ABSTRACT
The PARTNER project (Paediatric Rare Tumours Network - European Registry) was launched in 2016. PARTNER aims to create a European Registry dedicated to children and adolescents with very rare tumors (VRT). It links existing national registries and provides a registry for those countries in which a VRT registry has not yet been created. This consortium is composed of the various national cooperative groups and their respective member institutions. The strategic value of this project is based on the Europe-wide data collection concerning the treatment of VRTs. These data are provided to experts and constitute the basis for new clinical practice guidelines for use by ERN (European Reference Network) and non-ERN institutions. The proposed tasks and milestones will increase collaboration in the field of pediatric oncology among member states and will also facilitate the inclusion of low health expenditure average rate (LHEAR) countries in this process. In addition, this project creates a platform for VRTs that may represent a model on how to elaborate a comprehensive approach (case registration, international case consultation and treatment recommendations, and website to provide information for parents/patients) for rare diseases.
Subject(s)
Neoplasms , Adolescent , Child , Europe/epidemiology , Humans , Medical Oncology , Neoplasms/therapy , Rare Diseases/epidemiology , Rare Diseases/therapy , RegistriesABSTRACT
Nasopharyngeal carcinoma (NPC) is a rare pediatric tumor. Collaborative studies performed over the last decades showed improved results compared to historical data, but standardized guidelines for diagnosis and management of pediatric NPC are still unavailable. This study presents a European consensus guideline for the diagnosis and treatment of pediatric NPC developed by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT). Main recommendations include induction chemotherapy with cisplatin and 5-flurouracil, concomitant chemoradiotherapy in advanced disease, and to consider maintenance treatment with interferon beta (IFN-ß) for selected high-risk patients. Dose adjustments of radiotherapy based on response to induction chemotherapy may decrease the rates of long-term treatment-related complications that affect most of the survivors.
Subject(s)
Carcinoma , Nasopharyngeal Neoplasms , Adolescent , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Carcinoma/pathology , Chemoradiotherapy , Child , Cisplatin , Fluorouracil , Humans , Induction Chemotherapy , Nasopharyngeal Carcinoma/drug therapy , Nasopharyngeal Carcinoma/therapy , Nasopharyngeal Neoplasms/drug therapy , Nasopharyngeal Neoplasms/therapy , Neoplasm StagingABSTRACT
Although rhabdomyosarcoma is the most frequent soft tissue tumor in children, there are extremely few reports of this tumor arising from the urachus. The authors describe another case in a 6-year-old female associated with constipation and a painless suprapubic mass. The specimen had the pathological criteria used to define urachal sarcoma (cytological, histological, and immunohistochemical findings) and urachal remnants were not observed. After complete resection of the primary tumor the patient was treated with chemotherapy (ifosfamide, vincristine, and dactinomycin) and remains alive at 4 years' follow-up with no signs of recurrence. Nowadays the management of children with urachal rhabdomyosarcoma usually includes surgery and chemotherapy. Multimodal therapy and surgery are constantly evolving and have significantly improved overall survival of these patients especially in R0 resected patients.
