Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma

Very rare tumors (VRTs) account for up to 11% of childhood cancers. Dedicated national groups and registries only exist in some European countries. Pleuropulmonary blastoma (PPB) is a very rare intrathoracic pediatric tumor with a potentially severe prognosis. Due to its rarity, it sometimes goes unrecognized. We investigated PPB diagnostic capability and possible correlations between diagnostic performance and VRT-dedicated activities. The number of cases of PPB registered between 2000 and 2014 at pediatric oncology centers in Europe was compared with the number of expected cases. Data sources included VRT registries, population-based cancer registries, and hospital registries. Data were obtained for 25 countries, grouped into 4 geographical regions. The expected cases were 111, and the observed cases were 129. The observed-to-expected ratio was 1.86 for Northern Europe, 1.33 for Southern Europe, 1.22 for Central Europe, and 0.65 for Eastern Europe. More cases than expected were registered in all countries with an official VRT registry. Conclusion: The number of cases observed is consistent with expectations, but disparities exist across Europe. Difficulties in diagnosing PPB emerged in most Eastern countries. The incidence rate of PPB may be underestimated. The creation of VRT-dedicated groups and a European Registry for VRTs could help to reduce inequalities.What is Known:\u2022 Very rare pediatric tumors are often not recognized, despite representing almost 11% of childhood cancers.\u2022 Pleuropulmonary blastoma is a rare pediatric tumor with a poor prognosis.What is New:\u2022 The ability to diagnose and register pleuropulmonary blastoma varies in Europe.Registries dedicated to very rare pediatric tumors improve the diagnostic rates.\u2022 The incidence rate of pleuropulmonary blastoma may currently be underestimate

Adrenocortical Tumors in Children and Adolescents: The European PARTN-ER Project for Consensus Guidelines Development.

Background and Aims: Adrenocortical carcinomas (ACC) are rare diseases. Several collaborative studies performed over the last decades showed improved results compared to historical data, but standardized guidelines for diagnosis andmanagement of pediatric ACC are still unavailable. Methods: We present European consensus guidelines developed by the Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) according to the ESMO scale, based on the evidence collected from published series, case reports and personal expertise. Results: ACT should be removed without rupture [Level V; Grade B]. Preferred option is open approach through a lateral transverse abdominal incision or a midline laparotomy in case of huge masses [Level IV; Grade B]. Mini-invasive surgery should be discouraged when volume exceeds 200cm3, suspicious regional nodal involvement and/or signs of local invasion are present [Level IV; Grade B]. All enlarged lymph nodes detected at radiology or intra-operatively should be removed [Level III; Grade A]. A systematic biopsy of the regional nodes should be performed, though they are not found suspicious pre- or intraoperatively [Level IV; Grade C]. Adjuvant therapy should be considered in advanced-stage ACT or in case of incomplete tumor resection [Level IV; Grade B]. First-line recommended regimen is CED (cisplatinum-etoposide-doxorubicin) plus mitotane [Level IV; Grade B]. For COG stage II tumors, mitotane is considered if several risk factors are associated: unfavorable histology (5-item score or Wieneke score >3), older age, hormonal secretion [Level IV; Grade C]. For unresectable tumors, gross/macroscopic residual disease, retroperitoneal lymph nodes involvement, CED with mitotane are considered [Level III; Grade B]. No data exist about the optimal duration of therapy. CED is usually scheduled in 6 to 8 cycles. Mitotane is continued for 1 to 2 years depending on patient\u2019s tolerance and compliance [Level IV; Grade C]. Conclusions: This European PARTN-ER project leads to a consensus strategy regarding the treatment of children and adolescents with ACC

Nasopharyngeal carcinoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

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-\u3b2) 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

Free radicals and antioxidants at a glance using EPR spectroscopy

The delicate balance between the advantageous and detrimental effects of free radicals is one of the important aspects of human (patho)physiology. The controlled production of reactive oxygen and nitrogen species has an essential role in the regulation of various signaling switches. On the other hand, imbalanced generation of radicals is highly correlated with the pathogenesis of many diseases which require the application of selected antioxidants to regain the homeostasis. In the era of growing interest for redox processes, electron paramagnetic resonance (EPR) spectroscopy is arguably the best-suited technique for such research due to its ability to provide a unique insight into the world of free radicals and antioxidants. Herein, I present the principles of EPR spectroscopy and the applications of this method in assessing: (i) the oxidative status of biological systems, using endogenous long-lived free radicals (ascorbyl radical (Asc(center dot)), tocopheroxyl radical (TO center dot), melanin) as markers; (ii) the production of short-lived radicals (hydroxyl radical (OH center dot), superoxide radical anion (O-2(-)), sulfur-and carbon-centered radicals), which are implicated in both, oxidative stress and redox signaling; (iii) the metabolism of nitric oxide (NO center dot); (iv) the antioxidative properties of various drugs, compounds, and natural products; (v) other redox-relevant parameter. Besides giving a comprehensive survey of up-to-date literature, I also provide illustrative examples in sufficient detail to provide a means to exploit the potential of EPR in biochemical/physiological/medical research. The emphasis is on the features and characteristics (both positive and negative) relevant for EPR application in clinical sciences. My aim is to encourage fellow colleagues interested in free radicals and antioxidants to expand their base knowledge or methods used in their laboratories with data acquired by EPR or some of the EPR techniques outlined in this review, in order to boost up the exciting area of redox science