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首页> 外文期刊>Journal of Clinical and Diagnostic Research >Primary MPNST in Childhood- A Rare Case Report
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Primary MPNST in Childhood- A Rare Case Report

机译：儿童期主要MPNST-罕见病例报告

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Malignant peripheral nerve sheath tumour usually occurs between 20-50 years of age, comprising about 5-10% of soft tissue sarcomas. Only 1.7% of them have been reported to occur in children < 5 months of age according to the literature. Here, we are describing 18 mnth old male child presented with a swelling in the lower back. MRI showed a sacrcoccygeal swelling extending to and communicating with CSF at lower sacral level. Birth history of the child was normal with normal apgar score. The histological diagnosis was malignant peripheral nerve sheath tumour. IHC showed focal positivity of GFAP and S100. Primary spinal MPNST in children are rarer. A careful neurological examination is warranted in children. Early diagnosis and referral to multidisciplinary team are important in ensuring the best diagnosis and optimal therapy in this young age.
机译：恶性周围神经鞘瘤通常发生在20-50岁之间，约占软组织肉瘤的5-10％。据文献报道，其中只有1.7％发生在5个月以下的儿童中。在这里，我们描述的是18个月大的男童，其下背部有肿胀。 MRI显示sa骨sw肿扩展至较低SF骨水平并与CSF交流。该儿童的出生史正常，apgar评分正常。组织学诊断为恶性周围神经鞘瘤。 IHC显示出GFAP和S100的病灶阳性。儿童原发性脊柱MPNST少见。儿童应进行仔细的神经系统检查。尽早诊断并转诊至多学科团队对于确保这一年轻时代的最佳诊断和最佳治疗至关重要。

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