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成人髓母细胞瘤:病例报告。

Adult medulloblastoma: a case report.

机构信息

Department of Clinical Oncology, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.

Arusha Lutheran Medical Centre, Arusha, Tanzania.

出版信息

J Med Case Rep. 2022 Aug 25;16(1):330. doi: 10.1186/s13256-022-03531-3.

DOI:10.1186/s13256-022-03531-3
PMID:36002876
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9404560/
Abstract

BACKGROUND

Medulloblastoma is a malignant brain tumor that is common in children but very uncommon in adults, especially those older than 40 years, accounting for less than 1% of all primary brain tumors in adults. Although surgery and radiotherapy play an important role treatment of adult medulloblastoma, the use of chemotherapy is controversial. This is the first instance of adult medulloblastoma at the Ocean Road Cancer Institute in Tanzania.

CASE DESCRIPTION

We report the case of a 51-year-old female of African ethnicity who was diagnosed with high-risk hemispheric posterior cranial fossa medulloblastoma of classic type with World Health Organization central nervous system grade 4 and Chang stage M0. Immunohistochemistry, reticulin stain, and molecular subtyping could not be done because they were not available. She was treated by subtotal posterior cranial fossa tumor resection followed by adjuvant concurrent chemo-craniospinal radiation and adjuvant chemotherapy.

CONCLUSION

Even in adults over 50 years old, medulloblastoma should be included in the differential diagnosis of posterior fossa tumor. Adult medulloblastoma is a very rare and very heterogeneous tumor, but it has a good prognosis. Immunohistochemistry and molecular subclustering are difficult to implement in low-income countries such as Tanzania owing to cost. Treatment of adult medulloblastoma is highly heterogeneous among (and even within) facilities. There is no evidence that the extent of resection enhances survival. While craniospinal radiation therapy improves survival, there is controversy about the role of chemotherapy in managing adult MB.

摘要

背景

髓母细胞瘤是一种常见于儿童但非常罕见于成人的恶性脑肿瘤,尤其是年龄在 40 岁以上的成人,在所有成人原发性脑肿瘤中不到 1%。尽管手术和放疗在成人髓母细胞瘤的治疗中发挥着重要作用,但化疗的应用存在争议。这是坦桑尼亚海洋路癌症研究所首例成人髓母细胞瘤病例。

病例描述

我们报告了一例 51 岁的非洲裔女性病例,被诊断为经典型具有 4 级世界卫生组织中枢神经系统分级和 M0 Chang 分期的高风险半球后颅窝髓母细胞瘤。由于无法进行免疫组织化学、网状纤维染色和分子亚型分类,因此无法进行这些检查。她接受了部分后颅窝肿瘤切除术,随后进行辅助同步放化疗和辅助化疗。

结论

即使是 50 岁以上的成年人,也应将髓母细胞瘤纳入后颅窝肿瘤的鉴别诊断。成人髓母细胞瘤是一种非常罕见且非常异质性的肿瘤,但预后良好。由于成本原因,免疫组织化学和分子亚群分类在坦桑尼亚等低收入国家难以实施。成人髓母细胞瘤的治疗在(甚至在)不同机构之间存在高度异质性。目前尚无证据表明切除范围可以提高生存率。虽然全脑脊髓放疗可以提高生存率,但化疗在治疗成人 MB 中的作用存在争议。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fb0/9404560/efc00a753271/13256_2022_3531_Fig7_HTML.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fb0/9404560/efc00a753271/13256_2022_3531_Fig7_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fb0/9404560/6265de0390fd/13256_2022_3531_Fig1_HTML.jpg
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