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Liver; Pediatrics; Sarcoma Fetge; Pediatria; Sarcoma Hígado; Pediatría; Sarcoma Background To assess the outcomes of pediatric patients with undifferentiated embryonal sarcoma of the liver (UESL) and treatment including at least surgery and systemic chemotherapy. Methods This study included patients aged up to 21 years with a pathological diagnosis of UESL prospectively enrolled from 1995 to 2016 in three European trials focusing on the effects of surgical margins, preoperative chemotherapy, use of radiotherapy (RT), and chemotherapy. Results Out of 65 patients with a median age at diagnosis of 8.7 years (0.6–20.8), 15 had T2 tumors, and one had lymph node spread, 14 were Intergroup Rhabdomyosarcoma Study (IRS) I, nine IRS II, 38 IRS III, and four IRS IV. Twenty-eight upfront surgeries resulted in five operative spillages and 11 infiltrated surgical margins, whereas 37 delayed surgeries resulted in no spillages (p = .0119) and three infiltrated margins (p = .0238). All patients received chemotherapy, including anthracyclines in 47. RT was administered in 15 patients. With a median follow-up of 78.6 months, 5-year overall and event-free survivals (EFS) were 90.1% (95% confidence interval [CI]: 79.2–95.5) and 89.1% (95% CI: 78.4–94.6), respectively. Two out four local relapses had previous infiltrated margins and two out of three patients with metastatic relapses received reduced doses of alkylating agents. Infiltrated margins (p = .1607), T2 stage (p = .3870), use of RT (p = .8731), and anthracycline-based chemotherapy (p = .1181) were not correlated with EFS. Conclusions Multimodal therapy improved the outcome of UESL. Neoadjuvant chemotherapy for pediatric patients increases the probability of complete surgical resection. The role of anthracyclines and RT for localized disease remains unclear.

Purpose Non-operative treatment (NOT) for pediatric acute appendicitis complicated with intra-abdominal abscess (IAA) has been introduced in the last decade. The study aimed to report our experience with this approach and investigate potential predictors of success. Methods Medical records of patients affected by appendicitis complicated with IAA between January 2013 and December 2020 were reviewed. The interval before delayed appendectomy, rate of re-admission before delayed appendectomy and the rate of delayed appendectomy were the endpoints of NOT. The outcomes were compared between patients treated by NOT and patients who underwent urgent surgery for complicated appendicitis. Results In the study period, twenty-six patients (37%) underwent NOT, and 45 (63%) underwent urgent surgery for complicated appendicitis with IAA. A delayed appendectomy was performed in 24 children (92%). The median time before surgery was 32 days (IQR 20–58 days). Eight children (31%) were re-admitted before delayed appendectomy, and three cases presented a disease relapse. An appendicolith was detected in two of them. Even though the overall hospital stay was longer for NOT (p = 0.0009), all these patients underwent laparoscopic appendectomy, and no conversions were reported (p = 0.0001; p = 0.0006). In addition, no difference in the rate of post-operative complications was found between the two groups (p = 0.62). Conclusions NOT presented a high rate of success. The presence of appendicolith at diagnosis might be considered a negative predictor of success. NOT might increase the success of mini-invasive surgery when compared to urgent surgery.

Introduction Vascular complications are severe complications of pediatric kidney transplantation (KT). We aimed to investigate whether a complex bench surgery (BS) affects the outcomes. Methods All pediatric KT performed at the University Hospital of Padua from 2015 to 2019 were analyzed, comparing those in which a standard BS was possible to those that necessitated a complex BS. The rates of vascular complications, patients' outcome, and graft survival were compared in the two groups. Results Eighty KTs were performed in 78 patients with a median age of 11 years (interquartile range [IQR] 4.3–14) and a median body weight of 24 kg (IQR 13–37). Thirty-nine donor kidneys (49%) needed a complex BS due to anomalies of renal veins in 12 (31%) and renal arteries in 16 (41%). The remaining 11 grafts (28%) underwent an elongation of the vein. There was no difference in the rate of primary graft non function (p = 0.97), delayed graft function (p = 0.72), and overall survival (p = 0.27). The rates of vascular complications, bleedings, and venous graft thrombosis were similar (p = 0.51, p = 0.59, p = 0.78, respectively). No arterial thrombosis or stenosis was reported. Conclusion Complex BS did not compromise survival of the graft and did not put the allograft at risk of vascular complications, such as bleedings or thrombosis.

