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胸膜恶性外周神经鞘瘤 1 例并文献复习

A rare case of malignant peripheral nerve sheath tumor of pleura and review of literature.

机构信息

Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India.

Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India.

出版信息

Indian J Pathol Microbiol. 2021 Jan-Mar;64(1):158-160. doi: 10.4103/IJPM.IJPM_948_19.

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma arising from peripheral nerves. They can be found in isolation, but about half of the cases are associated with neurofibromatosis type 1 (NF1), an autosomal dominant genetic disorder. They usually arise from the nerve plexus in extremities and trunk. MPNSTs arising from the viscera or internal organs are very rare. We hereby report a rare case of sporadic MPNST arising from pleural surface in a middle-aged male. The diagnosis of such a rare entity involved multimodal investigations with consideration of wide differential diagnosis.

摘要

恶性外周神经鞘瘤(MPNST)是一种起源于周围神经的软组织肉瘤。它们可以单独出现,但约一半的病例与神经纤维瘤病 1 型(NF1)有关,这是一种常染色体显性遗传疾病。它们通常起源于四肢和躯干的神经丛。起源于内脏或内部器官的 MPNST 非常罕见。我们在此报告一例罕见的中年男性胸膜表面散发性 MPNST。这种罕见实体的诊断涉及多模态检查,并考虑了广泛的鉴别诊断。

相似文献

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Malignant Peripheral Nerve Sheath Tumor.恶性外周神经鞘膜瘤
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