Propofol-based palliative sedation in terminally ill children with solid tumors: A case series.

Rationale: The palliative sedation therapy is defined as the intentional reduction of the alert state, using pharmacological tools. Propofol is a short-acting general anesthetic agent, widely used for induction and maintenance of general anesthesia and rarely employed in palliative care.

Patient Concerns And Diagnoses: This case series describes 5 pediatric oncology inpatients affected by relapsed/refractory solid tumors received palliative sedation using propofol alone or in combination with opioids and benzodiazepines.

NRAS(Q61K) mutated primary leptomeningeal melanoma in a child: case presentation and discussion on clinical and diagnostic implications.

Background: Primary melanocytic neoplasms are rare in the pediatric age. Among them, the pattern of neoplastic meningitis represents a peculiar diagnostic challenge since neuroradiological features may be subtle and cerebrospinal fluid analysis may not be informative. Clinical misdiagnosis of neoplastic meningitis with tuberculous meningitis has been described in few pediatric cases, leading to a significant delay in appropriate management of patients.

Transient global ventricular dysfunction in an adolescent affected by pancreatic adenocarcinoma.

Background: Takotsubo cardiomyopathy (TC) is characterized by a transient decrease in ejection fraction and a reversible left ventricular dysfunction. The pathophysiology of TC is not completely understood. Heterogeneous and multifactorial mechanisms are involved: drugs, emotional and physical stress, genetic and hormonal factors.

Comparison of two different conditioning regimens before autologous transplantation for children with high-risk neuroblastoma.

Background: Although high-dose chemotherapy (HDC) represents the standard of treatment for high-risk neuroblastoma (NBL), the most effective conditioning regimen still remains to be identified.

Patients And Methods: Forty-one high-risk NBL entered into local protocol based on induction chemotherapy, surgery and HDC with either etoposide/thiotepa/cyclophophamide (ETC) or i.v.

Expression of multidrug resistance-associated proteins in paediatric soft tissue sarcomas before and after chemotherapy.

Expression of multidrug resistance (MDR) proteins is thought to significantly contribute to the different biological/clinical behaviour of soft tissue sarcomas (STS) of various histological types and clinicopathological stages, as they are responsible for active efflux of cytotoxic drugs from tumour cells. We investigated the expression of 3 MDR proteins, i.e.

Bevacizumab in pediatric patients: how safe is it?

Background: Bevacizumab is a monoclonal antibody directed against vascular endothelial growth factor (VEGF). The safety profile of bevacizumab was evaluated in a cohort of children with either recurrent or poor-prognosis malignancies.

Patients And Methods: Bevacizumab was administered intravenously at the dosage of 5-10 mg/kg every 14-28 days alone or in combination with other agents.

Rhabdomyosarcoma in adolescents: a report from the AIEOP Soft Tissue Sarcoma Committee.

Background: In many types of cancer, the survival rates are reported to be less favorable for adolescents compared with younger children. To investigate whether this is true for adolescents with rhabdomyosarcoma (RMS), the results obtained in patients enrolled in protocols run by the Italian Soft Tissue Sarcoma Committee (STSC) were analyzed.

Methods: From 1988 through 2005, 643 patients were registered (567 children ages birth-14 years and 76 adolescents ages 15-19 years) and treated in 4 STSC protocols.

Microscopic neoplastic thrombosis in localised nephroblastoma: does it influence outcome?

Introduction: Microscopic neoplastic thrombosis (MNT) is reported to occur frequently in Wilms tumour (WT). The aim of this study is to determine whether MNT influences prognosis in localised WT.

Patients And Methods: Records and slides of 80 consecutive, unselected, localised WT patients were retrospectively reviewed.

Sequential high-dose chemotherapy for children with metastatic rhabdomyosarcoma.

Aim: The RMS4.99 study was designed to explore the role of multiple sequential high-dose chemotherapy cycles administered early in the treatment of children with metastatic rhabdomyosarcoma.

Patients And Methods: Seventy patients were enrolled and received three cycles of initial standard chemotherapy, followed by a course of cyclophosphamide and etoposide to obtain peripheral blood stem cells (PBSC), then three consecutive high-dose combinations followed by PBSC rescue.

Rhabdomyosarcoma associated hypertrophic osteoarthropathy in a child: detection by bone scintigraphy.

Hypertrophic osteoarthropathy (HOA) is characterized by digital clubbing, long bone periosteal reaction, and polyarthralgias. Primary familial HOA is very rare and is not associated with underlying disorders and has a good prognosis. Secondary pediatric nonneoplastic HOA is associated with cystic fibrosis, congenital heart disease, biliary atresia, and inflammatory bowel disease.

Pancreatitis as an unusual presentation of rhabdomyosarcoma.

The most common etiologies of acute pancreatitis in children are trauma, multi-system disease, drugs, infections, idiopathic and congenital anomalies of the pancreaticobiliary system. Acute pancreatitis is rarely associated with underlying childhood malignancies. We report a 12-year-old male with acute pancreatitis as the presenting symptom of an alveolar metastatic rhabdomyosarcoma.