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.

End stage renal disease (ESRD) requiring kidney transplant (KT) remains an important cause of morbidity in anorectal malformations (ARM) patients. Current literature is scarce on defining the risk factors for ESRD and the outcomes of KT in ARM patients.This study aimed to identify predisposing factors to ESRD in ARM patients and verify if the long term outcome of KT in these patients differs from pure urological anomalies (UA).Databases of ARM and KT patients treated at our center between 2000 and 2016 were used for comparing characteristics of ARM cases which developed ESRD and those who did not, and the outcome features of MAR-KT vs UA-KT.Out of 117 ARM patients, 9 developed ESRD. All of them had a complex ARM. Association with UA were significantly higher in ARM-KT compared to other ARM patients (100% vs. 52%, p = 0.001). The most common UA associated to KT in ARM patients was renal dysplasia. During the same period 23 patients underwent KT as a sequelae of pure urological anomalies (UA-KT group). The most represented UA were primary vesico-ureteral reflux (65.2%) and posterior urethral valves (14%) in UA-KT (table 2). ARM-KT patients required more often hemodialysis before KT (50% vs. 8.7%, p = 0.05) and an aorto-caval anastomosis at the transplant (75% vs. 30%, p = 0.04) compared to UA-KT. Moreover ARM-KT patients experienced more often graft failure and and the need for a second KT (50% vs. 8.6%, p = 0.02).To our knowledge, this is the first study describing the differences in terms of risk factors and outcomes of KT in ARM patients. We observed a need for KT in 7% of ARM, which is at the higher end of the range reported in the literature. Bilateral dysplasia and cloaca malformation seem the leading cause to ESRD for ARM patients compared to vesico-ureteral reflux and posterior urethral valve in UA-KT. Our experience showed that KT has worst outcome in ARM vs pure UA patients.Patients with complex ARM are more frequently associated to renal dysplasia which lead to KT. Graft in ARM patients seems to be the more vulnerable and prone to failure.

Rhabdomyosarcoma of the perianal/perineal region (PRMS) is rare, with poor survival and limited understanding of the functional consequences of treatment.International Society of Pediatric Oncology (SIOP) malignant mesenchymal tumor (MMT) 95, Italian RMS 96, and European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 studies were interrogated to identify factors that impact survival; in RMS 2005, functional outcomes were analyzed.Fifty patients (nonmetastatic) were identified, median age 6.4 years (range: 0.1-19.6): 29 male, 21 female. Tumors were5 cm in 33 patients. Histopathological subtype was alveolar in 35. Lymph nodes were involved in 23 patients. In RMS 2005, 16/21 (76%) tested alveolar tumors had positive FOXO1 fusion status. Diagnostic biopsy was performed in 37. Primary resection (13) was complete (R0) in one. Delayed primary excision (16) was complete in three. Radiotherapy (RT) in 34/50 patients included external beam (28), brachytherapy (3), and both (3). Nodal RT was given in 16/23 N1 patients (70%). Median follow-up of alive patients (29) was 84.1 months (range: 3.6-221.1). Relapse or progression occurred in 24 patients (48%), 87% were fatal and most events (63%) were locoregional. Five-year event-free survival (EFS) was 47.8 (95% CI: 32.8-61.3), and 5-year overall survival (OS) was 52.6 (95% CI: 36.7-66.2), with age ≥10 years and tumor size5 cm impacting 5-year EFS and OS (p .05). Functional outcome data showed bowel, genito-urinary, and psychological issues; fecal incontinence in four of 21 survivors, and urinary symptoms in two of 21.About 60% of patients with nonmetastatic PRMS survive; older patients and those with large tumors have the worst outcomes. Biopsy should be the initial procedure, and definitive local therapy individualized. Quality-of-life and functional studies are needed to better understand the consequences of treatment.

