关键词: Li-Fraumeni syndrome alkylating agent case report chemotherapy genetic predisposition medulloblastoma (MB) therapy-related myelodysplastic syndrome/acute myeloid leukemia

来  源:   DOI:10.3389/fonc.2024.1364199   PDF(Pubmed)

Abstract:
UNASSIGNED: Medulloblastoma is the most common malignant brain tumor in children, often requiring intensive multimodal therapy, including chemotherapy with alkylating agents. However, therapy-related complications, such as therapy-related myeloid neoplasms (t-MNs), can arise, particularly in patients with genetic predisposition syndromes. This case report presents three pediatric cases of medulloblastoma with subsequent development of t-MNs, highlighting the potential role of genetic predisposition and the importance of surveillance for hematological abnormalities in long-term survivors.
UNASSIGNED: We describe three cases of pediatric medulloblastoma who developed t-MNs after receiving chemotherapy, including alkylating agents. Two of the patients had underlying genetic predisposition syndromes (TP53 pathologic variants). The latency period between initial diagnosis of medulloblastoma and the development of secondary cancer varied among the cases, ranging from 17 to 65 months. The three cases eventually succumbed from secondary malignancy, therapy-related complications and progression of primary disease, respectively.
UNASSIGNED: This report highlights the potential association between genetic predisposition syndromes and the development of therapy-related myeloid neoplasms in pediatric medulloblastoma survivors. It underscores the importance of surveillance for hematological abnormalities among such patients.
摘要:
髓母细胞瘤是儿童最常见的恶性脑肿瘤,通常需要强化多模式治疗,包括用烷化剂进行化疗。然而,治疗相关并发症,如治疗相关的髓系肿瘤(t-MN),可以出现,特别是在患有遗传易感性综合征的患者中。该病例报告介绍了三例小儿髓母细胞瘤,随后发展为t-MNs,强调遗传易感性的潜在作用以及监测长期幸存者血液学异常的重要性。
我们描述了三例接受化疗后发展为t-MN的小儿髓母细胞瘤,包括烷化剂。其中两名患者具有潜在的遗传易感性综合征(TP53病理变异)。髓母细胞瘤的初始诊断和继发性癌症的发展之间的潜伏期在病例中有所不同。从17到65个月不等。三例最终死于继发性恶性肿瘤,治疗相关并发症和原发疾病进展,分别。
本报告强调了遗传易感性综合征与儿童髓母细胞瘤幸存者治疗相关髓系肿瘤发展之间的潜在关联。它强调了监测此类患者中血液学异常的重要性。
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