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Cancers Jul 2021Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare pediatric neoplasm of neural crest origin. In most cases, it develops in infants as a localized tumor of... (Review)
Review
Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare pediatric neoplasm of neural crest origin. In most cases, it develops in infants as a localized tumor of the maxilla, and surgery is usually curative. In less than 10% of patients with inoperable, metastatic or persistently recurring MNTI, chemotherapy (CHT) may be considered; however, its role is still unclear. The aim of our study was to assess the efficacy of CHT in children with large, inoperable, metastatic and/or recurrent MNTI. Four such infants, treated with CHT in Polish and German centers of pediatric oncology, were presented. Additionally, a systematic literature search of the PubMed/MEDLINE, Scopus and Web of Science databases was performed, yielding 38 similar cases within the last 42 years. Neoadjuvant CHT, based mainly on the protocols for neuroblastoma, was often effective, allowing for complete delayed surgery in most cases. However, the role of adjuvant CHT in preventing recurrences after incomplete resection of MNTI remains unclear. Disseminated inoperable MNTI was almost universally associated with poor response to CHT and unfavorable outcome. Further investigations to elaborate standards of management in patients with inoperable, metastatic or persistently recurring MNTIs are necessary to improve outcomes.
PubMed: 34359769
DOI: 10.3390/cancers13153872 -
Anales de Pediatria (Barcelona, Spain :... Jul 2005NB is the most frequent pediatric cancer arising in the sympathetic nervous system and represents a serious healthcare challenge because: 1) it is the most frequent... (Review)
Review
INTRODUCTION
NB is the most frequent pediatric cancer arising in the sympathetic nervous system and represents a serious healthcare challenge because: 1) it is the most frequent neoplasm in the first decades of life; 2) it biological behavior is unpredictable (spontaneous regression, maturation to ganglioneuroma, and localized and metastasized variants); and 3) little is known about most of the risk factors involved in its etiopathogenesis. The objective of this study was to disseminate knowledge of constitutional and environmental (physical, chemical, biological and social) risk factors linked to the development of neuroblastoma (NB), with various levels of scientific evidence. To seek collaboration among pediatricians in the research project "Environment and Pediatric Cancer".
MATERIAL AND METHODS
We performed a systematic review of the literature published in the previous 25 years on risk factors for NB diagnosed in the first two decades of life, using Medline, the Science Citation Index and Embase. Search profiles were: "neuroblastoma/childhood sympathetic nervous system neoplasms and risk factors/etiology/epidemiology". The most interesting articles and the most relevant references contained therein were selected.
RESULTS
With greater or lesser scientific evidence, the following risk factors increase the risk of developing NB: genetic factors; geographic factors; ethnic factors; socioeconomic factors; infectious factors; physical factors; parental occupational exposure; gestational factors; and perinatal and maternal factors. Preventive factors associated with a lower risk of developing NB are breastfeeding and intake of vitamin supplements during pregnancy.
CONCLUSIONS
The main barriers to the identification of evidence-based risk factors involved in the development of NB are its complex biology and clinical course, its relative rarity and the difficulty of performing epidemiological studies. Research on constitutional and environmental factors involved in its etiopathogenesis should be stimulated. The best preventive strategy is to recommend breastfeeding for more than 6 months.
Topics: Adolescent; Child; Child, Preschool; Humans; Infant; Neuroblastoma; Risk Factors
PubMed: 15989872
DOI: 10.1157/13076768 -
Cancer Medicine Jul 2020Regular off-treatment imaging is often used to assess for recurrence of disease after childhood cancer treatment. It is unclear if this increases survival, or what...
BACKGROUND
Regular off-treatment imaging is often used to assess for recurrence of disease after childhood cancer treatment. It is unclear if this increases survival, or what burden surveillance places on patients, families, or health-care services. This systematic review examines the impact of routine surveillance imaging after treatment of pediatric extracranial solid tumors.
METHODS
Collaborative patient and public involvement informed the design and interpretation of this work. Thirteen electronic databases, conference proceedings, and trial registries were searched alongside reference list checking and forward citation searching from 1990 onwards. Studies were screened and data were extracted by two researchers. Risk of bias was assessed using a modified ROBINS-I tool. Relevant outcomes were overall survival, psychological distress indicators, number of imaging tests, cost-effectiveness, and qualitative data regarding experiences of surveillance programs. PROSPERO (CRD42018103764).