Infants (12 months) with rhabdomyosarcoma have historically had poorer outcome than the older age groups. We present outcomes for infants and young children aged 12-36 months with localised rhabdomyosarcoma with a particular emphasis on infants.All children less than 36 months of age enrolled on the EpSSG RMS 2005 study for localised disease are included. Treatment comprised chemotherapy, local surgery and/or radiation therapy adapted to risk group and age. Main outcome measures were event free survival (EFS) and overall survival (OS).Outcome data were available for 485/490 patients aged less than 36 months, 110 were infants. Infants received chemotherapy according to the risk group with no toxic deaths. Radiotherapy was delivered to 33.6% of infants and 63.5% of 12-36 months old, with respectively 41.7% and 22.2% receiving brachytherapy. Radical surgery was performed in 62% of infants and 57.1% of 12-36 months old. Median follow up for patients who are alive (n = 393) was 72.7 months (range 6.9-158.2). Five-year OS for infants was 88.4% (95%CI 80.3-93.2), which is significantly better than the OS in 12-36 months old patients of 78.0% (95%CI 73.2-82.0; p = 0.0204). Five-year EFS for infants was 72.5% (95%CI 62.8-80.0) compared with 66.1% (95%CI 61.0-70.7; p = 0.2663) for 12-36 months old.Infants treated on RMS 2005 achieved excellent EFS and OS. The EpSSG RMS 2005 chemotherapy regimen, combined with an increase in the application of adequate local therapy, improvements in imaging and supportive care and potentially favourable patients' characteristics may have contributed to these results.

Pediatric abdominal surgery is constantly evolving, alongside the advent of new surgical technologies. A combined use of new tools and traditional surgical approaches can be useful in the management of complex cases, allowing less invasive procedures and sometimes even avoiding multiple interventions. This combination of techniques has implications even from the anesthetic point of view, especially in post-operative pain control. Thereby, tertiary level centres, including highly-specialized professionals and advanced equipment, can maximize the effectiveness of treatments to improve the final outcomes. Our paper aims to present some possible combinations of techniques recently used at our institution to provide a one-session, minimally invasive treatment within different areas of abdominal surgery.

Background A unique Case of metachronous contralateral cystadenoma diagnosed 2 years after a juvenile granulosa cell tumor (JGCT) of the ovary is reported. Case One year after a left ovariectomy for JGCT, a 7 year-old girl was found to have, during her standard ultrasound follow-up, a 18-mm cyst in the right ovary, which was initially considered to be a follicular cyst. Six months later, the mass appeared to be increased and characterized by multivacuolar features, and inhibin A was mildly elevated. A laparoscopic-assisted cystectomy was performed, sparing healthy ovarian tissue around. The pathology report showed a benign mucinous cystadenoma.

PROCEDURE The survival of children with rhabdomyosarcoma (RMS) has gradually improved as a result of the adoption of multidisciplinary treatments. Dedicated skills and facilities are indispensable and more readily available at reference centers. In this study, we examined the role of centers' experience (based on the number of patients treated) in their management of patients with RMS. METHODS We analyzed 342 patients with localized RMS enrolled in the European RMS 2005 protocol from October 2005 to December 2016 at 31 Italian centers that are part of the Soft Tissue Sarcoma Committee (STSC). We grouped the centers by the number of patients each one enrolled (Group 1: >40; Group 2: 10; and Group 3

