service for management of neonatal inguinal hernias which could be adopted at children's hospitals throughout the UK.Originality/valueThis study highlights a successful audit process, in which the extent of a problem is quantified and analysed, strategies for improvement are identified and implemented, and second cycle audit confirms the impact of change and an improved service.

To report the results from International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors studies (MMT 89 and 95) of males with nonmetastatic paratesticular rhabdomyosarcoma. From 1989 to 2003, 159 patients were included. Radical inguinal orchidectomy was recommended, but retroperitoneal lymph node (LN) assessment was based on imaging alone. The treatment was stratified by stage (SIOP tumor-node-metastasis staging system) and histology. Median age at presentation was 5.6 years (range 0.3-17.6) and 120 patients were of 5 cm more frequently compared to patients of

Lipoblastoma is a rare benign mesenchymal tumor of embryonal fat that occurs almost exclusively in infants and children. Although lipoblastomas can affect any part of the body, only 5 cases affecting the scrotum have previously been reported. We present a case of an intrascrotal lipoblastoma with a novel karyotype.

Aneurysms and pseudoaneurysms of the portal venous system are rarely seen following abdominal trauma but clinicians need to be aware of them as possible vascular complications following blunt trauma. This case report of a 10 year old boy following a handlebar injury demonstrates a clear causal relationship between trauma and portal venous pseudoaneurysm. Portal venous aneurysms have a prevalence of less than 0.4% and most are found in patients with underlying hepatocellular disease. Many are asymptomatic in which case surveillance is an accepted management strategy, with Doppler ultrasound proving useful. Complications including thrombosis, distal embolism, compression of the biliary tree and haemorrhage are usually indications for surgical management. Portal venous pseudoaneurysms may be managed conservatively but transcatheter embolisation can be used if there are ongoing complications or haemorrhage.

Background Localised pelvic rhabdomyosarcomas (pRMS) are rare tumours with a poorer prognosis than the majority of RMS. This study analysed patient outcome according to the type of local therapy delivered and the effect of disease-related factors on prognosis. Patients and methods 97 children with localised pRMS were enrolled in the SIOP-MMT84, 89 and 95 studies. After primary surgery or biopsy, all children received ifosfamide/actinomycin/vincristine-based chemotherapy. Radiotherapy and surgery were planned in patients failing to achieve complete remission. Results Median age at diagnosis was 52 months [5 months–18 years]. IRS staging was I for five patients, II for 15 and III for 77. Patients had embryonal RMS (N = 41), alveolar RMS (N = 29), botryoid RMS (N = 3), or not otherwise specified RMS (N = 24). Outcome 87 patients achieved local control (90%), 37 relapsed (43%), mainly locally (84%). With a median follow-up of more than 10 years [4–22 years], 5-year OS was 66% (95% CI: 56–75%) and EFS was 52% (95% CI: 42–61%). Among the 18 IRS-I/II patients treated without radiotherapy, 15 survived. Seven out of the 20 IRS-III patients treated without local therapy died. In multivariate analysis, IRS staging, age greater than 10 years and lymph node involvement had a negative impact on OS. Perineal/perianal locations had a trend towards a worse prognosis. Conclusion pRMS still have a relatively poor prognosis. Radiotherapy or brachytherapy is necessary for all IRS-III patients including those with radiological complete remission after neoadjuvant chemotherapy with or without surgery. Radiotherapy may be withheld in IRS-I patients and children under 3 years with IRS-II pRMS.

We report a rare case of an intrarenal teratoma in a 6-month-old male. Following biopsy, he was successfully managed with a primary nephrectomy. This patient presented as would a Wilms tumor. According to SIOP guidelines, the diagnosis of Wilms tumor is made on clinical and imaging information only, prior to neoadjuvant chemotherapy. This patient was investigated, according to UK (CCLG) guidelines, with a biopsy prior to treatment and therefore avoided unnecessary chemotherapy.

We report the case of a 27-month-old male with an unusual complication of aplastic anaemia and neutropenic enterocolitis. He suffered persistent neutropaenic sepsis and clinical deterioration forced the strategy of matched sibling haematopoietic stem cell transplantation. With engraftment and clinical recovery post-transplant, enteral feeding was re-established. Despite continued improvement the child began to vomit faeculent stomach content. Barium swallow showed gastro-colic and gastro-enteric fistulisation with contrast passing directly from stomach into descending colon and directly into jejunum. Laparotomy confirmed complex fistulae between the gastric body, the splenic flexure of the colon and the jejunum. The diagnosis and management of abdominal pathology secondary to severe pancytopaenia is challenging. Often the patient does not manifest the usual signs of acute abdominal pathology, making the decision to operate and the timing of surgery difficult. Counfounding this is the danger of performing surgery in a pancytopaenic patient. Our case illustrates these challenges and reports the unanticipated finding of a complex gastro-colic fistula. Pediatr Blood Cancer 2014;61:358–359. © 2013 Wiley Periodicals, Inc.