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).

New therapies of ACC in preclinical and clinical trials

New anti-steroidogenic drugs: osilodrostat (Recordati) and the Orphagen new drug (no conflict of interest).

It is clear that we cannot design any immunotherapy clinical trial without full inhibition of the steroidogenesis. The successful cases treated with checkpoint inhibitors (nivolumab and Ipilimumab) were in patients with nonfunctional ACC (cases presented at Varandas Workshop).

Two metabolic profiles in R1 children


Inhibitory of checkpoints: Nivolumab and Ipilimumab

Unfortunately, we do not have yet the possibility to get these expensive antibodies from the Public System (in Brazil called SUS) because we need more scientific data to justify this for ACC patients. However, it is in our mind to support fundraising for use of this consortium. It is expected each Hospital could start fundraising campaigns to import Nivolumab and Ipilimumab.

It is expected that each Hospital could start fundraising campaigns to import nivolumab and Ipilimumab, however we could also (fostered by SOBOPE) negotiate directly with the industry.

Inhibition of checkpoints (without hipercortisolism): Nivolumab + Ipilimumab or Pembrolizumab

In Europe, immunotherapy is NOT reimbursed for either pediatric or adult ACC. There is an ongoing French trial (Spencer Trial, PI Eric Baudin) with Nivolumab + adjuvant vaccine based on microbiota-derived peptides in patients with advanced ACC (only for adult patients and patients are first selected on HLA-I haplotype).

MD Anderson Cancer Center, Muhammad Habra utilizes immunotherapy (Pembrolizumab) for his patients with advanced ACC.
In the USA, immunotherapy is NOT FDA-approved for ACC, but they have the possibility to prescribe it. We as a consortium or Raul could ask Habra?

Finally, we could ask Bristol-Meyer for a, single-patient, compassionate use. They are usually quite restrictive, but never say never!

The Xenograft model (mouse with pediatric ACC)

  • T Lymphocytes-CD8+
  • T Lymphocytes-CD8+ and hybrid cells
  • Only hybrid cells

Pequeno Principe Therapeutic Vaccine:
The hybrid cell (Dendritic cell +ACC cell) incubated with T lymphocytes

B7-H3 CAR-T Cells for ACC:

Xenograft Mouse Models (concluded study) and ongoing Clinical Trial (Phase 1) not for international patients

Promising preliminary data for B7-H3 CAR T cell therapy in pediatric solid tumors, including the SJ-ACC xenograft models. Single dose also eliminated SJACC4 and MAST449A (not SJACC3, low expression of B7-H3).

3CAR: B7-H3-specific CAR T-cell Therapy for Children and Young Adults with Solid Tumors
Chris DeRenzo and Rebecca Epperly (St. Jude BMT & Cell Therapy)

3CAR is a Phase 1 clinical trial evaluating the safety and efficacy of autologous B7-H3-specific CAR T-cell therapy for children and young adults with relapsed or refractory solid tumors.

Primary objective

To determine the safety and maximum tolerated dose of one intravenous infusion of autologous, B7-H3-CAR T cells in patients 21 years old and younger with relapsed or refractory B7-H3-positive solid umors after lymphodepleting chemotherapy

Secondary objective

To evaluate the antitumor activity of B7-H3-CAR T cells

Inclusion criteria:

21 yrs or younger; Relapsed or refractory B7-H3+ solid tumor; Measurable disease and Adequate heart, lung, liver, kidney and bone marrow function


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