Shaterian Ashkaun, Ozyurekoglu Tuna
Department of Hand and Microsurgery, Kleinert Institute for Hand and Microsurgery, Louisville, Kentucky, United States.
J Hand Microsurg. 2020 Sep 22;14(2):160-162. doi: 10.1055/s-0040-1715424. eCollection 2022 Apr.
Schwannomas represent benign peripheral nerve sheath tumors. Their phenotypic presentations in schwannomatosis and segmental schwannomatosis have been well described. To date, however, cases of schwannomatosis or multiple schwannomas localized to a single nerve fascicle have been limited in the literature. In this study, we identify a case of a 48 year-old non-neurofibromatosis male who presented with symptomatic schwannomas localized to a single nerve fascicle of the upper extremity. Intraoperative exploration revealed four schwannomas arising from a 15-cm segment of ulnar nerve fascicle. Surgical excision was successful, without neurological deficit or recurrence. This study identifies a case of schwannomatosis localized to a single nerve fascicle that may represent a linear variant of segmental schwannomatosis. The presentation may represent a temporary linear appearance in progression to "nonlinear" segmental variant; however, a molecularly distinct subset of schwannomas cannot be excluded.
施万细胞瘤是良性周围神经鞘瘤。其在神经鞘瘤病和节段性神经鞘瘤病中的表型表现已有详细描述。然而，迄今为止，文献中局限于单个神经束的神经鞘瘤病或多发性施万细胞瘤病例有限。
在本研究中，我们确定了一例48岁非神经纤维瘤病男性患者，其上肢单个神经束出现有症状的施万细胞瘤。术中探查发现4个施万细胞瘤起源于尺神经束的15厘米节段。手术切除成功，无神经功能缺损或复发。
本研究确定了一例局限于单个神经束的神经鞘瘤病病例，这可能代表节段性神经鞘瘤病的一种线性变体。这种表现可能代表在进展为“非线性”节段性变体过程中的一种暂时线性外观；然而，不能排除分子上不同的施万细胞瘤亚群。
