Department of Neurology, University Hospital Hamburg-Eppendorf, Hamburg, Germany
Department of Oral and Maxillofacial Surgery, University Hospital Hamburg-Eppendorf, Hamburg, Germany.
In Vivo. 2020 May-Jun;34(3):1031-1036. doi: 10.21873/invivo.11872.
BACKGROUND/AIM: Plexiform neurofibromas (PNFs) are benign tumors of the periph eral nerves sheath, which can damage neighboring organs, impair functions, cause pain and serious maxillofacial disfigurement, and have a high risk of malignant transformation. Complete resection is usually not possible since PNFs often extend through multiple layers of tissue. Therefore, it is necessary and beneficial to find a reasonable drug treatment for PNFs. Propranolol-treatment is the first-line therapy for infantile hemangiomas and the side effects are reversible and mostly benign. The present study aimed to examine the possible effect of propranolol for suppressing PNFs in vitro.
Paired primary Schwann-cell-rich cultures and fibroblast-rich cultures were obtained from 4 PNFs of unrelated patients. Human Schwann cells (HSCs) were used as the control. These cultures were treated with propranolol for 7 days at concentrations up to 150 μM. Cells were then measured for their viability and immune-stained with S100 to label the tumorous Schwann cells.
Propranolol inhibited the viability of the tumorous Schwann cells in a dose-dependent manner, while did not substantially suppress viability of the non-tumorous fibroblasts derived from the same PNFs.
Propranolol may provide a treatment option for suppressing the growth of PNFs.
背景/目的：丛状神经纤维瘤（PNFs）是外周神经鞘的良性肿瘤，可损伤邻近器官、影响功能、引起疼痛和严重的颌面畸形，并有恶性转化的高风险。由于 PNF 通常通过多层组织延伸，因此通常不可能进行完全切除。因此，寻找一种合理的 PNF 药物治疗方法是必要且有益的。普萘洛尔治疗是婴儿血管瘤的一线治疗方法，其副作用是可逆的，且大多是良性的。本研究旨在探讨普萘洛尔抑制体外 PNF 的可能作用。
从 4 名无关患者的 4 个 PNF 中获得配对的富含施万细胞的原代培养物和富含成纤维细胞的培养物。用人 Schwann 细胞（HSCs）作为对照。这些培养物在 7 天内用高达 150 μM 的普萘洛尔处理。然后测量细胞活力，并使用 S100 免疫染色标记肿瘤性施万细胞。
普萘洛尔以剂量依赖性方式抑制肿瘤性施万细胞的活力，而对源自同一 PNF 的非肿瘤性成纤维细胞的活力没有显著抑制。
普萘洛尔可能为抑制 PNF 生长提供一种治疗选择。