BACKGROUND Allograft venous thrombosis is a severe complication after kidney transplantation (KT). Early diagnosis and prompt treatment are crucial in preserving the survival of the allograft. In this study, we aimed to describe an emergent strategy for the management of acute allograft venous thrombosis. CASE PRESENTATION A 4-year-old girl, weighing 13.5 kg, was diagnosed with bilateral congenital renal hypodysplasia, urogenital sinus and anorectal malformation. The patient was referred to our department for living-donor KT. Her mother was eligible as a donor, presenting a body weight ratio of 1:4.5. Thrombosis of the inferior vena cava (ICV) was also identified, without any predisposing factor for thrombophilia. KT was performed by an extraperitoneal approach without complications. Venous anastomosis required a human vascular graft sutured to the ICV, and renal artery was anastomosed to the aorta. On postoperative day (POD) 8, acute abdominal pain and hematuria led to the diagnosis of an allograft venous thrombosis. An emergent laparotomy was required to explant the allograft, followed by bench surgery. The allograft was irrigated with thrombolytic agents and lactated Ringer's solution and then after removing the venous vascular graft, it was reimplanted through vascular anastomosis with the ICV and aorta. The recovery of perfusion and function was good with diuresis since day 4 after re-surgery. At 2-year follow-up, the child presented normal allograft function with an estimated GFR of 65 ml/min/1.73 m2 . CONCLUSION According to our experience, explantation of the kidney allograft, followed by irrigation with thrombolytics in bench surgery, and reimplantation resulted in unexpected optimal outcomes in the case of allograft venous thrombosis.

Neuroblastoma (NB) is the most common extracranial, solid, pediatric malignancy and, despite the constant progress of treatment and development of innovative therapies, remains a complex, challenging disease causing major morbidity and mortality in children. There is significant variability in the management of neuroblastoma, partially due to the heterogeneity of the clinical and biological behavior, and partially secondary to the different approaches between treating institutions. Anesthesia takes an integral part in the multidisciplinary care of patients with NB, from diagnosis to surgery and pain control. This paper aims to review and discuss the critical steps of the perioperative and operative management of children undergoing surgery for neuroblastoma. Anesthesia and analgesia largely depend on tumor location, surgical approach, and extension of the surgical dissection. Attention should be paid to the physio-pathological changes on cardiovascular, gastrointestinal, and immune systems induced by the tumor or by chemotherapy. At the time of surgery meticulous patient preparation needs to be carried out to optimize intraoperative monitoring and minimize the risk of complications. The cross-sectional role of anesthesia in cancer care requires effective communication between all members of the multidisciplinary team.

Background Irinotecan is a drug active against pediatric sarcomas with a toxicity profile that theoretically allows for its association with more myelotoxic drugs. We examined the feasibility of a dose-density strategy integrating irinotecan in standard chemotherapy regimens for patients with high-risk sarcomas. Methods Between November 2013 and January 2020, 23 patients ≤25 years old were included in the study. Eleven patients newly diagnosed with metastatic disease received nine cycles of IrIVA (irinotecan-ifosfamide-vincristine-actinomycin D; ifosfamide 3 g/m2 on days 1 and 2, vincristine 1.5 mg/m2 on day 1, actinomycin D 1.5 mg/m2 on day 1, irinotecan 20 mg/m2 for 5 consecutive days starting on day 8) as first-line therapy. Two relapsed patients received IrIVA and 10 IrVAC (irinotecan-vincristine-actinomycin D-cyclophosphamide; cyclophosphamide 1.5 g/m2 on day 1 instead of ifosfamide). Feasibility was assessed in terms of toxicity and time to complete the treatment. Results Seventeen rhabdomyosarcomas, four Ewing sarcomas, two desmoplastic small round cell tumors received a total of 181 cycles (range 2-10). Grade 4 neutropenia occurred in 62.4% of the cycles. Thirteen patients had febrile neutropenia. Diarrhea occurred in 14 cycles. The median time to complete the treatment was 195 days (range 170-231), 83.4% of cycles were administered on time or with a delay Conclusions A dose-density strategy combining irinotecan with standard chemotherapy is feasible. This approach will be investigated in the next trial coordinated by the European pediatric Soft tissue sarcoma Study Group.

