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髓母细胞瘤骨髓转移。4例报告并文献复习。

Medulloblastoma metastatic to the marrow. Report of four cases and review of the literature.

作者信息

Spencer C D, Weiss R B, Van Eys J, Cohen P, Edwards B

出版信息

J Neurooncol. 1984;2(3):223-35. doi: 10.1007/BF00253274.

Abstract

Medulloblastoma is a malignant cerebellar tumor seen primarily in the pediatric age group that has a known ability to metastasize extraneurally. The skeleton is the most common site of extraneural metastases, but metastases to the bone marrow can also occur. Four cases of medulloblastoma metastatic to the marrow are reported. In addition, 31 cases from the medical literature are reviewed. Clinical features include bone tenderness, cytopenias and elevated serum alkaline phosphatase and lactic dehydrogenase levels. Skeletal involvement, especially of the pelvic bones, is frequently seen radiographically. Weight loss, soft tissue masses and a requirement for blood transfusion are also associated features. Marrow biopsy specimens are characterized by the presence of a small cell tumor often with fibrosis, necrosis and osteoblastic activity. The symptomatic response to chemotherapy is rapid, but chemotherapy resistance appears quickly. Only 1 in 4 cases diagnosed antemortem in this review lived for more than a year. We conclude that marrow aspiration and biopsy are indicated in the evaluation of patients with medulloblastoma and may serve to diagnose the cause of cytopenias, to verify extraneural spread and to provide prognostic information.

摘要

髓母细胞瘤是一种主要见于儿童年龄组的恶性小脑肿瘤,已知具有神经外转移能力。骨骼是神经外转移最常见的部位,但也可发生骨髓转移。本文报告了4例髓母细胞瘤骨髓转移病例。此外,还复习了医学文献中的31例病例。临床特征包括骨压痛、血细胞减少以及血清碱性磷酸酶和乳酸脱氢酶水平升高。影像学检查常可见骨骼受累,尤其是骨盆骨。体重减轻、软组织肿块和输血需求也是相关特征。骨髓活检标本的特征是存在一个小细胞肿瘤,常伴有纤维化、坏死和成骨活性。化疗的症状缓解迅速,但很快就会出现化疗耐药。在本次综述中,生前诊断的病例中只有四分之一存活超过一年。我们得出结论,在髓母细胞瘤患者的评估中,骨髓穿刺和活检是必要的,可能有助于诊断血细胞减少的原因、证实神经外扩散并提供预后信息。

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