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ICPACT is a collaborative platform that aims to integrate ACT researchers and institutions to advance pediatric ACT research.

The initiative led by Bonald C. Figueiredo (Pequeno Principe Hospital and Research Institute, Curitiba, Brazil), is focused on mapping new members who are dedicated to integrating pediatric ACT research, securing funding for a structured ICPACT website, periodical Workshops hosted by each member and organizing multi-institutional projects.

Under new leadership by Camila Daiggi (starting in November 2024) the goals include launching "The Stage II Project" at the Boldrini Research Center (Campinas – SP, Brazil), implementing the REDCap Boldrini system, convening all ACT researchers and families in an international workshop at the Boldrini Center in November 2024 sponsored by CNRS-IPMC (PI Enzo Lalli), and empowering members to coordinate in different departments (pre-clinical research, clinics, genetics, pathology, epidemiology, etc).

THE INTERNATIONAL CONSORTIUM ON PEDIATRIC ADRENOCORTICAL TUMOR

Summary of the ICPACT Plenum on June 16th, 2023, from 7PM to 8:20PM

We (Ana Prcicote, Bonald Figueiredo, Camila Daiggi, Carmem Fiori, Emília Pinto, Karla Rodrigues, Lauro Souza, Leila Grisa, Mara Pianovski, Raul Ribeiro, Simoni Abib, Tatiana El Jaick, Verena Wiegering, Vilani Kremer) from 9 institutions and 3 countries met on this day to discuss our agenda. We decided to keep a memory of our activies and decisions by storing all important files and each Plenum Report in our future independent ICPACT hot site expected to be on-line within one month (access available with login and password). We considered as Foundering Members the Colleagues (Table 1) who had the opportunity to reply to the questions of the Survey. However, to the best interest of the pediatric adrenocortical tumor patients we welcome new members from different areas and countries to join this ICPACT. Radiologists and other specialists are needed in this ICPACT.

In this Plenum we reviewed all questions of the Survey (available in the hot site) and decided to maintain as an InternaIonal ConsorIum. Since the largest number of pediatric ACT cases (~approximately 6 cases/year) and a total number over 120 cases are from Centro InfanIl Boldrini in Campinas, we follow the decision of the majority of the respondents, where the first author (Camila Daiggi) will first submit the research proposal on stage II cases to the Ethics Committee in Boldrini and later to the other Institutions. The majority decided to split stage II in two levels (IIA and IIB) but it is still necessary to define the parameters for IIA and IIB. For this objective, we collectively need to edit the EXCEL FILE with the proposed stage II parameters in a Google DRIVE (hUps://docs.google.com/spreadsheets/d/1kbl8WLI5IYIj- lKDveYpoGuuVGdc4vg8rEE_83am0v8/edit?usp=sharing). In this drive each Member should write his/her name for suggestions entered in each column. Ideally, we should have at least 3 Pathologists to help in the histopathological analyses of retrospective cases of stage (IIA and IIB), and the suggested names were Pathologists from Boldrini, Pequeno Príncipe and Erastinho Hospital. For Southern Brazil is important to search somatic or germline mutations in paraffin tissue sections (4 µm tissue sections in a tube) first for TP53 p.R337H in all ACTs, and if positive, we also genotype XAF1 p.E134*. All cases not yet genotyped may be sent to Bonald C. Figueiredo in Curitiba (Av. Munhoz da Rocha, no. 490, Bairro Cabral, Curi9ba-PR, Brazil, 80035-000) and when are negative for p.R337H they may be sent for whole P53 gene sequencing, possibly by Gerard Zambetti’s laboratory in St. Jude Children’s Research Hospital, Memphis, TN, USA under responsibility of Emília Pinto. The second objective established in this Consortium was approved by all respondents (i.e., to monitor mitotane and its metabolites in pediatric and adult patients using the 2D chromatography by the Lab of Lauro Souza at Pequeno Príncipe Research Institute filling out the presented form to send samples to Curitiba). And finally, the group decided to have a fixed date and time for future ICPACT Meetings. Given the difficulty to find the best day for everybody, we may test the fixed day and time suggested by Mara Pianovski, as THE SECOND WEDNESDAY OF EACH MONTH AT 5PM (Brazilian time). That said, our next meeting is July 12th at 5PM and we will again discuss the best day and time for these meetings. I, Bonald C Figueiredo, wrote this report to the best of my knowledge.

Curitiba, June 16th, 2023
Bonald C. Figueiredo, MD, PhD ICPACT Chairperson
Pelé Pequeno Príncipe Research Institute, Scientific Director Coordinator of the PhD Program in Biotechnology Applied to Pediatrics Faculdades Pequeno Príncipe

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