A multimodal strategy based on surgery, radiotherapy, ICE regimen and high dose chemotherapy in atypical teratoid/rhabdoid tumours: a single institution experience.

Purpose: Atypical Teratoid/Rhabdoid Tumour is a rare and aggressive childhood tumour. The outcome of a series treated with the same multimodal strategy was reported.

Patients: The patients were treated with surgery, 2 courses of ifosfamide/carboplatin/etoposide(ICE), 2 courses of cyclophosphamide/etoposide/carboplatino/thiotepa (CECAT) or 2 other ICE courses, high dose chemotherapy (HDC) and radiotherapy.

Clinical significance of CXC chemokine receptor-4 and c-Met in childhood rhabdomyosarcoma.

Purpose: The CXC chemokine receptor-4 (CXCR4)/stromal-derived factor-1 and c-Met/hepatocyte growth factor axes promote the metastatic potential of rhabdomyosarcoma cell lines in experimental models, but no data are available on their role in rhabdomyosarcoma tumors. The expressions of CXCR4 and c-Met were evaluated in primary tumors and isolated tumor cells in marrow, and were correlated with clinicopathologic variables and survival.

Experimental Design: Forty patients with recently diagnosed rhabdomyosarcoma were retrospectively enrolled.

Synovial sarcoma of children and adolescents: the prognostic role of axial sites.

Background: The outcome of patients with non-extremity synovial sarcoma (SS) is generally worse than that of patients with limb tumours.

Methods: The present study analysed a series of 115 consecutive SS patients treated in Italian paediatric protocols (period 1979-2005), mainly focusing on the 30 cases arising from 'axial' sites (16 head-neck, 8 trunk, 4 lung-pleura and 2 retroperitoneum).

Results: Initial gross resection was achieved in 40% of axial cases and in 80% of limb SS (p<0.

Pleuropulmonary blastoma, a distinctive neoplasm of childhood: report of three cases.

Background: Pleuropulmonary blastoma (PPB) is a rare, aggressive dysontogenetic neoplasm affecting children. It was identified as a distinct entity by Manivel in 1988 and later subdivided into three types on the basis of the histological pattern, with increasing malignancy from type I (cystic) through type II (solid/cystic) to type III (solid).

Objective: To report on the imaging findings, clinical presentation, and differential diagnosis, mainly cystic malformations.

High histologic and overall response to dose intensification of ifosfamide, carboplatin, and etoposide with cyclophosphamide, doxorubicin, and vincristine in patients with high-risk Ewing sarcoma family tumors: the Bambino Gesù Children's Hospital experience.

Background: Ewing sarcoma (ES) and extraosseous ES/primitive neuroectodermal tumors (PNET) share histopathologic features of the ES family of tumors (ESFT). The authors report on their results from a regimen of ifosfamide, carboplatin, and etoposide (ICE) with cyclophosphamide, doxorubicin, and vincristine (CAV) dose intensification in patients with high-risk ESFT.

Methods: Since 1990, patients with ESFT and with 1 or more of the following risk factors were reviewed: tumor volume > 200 mL, tumor site with a poor prognosis, and pulmonary and/or bone marrow metastases.

Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group.

Purpose: To assess the value of chemotherapy and radiotherapy in children with malignant peripheral nerve sheath tumors (MPNSTs) and to identify risk factors associated with outcome.

Patients And Methods: A total of 167 untreated eligible patients enrolled onto the Italian and German studies between 1975 and 1998 entered this analysis. Seventeen percent of patients had neurofibromatosis type 1 (NF1).

Antibiotic lock with vancomycin and urokinase can successfully treat colonized central venous catheters in pediatric cancer patients.

We used an antibiotic lock technique with vancomycin in combination with urokinase in 10 consecutive eligible children with Gram-positive catheter-related bacteremia persisting after appropriate intravenous antibiotics. Treatment was successful in sterilizing all colonized central venous catheters, avoiding device removal and delay of further chemotherapy. The antibiotic lock technique may represent a safe and effective therapeutic option in patients with selected, uncomplicated catheter-related bacteremias resistant to systemic antimicrobial therapy, particularly when maintaining a venous access is mandatory.

A multicenter, randomized, double blind placebo-controlled trial of amoxicillin/clavulanate for the prophylaxis of fever and infection in neutropenic children with cancer.

Aim Of The Study: To evaluate the effectiveness of oral amoxicillin/clavulanate (25 mg/kg every 12 h) for prevention of fever and/or infection in neutropenic children with cancer.

Methods: Multicenter, prospective, randomized, double blind placebo-controlled trial.

Results: In the intention-to-treat analysis, amoxicillin/clavulanate had a 12% benefit increase in terms of reduction in the incidence of febrile or infectious episodes, compared with placebo [44 of 83 (53%) vs.