16岁患者成神经管细胞瘤一例临床病例

S. Kopochkina, A. Goncharova, Z. Dushimova, A. Bykovskaya, K. Tokhmoldayeva, M. Zharaspayeva
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引用次数: 0

摘要

相关性:脑肿瘤是儿童最常见的实体肿瘤,髓母细胞瘤约占20%。哈萨克斯坦共和国肿瘤患者电子登记处(RK)报告了2021年27例经形态学证实的成神经管细胞瘤病例,其中18例为儿童(66.6%)。目的是用一个临床病例为例,说明进行分子遗传学研究以明确脑肿瘤诊断的可行性。方法:本文报告1例髓母细胞瘤的临床病例。检查包括大脑的计算机断层扫描,大脑的磁共振成像,以及术后材料的免疫组织化学病理形态学检查。结果:患者于哈萨克斯坦阿拉木图“哈萨克肿瘤与放射研究所”儿童肿瘤中心转诊,形态学诊断为“小脑蚓室管膜瘤伴第四脑室受压”。II级。左侧脑室腹腔造口术后(2021年11月14日),显微手术切除肿瘤后(2021年12月7日)。第二临床组。”由于形成的非典型定位,对术后材料进行了病理形态学检查和免疫组织化学研究。结论:形态学和免疫表型符合纤维组织增生/结节性髓母细胞瘤,IV级,ICD-O编码9473 /3。根据形态学资料修改治疗策略。结论:髓母细胞瘤是一种具有侵袭性的高度恶性肿瘤。选择正确的治疗策略取决于及时、准确的诊断,这使得诊断成为患者管理算法中的关键环节。这需要进行形态学和免疫组织化学检查。根据2021年WHO第5版中枢神经系统肿瘤分类,需要进行分子遗传学研究,以准确确定肿瘤的形态结构,并获得更好的治疗反应。这就需要修订哈萨克斯坦共和国通过的相关临床指南。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
MEDULLOBLASTOMA IN A 16-YEAR-OLD PATIENT: A CLINICAL CASE
Relevance: Brain tumors are the most common solid tumors in children, with medulloblastomas accounting for almost 20%. The Electronic Registry of Oncological Patients of the Republic of Kazakhstan (RK) reports 27 morphologically verified cases of medulloblastoma in 2021, 18 of them in children (66.6%). The aim was to show the feasibility of conducting a molecular genetic study to clarify the diagnosis of brain tumors using a clinical case as an example. Methods: The article describes a clinical case of a patient with medulloblastoma. The examinations made included computed tomography of the brain, magnetic resonance imaging of the brain, as well as pathomorphological revision with an immunohistochemical examination of postoperative material. Results: The patient was admitted to the Center of Pediatric Oncology of “Kazakh Institute of Oncology and Radiology” JSC (Almaty, Kazakhstan) with a referral morphological diagnosis of “Ependymoma of the cerebellar vermis with compression of the 4th ventricle. Grade II. Condition – after ventriculoperitoneostomy on the left side (14.11.2021), after microsurgical removal of the tumor (07.12.2021). 2nd clinical group.” Because of the atypical localization of the formation, a pathomorphological review with an immunohistochemical study of the postoperative material was carried out. Conclusion: the morphological picture and immunophenotype correspond to desmoplastic/nodular medulloblastoma, grade IV. ICD-O code 9471/3. The treatment tactics were revised based on morphological data. Conclusion: Medulloblastoma is a highly malignant tumor with an aggressive course. Choosing the right treatment tactics depends on a timely, accurate diagnosis, which makes the diagnosis a key link in the patient management algorithm. This requires conducting a morphological, immunohistochemical examination. According to the 5th version of the 2021 WHO classification of CNS tumors, a molecular genetic study is required to accurately determine the morphological structure of the tumor and achieve a better response to therapy. This necessitates a revision of the relevant clinical guidelines adopted in the Republic of Kazakhstan.
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