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内脏丛状神经鞘瘤:病例系列

Visceral plexiform schwannoma: A case series.

作者信息

Destro Francesca, Sharma Shilpa, Maestri Luciano, Vella Claudio, Collini Paola, Riccipetitoni Giovanna

机构信息

Department of Paediatric Surgery, Buzzi Children's Hospital, I-20154 Milan, Italy.

Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi 110029, India.

出版信息

Mol Clin Oncol. 2021 Jan;14(1):14. doi: 10.3892/mco.2020.2176. Epub 2020 Nov 26.

Abstract

Plexiform schwannoma (PS) is a benign tumour of the peripheral nerve sheath that is typically found in the skin. Fewer than 15 cases of visceral PS have been reported to date in both adults and children. We herein discuss a series of 3 patients (2 male and 1 female) with abdominal PS, aged 10-16 years (mean age, 12 years). All the patients had an acute presentation with abdominal pain, which was associated with rectal bleeding in 1 case and with walking difficulties in 1 case. Radiological investigations included abdominal magnetic resonance imaging (MRI) and computed tomography, along with neurofibromatosis screening (cerebral MRI and dermatological evaluation). Complete removal of the mass was possible in 2 of the patients (in 1 case by laparoscopically assisted surgery). Follow-up was uneventful. Abdominal PS is a rare occurrence. Due to its possible association with neurofibromatosis, the diagnosis of PS should prompt an investigation for other manifestations of this disorder.

摘要

丛状神经鞘瘤(PS)是一种外周神经鞘的良性肿瘤,通常见于皮肤。迄今为止,成人和儿童中报道的内脏PS病例少于15例。我们在此讨论3例腹部PS患者(2例男性,1例女性),年龄在10至16岁之间(平均年龄12岁)。所有患者均急性起病,表现为腹痛,其中1例伴有直肠出血,1例伴有行走困难。影像学检查包括腹部磁共振成像(MRI)和计算机断层扫描,以及神经纤维瘤病筛查(脑部MRI和皮肤科评估)。2例患者的肿块得以完全切除(1例通过腹腔镜辅助手术)。随访过程顺利。腹部PS较为罕见。由于其可能与神经纤维瘤病相关,PS的诊断应促使对该疾病的其他表现进行检查。

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