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手臂恶性周围神经鞘膜瘤:一种罕见的临床表现。

Arm malignant peripheral nerve sheath tumour: a rare clinical presentation.

作者信息

Nascimento Ricardo, Valença-Filipe Rita, Horta Ricardo, Silva Álvaro

机构信息

Plastic, Reconstructive and Aesthetic Surgery and Burn Unit, Centro Hospitalar de São João, Porto, Portugal.

出版信息

BMJ Case Rep. 2019 Aug 15;12(8):e229852. doi: 10.1136/bcr-2019-229852.

Abstract

Soft tissue sarcomas account for about 1% of malignant tumours. More than 50 subtypes of these tumours have been described with some being extremely rare, namely malignant peripheral nerve sheath tumours (MPNST). The authors present a case of a man aged 81 years with a medical history of an adenocarcinoma of the rectum, which was referred to our clinic due to a growing painless mass on the right arm. An MRI showed a well-delimited encapsulated mass involving the long head of the biceps muscle. Biopsy findings revealed a spindle cell tumour with cytonuclear pleomorphism. The patient underwent wide tumour excision and was successfully reconstructed with a latissimus dorsi functional muscle transfer. The presence of two pulmonary nodules on CT scan staging implied a lung biopsy that showed rectum primary tumour metastases. With these additional findings, the pathology department reassessed the case and reclassified the arm tumour as an MPNST, synchronous with pulmonary adenocarcinoma metastases of the rectum.

摘要

软组织肉瘤约占恶性肿瘤的1%。这些肿瘤有50多种亚型,其中一些极为罕见,如恶性外周神经鞘瘤(MPNST)。作者报告了一例81岁男性病例,其有直肠癌病史,因右臂出现无痛性肿块增大而转诊至我院。磁共振成像(MRI)显示一个界限清楚的包膜肿块,累及肱二头肌长头。活检结果显示为具有细胞核异形性的梭形细胞瘤。患者接受了广泛的肿瘤切除,并通过背阔肌功能性肌肉转移成功重建。CT扫描分期显示有两个肺结节,意味着需进行肺活检,结果显示为直肠原发性肿瘤转移。基于这些额外发现,病理科重新评估了该病例,并将手臂肿瘤重新分类为MPNST,与直肠肺腺癌转移同步。

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Primary malignant peripheral nerve sheath tumor at unusual location.罕见部位的原发性恶性外周神经鞘瘤。
J Neurosci Rural Pract. 2013 Aug;4(Suppl 1):S83-6. doi: 10.4103/0976-3147.116480.

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