Nanno Mitsuhiko, Sawaizumi Takuya, Takai Shinro
Department of Restorative Medicine of Neuro-musculoskeletal System, Orthopaedic Surgery, Nippon Medical School, Japan.
J Nippon Med Sch. 2011;78(6):388-92. doi: 10.1272/jnms.78.388.
Fibrolipomatous hamartoma of nerves without macrodactyly is a rare lesion characterized by fibrofatty proliferation causing epineural and perineural fibrosis with fatty infiltration around the nerve bundles. We report an unusual case of fibromatous hamartoma of the ulnar digital nerve of the thumb in a 43-year-old woman. Magnetic resonance imaging revealed a large fusiform mass along the nerve. The findings were unusual and pathognomonic and included a coaxial cable-like appearance on axial sections and a spaghettilike appearance on coronal sections on both T1- and T2-weighted images; these findings were useful for the diagnosis and preoperative evaluation of this lesion. Surgical exploration revealed a yellow, cordlike mass of the digital nerve enlarged by fat. Gross excision could not be done without extensive damage to the nerve. Therefore, a limited excision with biopsy of the fibrolipomatous tissue around the nerve bundles was performed. The histological appearance was consistent with fibrolipomatous hamartoma. There was no recurrence of the mass and no neurological deficit 3 years after surgery. Some authors have suggested that invasive excision can cause catastrophic sensory or motor deficits because of the extensive fatty infiltration of the nerve fascicles. In conclusion, the recommended treatment for this lesion is limited excision with only biopsy to confirm the diagnosis.
无巨指症的神经纤维脂肪瘤性错构瘤是一种罕见病变，其特征为纤维脂肪组织增生，导致神经外膜和神经束膜纤维化，并伴有神经束周围脂肪浸润。我们报告一例43岁女性拇指尺侧指神经的纤维脂肪瘤性错构瘤罕见病例。磁共振成像显示沿神经走行有一巨大梭形肿块。这些表现不同寻常且具有特征性，在T1加权像和T2加权像的轴位像上呈同轴电缆样外观，在冠状位像上呈意大利面条样外观；这些表现有助于该病变的诊断和术前评估。手术探查发现指神经有一黄色索状肿块，因脂肪而增粗。若不广泛损伤神经则无法进行整块切除。因此，对神经束周围的纤维脂肪组织进行了有限切除并活检。组织学表现符合纤维脂肪瘤性错构瘤。术后3年肿块无复发，也无神经功能缺损。一些作者认为，由于神经束广泛脂肪浸润，侵袭性切除可能导致灾难性的感觉或运动功能缺损。总之，对于该病变，推荐的治疗方法是仅进行有限切除并活检以确诊。
