Khadka Sulochana, Adhikari Prashant, Regmi Pradeep Raj, Timilsina Bibek, Thapa Anupam Singh, Panta Bhaskar Raj, Bhandari Sandeep
Nepalese Army Institute of Health Sciences, College of Medicine, Kathmandu, Nepal.
HAMS Hospital, Kathmandu, Nepal.
Ann Med Surg (Lond). 2022 Sep 5;81:104542. doi: 10.1016/j.amsu.2022.104542. eCollection 2022 Sep.
Lipofibromatous hamartoma of the nerve is the fibro-fatty overgrowth within the nerve. Most commonly they occur in the median nerve, ulnar nerves, and a few other nerves but the involvement of the sciatic nerve is very rare. The fibro-fatty infiltration causes palpable neurogenic mass and clinically presents lump, moderate numbness, tingling sensation, and pain in its territory. Magnetic resonance imaging is the gold standard for diagnosis.
We present a case of a 65 years old female, who presented to OPD with a tingling sensation which progressed to pain in the gluteal region and was associated with a tender swelling. MRI showed a giant space-occupying lesion in the sciatic nerve course. The mass was excised and then sent to the histopathological examination which designated the mass as lipofibromatous hamartoma.
Unless debilitating, lipomatosis of the nerve doesn't require any intervention as it is a benign condition. Lipofibromatous hamartoma is attributed to the accumulation of fatty and fibrous tissue in the epineurium. Diffusion-weighted imaging in association with conventional magnetic resonance imaging has increased diagnostic yield. The lesion was iso-intense to the subcutaneous fat and there were fine fibrillar appearances inside of it. Simple mass excision was performed on our patient without complications.
Lipofibromatous hamartoma of the nerve are rare soft tissue tumors of nerves and sciatic nerve involvement is even rarer. Correct and careful interpretation of the MRI findings can lead to diagnosis with ease and help prevent unnecessary biopsies.
神经脂肪纤维瘤性错构瘤是神经内纤维脂肪组织过度生长。最常见于正中神经、尺神经和其他一些神经，但坐骨神经受累非常罕见。纤维脂肪浸润导致可触及的神经源性肿块，临床上表现为肿块、中度麻木、刺痛感及其支配区域疼痛。磁共振成像（MRI）是诊断的金标准。
我们报告一例65岁女性，因出现刺痛感前来门诊就诊，刺痛感逐渐发展为臀区疼痛，并伴有压痛性肿胀。MRI显示坐骨神经走行区有巨大占位性病变。肿块被切除，随后送去做组织病理学检查，结果将肿块诊断为神经脂肪纤维瘤性错构瘤。
除非造成功能障碍，神经脂肪瘤病无需任何干预，因为它是一种良性疾病。神经脂肪纤维瘤性错构瘤归因于神经外膜中脂肪和纤维组织的积聚。扩散加权成像与传统磁共振成像相结合提高了诊断率。该病变与皮下脂肪呈等信号，内部有纤细的纤维状表现。我们的患者接受了单纯肿块切除术，无并发症发生。
神经脂肪纤维瘤性错构瘤是罕见的神经软组织肿瘤，坐骨神经受累更为罕见。对MRI结果进行正确、仔细的解读可轻松实现诊断，并有助于避免不必要的活检。