Extraperitoneal approach is sometimes recommended for kidney transplantation (KT) in children weighting15 kg. We hypothesized that this approach might be as successful as in patients with normal weight. Data of all consecutive KTs performed between 2013 and 2019 were retrospectively reviewed. Early outcomes and surgical complications were compared between children weighing ≤15 kg (low-weight (LW) group) and those weighing15 kg (Normal-weight (NW) group). All the 108 KTs were performed through an extraperitoneal approach. The LW group included 31 patients (mean age 3.5 ± 1.4 years), whose mean weight was 11.1 ± 2.0 kg. In the LW group,-a primary graft nonfunction (PNGF) occurred in one patient (3.2%), surgical complications occurred in nine (29%), with four venous thrombosis. In the NW group, PNGF occurred in one case (1.3%), delayed graft function (DGF) in eight (10%), surgical complications in 11 (14%) with only one case of venous thrombosis. In both groups, no need for patch during wound closure and no wound dehiscence were reported. The extraperitoneal approach can be effectively used in LW children. No differences were observed in the overall complication rate (P = 0.10), except for the occurrence of venous thrombosis (P = 0.02). This might be related to patients' characteristics of the LW group.

Pediatric adrenocortical tumors (ACT) are rare and sometimes aggressive malignancies, but there is no consensus on the outcome predictors in children. A systematic search of MEDLINE, SCOPUS, Web of Science, and the Cochrane Library for studies from 1994 to 2020 about pediatric ACT was performed. In 42 studies, 1006 patients, aged 0-18 years, were included. The meta-analyses resulted in the following predictors of better outcome: age

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

The current pandemic SARS-CoV-2 has required an unusual allocation of resources that can negatively impact chronically ill patients and high-complexity procedures. Across the European Reference Network on Pediatric Transplantation (ERN TransplantChild), we conducted a survey to investigate the impact of the COVID-19 outbreak on pediatric transplant activity and healthcare practices in both solid organ transplantation (SOT) and hematopoietic stem cell transplantation (HSCT). The replies of 30 professionals from 18 centers in Europe were collected. Twelve of 18 centers (67%) showed a reduction in their usual transplant activity. Additionally, outpatient visits have been modified and restricted to selected ones, and the use of telemedicine tools has increased. Additionally, a total of 14 COVID-19 pediatric transplanted patients were identified at the time of the survey, including eight transplant recipients and six candidates for transplantation. Only two moderate-severe cases were reported, both in HSCT setting. These survey results demonstrate the limitations in healthcare resources for pediatric transplantation patients during early stages of this pandemic. COVID-19 disease is a major worldwide challenge for the field of pediatric transplantation, where there will be a need for systematic data collection, encouraging regular discussions to address the long-term consequences for pediatric transplantation candidates, recipients, and their families. info:eu-repo/semantics/publishedVersion

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

Here we report the case of a disseminated Aspergillus flavus infection in an adolescent affected by acute lymphoblastic leukaemia at the beginning of first-line chemotherapy. Association of surgery and combination antifungal therapy (high dose liposomal amphotericin B and caspofungin) allowed infection improvement. 18-FDG PET-MRI was used for response-to therapy monitoring.

We evaluated an otherwise healthy, full-term female newborn for a soft, non-tender swelling of the left labium majus (figure 1). Ultrasound scan at birth confirmed the absence of indwelling intraperitoneal structures, ruling out the hypothesis of inguinal hernia. A 2-week follow-up ultrasound revealed a hypoechoic/isoechoic tissue with hyperechoic striae in its context. Doppler ultrasound imaging at 6 and 12 months excluded vascular malformations. The baby …

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.

