Dataset Information

Glioma dataset from Rabat: Clinicopathological, immunohistochemical and disease progression features of 32 Moroccan patients with diffuse Glioma.

ABSTRACT: The Moroccan Glioma Dataset contains the clinical data of 32 patients with glioma. The clinical data including demographic data (age, sex), tumor characteristics (tumor location, Glioma type, Karnofsky performance score, mitotic activity, cell density, necrosis, endotheliocapillary vascular proliferation, MRI contrast pick-up, corpus collosum infiltration and Oedema), treatment strategy (subtotal resection, gross resection, biopsy, radiotherapy, chemotherapy), expression pattern of tumor biomarkers (IDH1, HIF-1alpha, P53, Ki-67), and survival data (Kaplan-Meier curves for disease progression). The dataset can be used to relate tumor characteristics to tumor biomarkers and to predict disease progression for a better treatment management. The data were presented, analyzed, and described in the article "Immunohistochemical expression of HIF-1α, IDH1 and TP53: prognostic profile of Moroccan patients with diffuse glioma" published in Journal of Chemical Neuroanatomy [1].

SUBMITTER: Sfifou F

PROVIDER: S-EPMC9126762 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature

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Glioma dataset from Rabat: Clinicopathological, immunohistochemical and disease progression features of 32 Moroccan patients with diffuse Glioma.

Sfifou Fatima F Ouzir Mounir M Hakkou El Mehdi EM Obtel Majdouline M Errihani Hassan H Bouzidi Abderrahmane Al AA Abouqal Redouane R Ouahabi Abdessamad El AE Cherradi Nadia N

Data in brief 20220512

The Moroccan Glioma Dataset contains the clinical data of 32 patients with glioma. The clinical data including demographic data (age, sex), tumor characteristics (tumor location, Glioma type, Karnofsky performance score, mitotic activity, cell density, necrosis, endotheliocapillary vascular proliferation, MRI contrast pick-up, corpus collosum infiltration and Oedema), treatment strategy (subtotal resection, gross resection, biopsy, radiotherapy, chemotherapy), expression pattern of tumor biomark ...[more]

PMID: 35620243

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Project description:BackgroundWilms tumour (WT), the second most reported childhood cancer in Morocco, is a malignant kidney tumour that affects children under 15 years old. Prognosis has improved with the adoption of multimodal treatment. However, data on WT in Morocco remain limited.AimsThis study aims to comprehensively describe and analyse the epidemiological, clinicopathological features and treatment outcomes of WT in Moroccan patients, including treatment response and recurrence rates.Methods and resultsA retrospective study involved 84 children under 15 years with WT, treated according to the SIOP protocol and followed at the Paediatric Haematology and Oncology Centre at Children's hospital of Rabat, between January 2014 and February 2018. The median age of participants was 36 months, with a male/female sex ratio of 0.79. Abdominal mass was the primary concern in 55 cases (66%). Five patients (6%) had bilateral WT. Metastatic WT occurred in 21 cases (25%). Stage III was predominant in 33 cases (43%). Twenty cases (26%) had high-risk WT, and IVC tumour thrombus was observed in 12 cases (14%). WT histotype correlated significantly with both sex and tumour localisation (p values of 0.040 and 0.013, respectively). Age correlated significantly with WT extension, overall stage and SIOP histology risk grades (p values of 0.003, 0.003 and 0.045, respectively). Overall stage was statistically related to the occurrence of IVC tumour thrombus (p = 0.002). Over a 5-year span post-nephrectomy, complete remission was achieved in 63 patients (75%), partial remission in one patient (1%), while 19 patients (23%) died and one patient (1%) relapsed.ConclusionThese findings are encouraging for a developing country. However, the elevated rates of Stages III and IVC thrombus in this series are still high, primarily attributed to delays in diagnosis and treatment and the limited number of paediatric haematology and oncology units at the time of the study. Nevertheless, further multicentric research is warranted to enrich Moroccan data and establish a national register for WT cases.

Project description:Abstract Diffuse midline glioma (DMG) is a uniformly fatal pediatric cancer that is in need of urgent “outside the box” therapeutic approaches. Recent studies show that tumor cells adapt to stresses created by oncogenic mutations and these oncogene-induced adaptations create vulnerabilities that can be exploited to therapeutic ends. To uncover these oncogene-induced vulnerabilities in DMGs we conducted a genome-wide CRIPSR knockout screen in three DMG lines. The top common DMG dependency pathway that we discovered is de novo pyrimidine biosynthesis. Under normal conditions pyrimidine nucleotide needs are met through the salvage pathway. However, in DMG tumorigenesis, pyrimidine nucleotide synthesis is rewired such that the cells become dependent on the de novo biosynthesis pathway. De novo pyrimidine synthesis is catalyzed by CAD, DHODH and UMPS; all three genes are identified as dependencies in our screen and have been validated using shRNA mediated gene knockdown. Interestingly, DMG cells did not exhibit a dependency on the de novo purine biosynthesis pathway. Using a small molecule inhibitor of DHODH, BAY2402234 [currently studied in phase I trial for myeloid malignancies (NCT03404726)], we have demonstrated and validated, (i) efficacy and specificity of de novo pyrimidine synthesis inhibition in vitro in DMG cells; (ii) de novo pyrimidine addiction is not attributable to cell proliferation; (iii) DHODH inhibition induces apoptosis by hindering replication and inciting DNA damage; (iv) DHODH and ATR inhibition act synergistically to induce DMG cell death; and (v) critical in vivo efficacy. The in vivo experiment documents that BAY2402234 crosses the blood-brain barrier, is present in the brain at therapeutically relevant concentrations, suppresses de novo pyrimidine biosynthesis in intracranial DMG tumors in mice, and prolongs survival of orthotopic DMG tumor bearing mice. Taken together, our studies have identified a novel metabolic vulnerability that can be translated for the treatment of DMG patients.

Dataset Information

Glioma dataset from Rabat: Clinicopathological, immunohistochemical and disease progression features of 32 Moroccan patients with diffuse Glioma.

Publications

Glioma dataset from Rabat: Clinicopathological, immunohistochemical and disease progression features of 32 Moroccan patients with diffuse Glioma.

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