PDF(Pubmed)
Abstract：
BACKGROUND: Lipomatosis of nerve (LN) is a rare disorder characterized by the massive enlargement of peripheral nerves, frequently accompanied by generalized fibroadipose proliferation and skeletal overgrowth.
METHODS: The authors have been routinely following a 20-year-old male for lipomatosis of median nerve at the wrist noted shortly after birth. He had undergone resection of the lesion accompanied by sural nerve grafting at another institution. Clinically, although his neurological loss of function has been stable, he has had continued soft tissue growth. Serial magnetic resonance imaging has revealed persistent LN proximal to the repair sites with evidence of fatty proliferation in the sural grafts and continued LN and fatty proliferation distally. There has been a progressive circumferential pattern of fibrosis around the proximal and distal suture lines, which has a similar radiological pattern to desmoid type fibromatosis (a pattern recently described in neuromuscular choristoma [NMC] desmoid-type fibromatosis).
CONCLUSIONS: Considering the similar reaction of nerve in both LN and NMC despite differing genetic cascades, the authors believe a unifying process occurs in both lesions. The pattern of circumferential fibroproliferation would be most consistent with neuron-mediated growth from unspecified trophic factors, supporting a previously reported a nerve-derived \"inside-out mechanism.\" The clinical consequences of this unifying process are presented.
METHODS: The authors have been routinely following a 20-year-old male for lipomatosis of median nerve at the wrist noted shortly after birth. He had undergone resection of the lesion accompanied by sural nerve grafting at another institution. Clinically, although his neurological loss of function has been stable, he has had continued soft tissue growth. Serial magnetic resonance imaging has revealed persistent LN proximal to the repair sites with evidence of fatty proliferation in the sural grafts and continued LN and fatty proliferation distally. There has been a progressive circumferential pattern of fibrosis around the proximal and distal suture lines, which has a similar radiological pattern to desmoid type fibromatosis (a pattern recently described in neuromuscular choristoma [NMC] desmoid-type fibromatosis).
CONCLUSIONS: Considering the similar reaction of nerve in both LN and NMC despite differing genetic cascades, the authors believe a unifying process occurs in both lesions. The pattern of circumferential fibroproliferation would be most consistent with neuron-mediated growth from unspecified trophic factors, supporting a previously reported a nerve-derived \"inside-out mechanism.\" The clinical consequences of this unifying process are presented.
摘要：
背景：神经脂肪瘤病（LN）是一种罕见的疾病，其特征是周围神经大量增大，常伴有全身纤维脂肪增生和骨骼过度生长。
方法：作者常规跟踪一名20岁男性出生后不久发现的腕部正中神经脂肪瘤病。他在另一个机构接受了病灶切除并伴有腓肠神经移植。临床上,虽然他的神经功能丧失一直稳定,他有持续的软组织生长。连续磁共振成像显示，修复部位近端持续存在LN，有证据表明腓肠移植物中脂肪增生，远端持续存在LN和脂肪增生。在近端和远端缝合线周围有一个渐进性的环形纤维化模式，其具有与纤维样型纤维瘤病（最近在神经肌肉性脉络膜瘤[NMC]纤维样型纤维瘤病中描述的模式）相似的放射学模式。
结论：考虑到尽管基因级联不同，LN和NMC的神经反应相似，作者认为两个病变都会发生一个统一的过程。环状纤维增殖的模式与来自未指定营养因子的神经元介导的生长最一致，支持先前报道的神经衍生的“由内而外的机制”。“这一统一过程的临床后果被提出。
方法：作者常规跟踪一名20岁男性出生后不久发现的腕部正中神经脂肪瘤病。他在另一个机构接受了病灶切除并伴有腓肠神经移植。临床上,虽然他的神经功能丧失一直稳定,他有持续的软组织生长。连续磁共振成像显示，修复部位近端持续存在LN，有证据表明腓肠移植物中脂肪增生，远端持续存在LN和脂肪增生。在近端和远端缝合线周围有一个渐进性的环形纤维化模式，其具有与纤维样型纤维瘤病（最近在神经肌肉性脉络膜瘤[NMC]纤维样型纤维瘤病中描述的模式）相似的放射学模式。
结论：考虑到尽管基因级联不同，LN和NMC的神经反应相似，作者认为两个病变都会发生一个统一的过程。环状纤维增殖的模式与来自未指定营养因子的神经元介导的生长最一致，支持先前报道的神经衍生的“由内而外的机制”。“这一统一过程的临床后果被提出。
