Châtillon Claude-Edouard, Guiot Marie-Christine, Corriveau Marc-Michel, Jacques Line
Department of Neurosurgery, McGill University Health Center, Montreal Neurological Hospital, Montreal, Canada.
Neurosurgery. 2006 May;58(5):E997; discussion E997. doi: 10.1227/01.NEU.0000210293.67328.A7.
Neurofibromas are benign neural sheath tumors arising from intraneural supporting cells. Such tumors are characteristic of neurofibromatosis Type I (von Recklinghausen disease) but also occur sporadically. Vascular involvement by neurofibromata is rare, but has been described in the past in the context of neurofibromatosis. There is, to our knowledge, no description of vascular involvement by a neurofibroma in a non-neurofibromatosis patient.
A 40-year-old woman presented with a 4 year history of a right thigh mass associated with diffuse lower extremity pain. She had no other clinical manifestations of neurofibromatosis and no known family member with neurofibromatosis Type I. Magnetic resonance imaging scans revealed a well-defined solid mass in the anteromedial aspect of the right thigh closely associated with the superficial femoral vein.
The vessel segment and encapsulated mass were resected "en bloc" after proximal and distal ligation of the vein. The pathological appearance of the mass was consistent with a benign neurofibroma that had infiltrated all layers of the vessel.
Vessel invasion by a benign sporadic neurofibroma is a rare occurrence with potentially severe implications for the patient. It suggests that surgical removal of asymptomatic benign-appearing lesions of that type should be considered if they are adjacent to important anatomical structures.
神经纤维瘤是起源于神经内支持细胞的良性神经鞘瘤。此类肿瘤是I型神经纤维瘤病（冯雷克林霍增氏病）的特征性表现，但也可散发性出现。神经纤维瘤累及血管的情况罕见，但过去曾在神经纤维瘤病的背景下被描述过。据我们所知，尚无非神经纤维瘤病患者出现神经纤维瘤累及血管的相关描述。
一名40岁女性，有右大腿肿块伴下肢弥漫性疼痛4年的病史。她没有神经纤维瘤病的其他临床表现，且家族中没有已知的I型神经纤维瘤病患者。磁共振成像扫描显示右大腿前内侧有一个边界清晰的实性肿块，与股浅静脉密切相关。
在静脉近端和远端结扎后，将血管段和包膜完整的肿块“整块”切除。肿块的病理表现符合良性神经纤维瘤，且已浸润血管各层。
良性散发性神经纤维瘤侵犯血管是一种罕见情况，对患者可能有严重影响。这表明，如果无症状的此类良性外观病变毗邻重要解剖结构，应考虑手术切除。