RESULTS
Of 17 727 records identified, 55 studies of 10 207 patients were included. All studies used observational methods. Risk of bias for all except one study was moderate, serious, or critical. Data were too few to conduct meta-analysis; however, narrative synthesis was performed. Surveillance strategies varied, and poorly reported, involving many scans and substantial radiation exposure (eg, neuroblastoma, median 133.5 mSv). For most diseases, surveillance imaging was not associated with increased overall survival, with the probable exception of Wilms tumor. No qualitative or psychological distress data were identified.
CONCLUSIONS
At present, there is insufficient evidence to evaluate the effects of routine surveillance imaging on survival in most pediatric extracranial solid tumors. More high-quality data are required, preferably through randomized controlled trials with well-conducted qualitative elements.
Topics: Diagnostic Imaging; Female; Humans; Male; Neoplasms; Population Surveillance; Survival Analysis
PubMed: 32431088
DOI: 10.1002/cam4.3110 -
Anales de Pediatria (Barcelona, Spain :... Aug 2006Neonatal tumors, which represent only 1.5-2% of all pediatric tumors, have distinctive features. Their incidence is imprecise, as selection criteria vary and there are... (Review)
Review
BACKGROUND
Neonatal tumors, which represent only 1.5-2% of all pediatric tumors, have distinctive features. Their incidence is imprecise, as selection criteria vary and there are few published studies.
OBJECTIVE
To analyze and disseminate findings on the histological, clinical, therapeutic and follow-up characteristics of neonatal tumors.
MATERIAL AND METHODS
We performed a retrospective study of the clinical records of patients diagnosed with neonatal tumors in La Fe University Children's Hospital in Valencia (Spain) between January 1990 and December 1999. Hamartomas, flat and cavernous cutaneous hemangiomas, nevi, lipomas, subcutaneous fibroma, lymphangiomas, and epidermal cysts were excluded. A systematic review of the literature published in the previous 25 years was performed using Medline, Cancerlit, Index Citation Science, and Embase. The search profile combined neonatal or congenital and tumor or cancer or neoplasm. The most interesting studies, as well as the most relevant references contained in these studies and published before the search period, were selected.
RESULTS
The clinical records of 72 patients with neonatal tumors (40 boys and 32 girls), representing 2.8% of all pediatric tumors, were reviewed. The most frequent tumors were hemangiomas (20.8%, 15 patients), neuroblastomas (16.7%, 12 patients), teratomas (12.5 %, 9 patients), and soft tissue tumors (9.7 %, 7 patients). Eighty-six percent of the patients were symptomatic during the first week of life. Although diagnosis was prenatal in 22.2 % of the patients, the most frequent findings on physical examination were identification of a mass or cutaneous lesion in 24 patients (33.3%) and an abdominal mass or hepatomegaly in 13 patients (18%). An associated disease, malformation or syndrome was found in 15 patients (20.8 %). Treatment included surgery (50% of patients) and drugs as monotherapy or coadjuvant therapy (13.9%). Thirteen patients received irradiation after the neonatal period. Spontaneous complete remission took place in six patients. Twenty patients died (27.8%). Mortality was highest in patients with central nervous system tumors or leukemias (83.3% and 75 % respectively). By contrast, none of the patients with hemangiomas or teratomas died. Mortality was low in patients with neuroblastoma (8.3%). At the time of the study, survival was 73 %, with a median follow-up of 8 years.
CONCLUSIONS
Due to their biological features, neonatal tumors represent a distinctive subgroup in pediatric oncohematology. The concept of neonatal tumor should be unified to allow the results of different research groups to be analyzed and compared. Despite the methodological limitations found, the clinical, diagnostic, therapeutic, and follow-up characteristics of our patients are similar to those of other published series. The differences found could be explained by the diverse selection criteria employed.
Topics: Female; Hospitals, Pediatric; Hospitals, University; Humans; Infant, Newborn; Male; Neoplasms; Retrospective Studies; Spain
PubMed: 16948973
DOI: 10.1157/13091478