Gas embolism is a possible complication during head and neck surgery and laparoscopic procedures. We are presenting our experience of a young girl who underwent a laparoscopic biopsy of the hepatic hilum lymph-nodes for a suspected lymphoma recurrence. Due to her previous clinical history, the patient carried a central venous catheter (CVC). After 20 min from the beginning of the laparoscopic procedure, clinical symptoms were noticed: resuscitation maneuvers were immediately performed and foamy blood was aspirated from the CVC. After the patient was stabilized, the procedure was converted into laparotomy and concluded. Gas embolism is a described complication during laparoscopic procedures, but it is very rare in children where only 5 cases have been described, none registering the death of the patient.

Background Sentinel Node Biopsy is an established staging technique in many adult malignancies. However, only few reports describe this procedure for the evaluation of regional lymph nodes in childhood and adolescents. Our experience with sentinel node biopsy in soft tissue sarcomas of extremities in children is reported. Methods Seventeen children were evaluated with sentinel node biopsy between 2002 and 2007: 11 at initial surgery, 5 at primary re-excision, 1 at local relapse. The diagnosis was rhabdomyosarcoma in 5 and other soft tissue sarcomas in 12: Ewing/PNET sarcoma 6, epithelioid sarcoma 1, malignant peripheral-nerve-sheath tumor 1, undifferentiated sarcoma 1, myxoid liposarcoma 2, adult-type fibrosarcoma 1. Primary sites included lower limbs (8), upper limbs (9). Mapping of nodes was performed with lymphoscintigraphy plus intra-operative injections with blue-dye in 14 cases, with lymphoscintigraphy and intra-operative injections alone in 2 and 1, respectively. Results Of the 17 lymphatic regions (9 axilla, 8 inguinal), 16 were identified with lymphoscintigraphy, 15 by intra-operative injections. Thirty-five lymph nodes were removed. Nodes were positive for metastasis in two patients with alveolar rhabdomyosarcoma and undifferentiated sarcoma. There were no complications. No further lymph node metastases were recognized either at diagnosis or during the follow-up (6–78 months). Conclusions Sentinel node biopsy was technically feasible, reliable, and free of complications. It could be an alternative to aggressive or random biopsies for extremity rhabdomyosarcoma and it can contribute to define those non-rhabdomyosarcoma soft tissue sarcomas that spread to regional nodes. Pediatr Blood Cancer 2009;52:51–54. © 2008 Wiley-Liss, Inc.

Background Invasive aspergillosis (IA) is a serious problem in patients suffering from hematological malignancies. Surgical resection has been reported to improve disease control and patient survival. There are few reports describing the role of surgery in children with pulmonary IA. Procedure From October 1998 to September 2005, 21 patients fulfilled the inclusion criteria. Demographic and clinical data, as well as type and duration of antifungal therapy; surgery and related complications; time elapsing from surgery to resumption of chemotherapy were collected retrospectively through a specially designed form filled in by each investigator. Results Eleven males and 10 females, aged between 2 and 17 years underwent one or more surgical lung resections for diagnostic and therapeutic purposes. Surgical complications were reported in three patients. Two patients, who underwent a wedge resection and a lobectomy, respectively, had no fungal lesions detected at surgery. Seventeen of 20 patients with malignancy resumed chemotherapy after a median of 19 days from surgery, range 7–81, and 11 of them underwent hematopoietic stem cell transplantation after a median time of 60 days from surgery, range 19–110. After a median follow-up of 1.7 years, 12 patients are alive while 9 patients have died from progression of their underlying disease. Conclusions This study suggests that the combination of medical antifungal therapy and early surgical excision is a feasible and an effective strategy in pediatric patients with IA. In order to avoid unnecessary surgical procedures, we advise checking the response to antifungal therapy by chest-computed tomography immediately before the date of surgery. Pediatr Blood Cancer 2007;49:909–913. © 2006 Wiley-Liss, Inc.

