Background: In 2018, the World Health Organization (WHO) launched the Global Initiative for Childhood Cancer (GICC). The goal is to achieve a global survival rate of at least 60% for all children with cancer by 2030. Morocco was designated as a pilot country for this initiative. Procedure: This retrospective study included a cohort of children aged 0-15 years, with one of the six indexed cancers (acute lymphoblastic leukemia [ALL], Burkitt lymphoma [BL], Hodgkin lymphoma, retinoblastoma [RB], Wilms tumor or nephroblastoma, low-grade glioma), diagnosed between January 1, 2017 and December 31, 2019 at the six Moroccan Pediatric Hematology and Oncology units. Patients were followedup until August 31, 2020. The Kaplan-Meier method was used to estimate survival rates, the log-rank test for comparing survival curves, and the Cox model for identifying prognostic factors.
Germline biallelic mutations in one of the mismatch repair genes, mutS homolog 2, mutS homolog 6, mutL homolog 1, or postmeiotic segregation increased 2, result in one of the most aggressive cancer syndromes in humans termed as constitutional mismatch repair deficiency (CMMRD). Individuals with CMMRD are affected with multiple tumors arising from multiple organs during childhood, and these individuals rarely reach adulthood without specific interventions. The most common tumors observed are central nervous system, hematological, and gastrointestinal malignancies. The incidence of CMMRD is expected to be high in low‐resource settings due to a high rate of consanguinity in these regions, and it is thought to be underrecognized and consequently underdiagnosed. This position paper is therefore important to provide a summary of the current situation, and to highlight the necessity of increasing awareness, diagnostic criteria, and surveillance to improve survival for patients and family members.
Background
In low‐ and middle‐income countries, therapeutic options for advanced, refractory, or relapsing malignancies are limited due to local constraints such as cost of drugs, distance from oncology centers, and lack of availability of new anticancer drugs. Metronomics, which combines metronomic chemotherapy (MC) and drug repositioning, allows for the provision of new therapeutic options for patients in this setting.
Aim of the study
To evaluate the activity and toxicity of a metronomic regimen in Moroccan pediatric patients with refractory or relapsing malignancies.
Patients and methods
From July 2014 to January 2018, patients with refractory/relapsing solid tumors treated in five pediatric oncology centers were consecutively enrolled. The metronomic regimen consisted of 28‐day cycles with daily oral administration of cyclophosphamide (30 mg/m2) from days 1 to 21, together with oral etoposide (25 mg/m2) from days 1 to 21 followed by break of one week and daily valproic acid (20 mg/kg) from days 1 to 28.
Results
Ninety‐eight children (median age, 8 years) were included. Underlying malignancies were neuroblastoma (24 patients), Ewing sarcoma (18), osteosarcoma (14), rhabdomyosarcoma (14), and miscellaneous tumors (28). A total of 557 cycles were given (median: 6; range, 1‐18 cycles). One‐year progression‐free survival of our patients was 19%, and one‐year overall survival was 22%. Complete response was obtained in three cases (3%), partial response in 11 cases (11%), and tumor stabilization for more than six months in 28 cases (28%).
Conclusion
This three‐drug metronomic combination was well tolerated and associated with tumor response and disease stabilization in 42 patients even for a long period.
Askin tumor is a rare malignant thoracopulmonary tumor that primarily affects children. In University Hospital Mohammed VI's Pediatric Oncology-Hematology department, two young patients with Askin's tumor were treated. Chest computed tomography imaging in both patients revealed an Askin's tumor, which was later confirmed histologically. Both patients got postoperative chemotherapy as well as a surgical excision of the tumor. One of them passed away as a result of a localized chest wall recurrence. In this article, we discuss our experiences with this serious pathology.
IntroductionLangerhans cell histiocytosis (LCH) is a rare nonmalignant disease characterized by proliferation and accumulation of clonal dendritic cells in various organs [1,2]. The clinical picture of LCH can vary widely. Often, it involves only a single organ such as skin or bone. Other patients can present with multisystem disease. Evolution course may vary from a limiting disease to a rapidly progressive one frequently fatal. [2][3][4]. Children with LCH in highincome countries are treated with standard protocols and have high survival rate (80-99%) [5][6][7][8][9][10][11][12]. Although the prognosis is good, late sequels are often described [12,13].
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