Al-Jabri Talal, Garg Sunil, Mani Ganapathyraman V
Department of Plastic Surgery, East and North Hertfordshire NHS Trust, AL7 4HQ, UK.
J Orthop Surg Res. 2010 Sep 28;5:71. doi: 10.1186/1749-799X-5-71.
Lipofibromatous hamartoma is a rare tumour of peripheral nerves which is characterised by an excessive infiltration of the epineurium and perineurium by fibroadipose tissue. To the best of our knowledge, only approximately 88 cases are reported in the literature. We report a rare case of lipofibromatous hamartoma of the median nerve causing secondary carpal tunnel syndrome in a 25 year old patient. This patient was treated conservatively with decompression and biopsy and experienced a complete resolution of symptoms post-operatively. Magnetic resonance imaging may be used to diagnose this lesion as it has very distinctive characteristics. Multiple conditions have been associated with this lesion and a greater understanding of these associations may clarify the pathogenesis. The architecture of the tumour makes excision very challenging and the surgical management remains controversial. A review of the literature regarding the etiology, pathogenesis and surgical management of lipofibromatous hamartoma is included.
脂肪纤维瘤性错构瘤是一种罕见的周围神经肿瘤，其特征是纤维脂肪组织过度浸润神经外膜和神经束膜。据我们所知，文献中仅报道了约88例。我们报告了一例罕见的正中神经脂肪纤维瘤性错构瘤，导致一名25岁患者继发腕管综合征。该患者接受了减压和活检的保守治疗，术后症状完全缓解。磁共振成像因其具有非常独特的特征，可用于诊断该病变。多种情况与该病变相关，对这些关联的更深入了解可能会阐明其发病机制。肿瘤的结构使得切除极具挑战性，手术管理仍存在争议。本文还包括了关于脂肪纤维瘤性错构瘤的病因、发病机制和手术管理的文献综述。