Sentinel lymph node biopsy (SLNB) is an established staging technique in many malignancies, but reports describing this procedure for the evaluation of regional lymph nodes in childhood and adolescents are still scarce. SLNB has progressively gained a role in the staging of pediatric melanoma and some histotypes among soft tissue sarcomas (rhabdomyosarcoma, synovial sarcoma, epithelioid sarcoma, and clear cell sarcoma). We report our experience with 29 patients who were evaluated with SLNB during the past 12 years: 9 alveolar rhabdomyosarcoma, 1 embryonal rhabdomyosarcoma, 7 Ewing Sarcoma/pPNET, 1 clear cells sarcoma, 2 epithelioid sarcoma, 1 epithelioid sclerosing fibrosarcoma, 3 myxoid liposarcoma, 1 adult fibrosarcoma, 1 malignant peripheral nerve sheath tumor, 1 undifferentiated sarcoma, 1 sarcoma not otherwise specified, and 1 melanoma. All, but one, patients underwent a lymphoscintigraphy, and in more than half of the patients, an intraoperative lymphatic mapping with a blue dye injection was performed. In our experience, SLNB was a feasible and reliable technique that allowed a targeted sampling of regional lymph nodes. This technique avoids aggressive operations or random biopsies, leading to an accurate staging without complications. Recently new imaging techniques, such as positron emission tomography–computed tomography scan, have obtained good results in identifying distant and lymphatic metastases, but they have been found to be less reliable than SLNB, especially for small volume metastatic nodal disease in sarcoma.

Background The surgical approach to masses located in the cervicothoracic juncton represents a challenge for surgeons. Many techniques have been described with good results. Methods We analyzed and compared the results obtained in 2 Italian pediatric surgery centers using 2 different techniques in patients with tumors of the thoracic inlet: center 1, using anterior cervical transsternal approach on 7 patients, and center 2, applying "trap-door" technique on 5 patients. Results Excision was incomplete in 5 patients and complete in 7 patients. Histologic examination revealed 5 patients with neuroblastoma; 3, ganglioneuroblastoma; 1, mixoid liposarcoma; 1, desmoid fibromatosis; 1, Castleman disease; and 1, Schwann cell tumor. The median duration of the procedure was 345 minutes in center 1 and 245 minutes in center 2. The median blood loss was 200 mL in both centers. The median hospital stay was 11 days in center 1 and 9 days in center 2. Globally, 5 patients developed postoperative complications. No significant differences were encountered comparing the main surgical outcome parameters between the 2 approaches. Conclusions Both techniques resulted in valid options to achieve a safe excision of thoracic inlet masses with a manageable complication rate and acceptable hospital stay. Surgical risk factors should be carefully investigated preoperatively. Postoperative pain control is important to guarantee early recovery.

Introduction Malignant sacrococcygeal (SC) germ cell tumours (GCT) may be diagnosed as primary pelvic tumour or malignant recurrence of foetal SC teratoma (FSCT) operated during the neonatal period. In order to evaluate the difference between these two populations, the authors report their experience with SC-GCT registered in the French TGM 95 protocol. Population and methods The protocol comprised risk-adapted-chemotherapy (CT) followed by surgery. Standard risk (SR: localized tumour completely resected) had no adjuvant therapy. Intermediate-Risk (IR: localized tumour, incomplete or no initial surgery with αFP 15,000 ng/ml and/or metastases) received Etoposide–Ifosfamide–Cisplatin. Results Fifty-seven patients with SC-GCT, aged 0–80 months (median 16), were registered between 1995 and 2005. Nineteen patients had secondary SC-GCT after FSCT. All patients received CT: 17 IR and 1 SR after reevolution; 39 HR (25 with metastases). 51 patients underwent delayed surgery, which was incomplete in 8 patients. Evolution Seventy-two percent of the secondary SC-GCT had systematic biological follow-up. αFP increasing was the first presenting sign in 80% of the cases. Patients with secondary SC-GCT had a lower median αFP level at diagnosis, were less frequently classified as HR and received less CT. The two groups with secondary vs. primary SC-GCT had a statistically similar favourable outcome (Overall Survival: 93.8% vs. 86.2%; Event-Free Survival: 89.2 vs. 78.2%; p > 0.34 and >0.32), respectively, but with less burden of therapy. Conclusions SC-GCT has a good overall prognosis provided complete surgery is achieved and CT is administered to IR and HR patients. SC-GCT in patients followed by αFP after treatment for FSCT had less tumour extension than newly-diagnosed patients, probably because of earlier detection of the disease.

Patients with non-metastatic rhabdomyosarcoma (RMS) have a cure rate of 50-90%, but up to one-third of them experience mostly a local recurrence. Second-line treatment is not standardized as for newly diagnosed tumors. We evaluated the role of surgery on local relapses in a series of patients with RMS.This retrospective analysis involves 70 patients enrolled in two consecutives Italian Studies, RMS88 and RMS96, who presented local recurrence. After relapse, 40/70 underwent a surgical excision (Surgery Group, SG), that was demolitive in 10/40; 24/40 had radiotherapy, 16/40 did not receive radiotherapy or data are not known. Thirty patients out of 70 did not receive any surgical treatment (No-Surgery Group, NSG), and 20/30 received radiotherapy.Overall survival (OS) after local relapse was 41.6% (mean follow-up 59 months, range 1-226). OS of SG patients was 54% versus 24.7% of the NSG patients (P = 0.0117). Furthermore, OS among the SG was 61.4% with and 41.8% without radiotherapy, and 37.1% with and 0% without radiotherapy among the NSG (P0.0001). One patient developed a second local relapse after excision without radiotherapy for the first one, and was cured with further treatment. Demolitive surgery did not improve survival compared to conservative surgery (40% vs. 58.4%, P = 0.1462).The treatment of recurrent RMS represents a challenge. In our experience, patients with local relapse had a poor prognosis. SG patients had a better outcome than NSG patients and those treated with resection plus radiotherapy had the best outcome; patients who did not receive any local treatment had an unfavorable outcome.

Vaginal strictures are rare entities in pediatric population, mainly due to congenital genito-urinary tract malformations and sequelae of their surgical treatment, recurrent vaginitis and multisciplinary therapy for malignant tumors. The therapy of choice is not standardized. Conservative treatments are favored and dilators seem to be very effective, but their use in children is difficult due to poor compliance. We report our experience with topical application of mitomycin-C in a female with vaginal stricture secondary to brachytherapy.

BACKGROUND. The purpose of the current study was to analyze the influence of the initial surgical approach (biopsy vs resection with macroscopic residual tumors) on the outcome of patients with localized Intergroup Rhabdomyosarcoma Study (IRS) Group III rhabdomyosarcoma (RMS) enrolled in the Italian studies between 1979 and 2003. METHODS. Among the 394 patients evaluated, 323 underwent biopsy, as recommended by the protocols, and 71 patients underwent surgical resection with macroscopic residual tumors (debulking operation [DO]), although this procedure was discouraged. All these patients were classified at the same risk group and received the same treatment. The different characteristics (patient age, tumor site, T classification and size, histology) and outcome in the 2 groups were considered. RESULTS. The estimated 5-year overall survival (OS) rates were 68.4% and 72.6%, respectively, after biopsy and DO(P = .38), and the rates of progression-free survival (PFS) were 56.5% and 61.7%, respectively, after biopsy and DO (P = .41). The outcome did not differ significantly when considering other variables such as tumor site, size, and histology. Age > 10 years appeared to have little benefit in patients with DO; the OS was 62% after biopsy and 83.1% after DO (P = .06); the PFS was 49.7% and 72.8%, respectively after biopsy and DO (P = .04). No surgical complications due to the 2 procedures were reported, but in 2 cases the initial DO resulted in a mutilation. CONCLUSIONS. No significant advantages of DO versus biopsy were detected with regard to patient outcome. Biopsy, which is less aggressive, appears to be thebest option for patients with IRS Group III RMS. Cancer 2007. © 2007 American Cancer Society.